ORL Health

Nasopharyngeal carcinoma • Myocardin • methylation

2010-04-28T22:42:00.001-07:00

Background

Epigenetic silencing of tumor suppressor genes plays an important role in nasopharyngeal carcinoma (NPC) tumorigenesis. In the present study, we explore a novel target gene of epigenetic silencing in NPC, Myocardin, which is inactivated by promoter hypermethylation.

Methods

Transcriptional expression levels of Myocardin were evaluated by reverse transcription-polymerase chain reaction (RT-PCR). Methylation status was addressed by methylation-specific PCR and bisulfite genomic sequencing.

Results

Myocardin mRNA expression was inactivated in 4 of 5 NPC cell lines. Myocardin was aberrantly methylated in 4 of 5 NPC cell lines (80%) and in 48 of 65 NPC primary tumors (73.8%, but not in any of the 12 normal nasopharyngeal tissues tested. Myocardinexpression could be reactivated in NPC cells after treatment with the demethylating agent 5-aza-2-deoxycytidine (5-aza-dC).

Conclusions

Epigenetic inactivation of Myocardin is a frequent and tumor-specific event in NPC. Our findings suggest that Myocardin is a candidate tumor suppressor gene in NPC.

Levels of a key type of immune cell are higher in head and neck cancer

2010-04-27T22:39:00.000-07:00

Levels of a key type of immune cell are higher in head and neck cancer patients whose tumors are linked to the human papillomavirus, or HPV, according to researchers at the University of Michigan Comprehensive Cancer Center.

The finding suggests a way to predict which tumors are most likely to respond to chemotherapy and radiation and allow doctors to choose the best treatment option up front.

"In the past, we would give toxic chemotherapy to a patient, look at how the tumor responded and then decide whether the patient needed surgery or radiation. Now with patients who have HPV-positive cancers, this study suggests we can look in the microscope, measure the level of these immune cells and, based on that, select a treatment that is going to be potentially less toxic for the patient and most effective at curing the cancer," says study author Gregory T. Wolf, M.D., professor and chair emeritus of otolaryngology at the U-M Medical School.

Results of the study will be presented April 29 at the American Head and Neck Society annual meeting.

The researchers looked at 66 patients with oropharyngeal cancer, which includes cancers of the tonsils and the tongue base. They measured levels of several immune system cells in the blood and tracked HPV status.

The HPV-positive patients had higher levels of a subset of T-lymphocyte cells, a type of immune cell that is responsible for killing tumor cells. Patients who responded to an initial round of chemotherapy also had higher levels of these cells, while patients whose cancer recurred had lower levels.

"When we looked at how successful chemotherapy and radiation were, the levels of those killer T-lymphocyte cells predicted who was going to do well. That ability to predict response was even better than when we look at whether the tumors were HPV-positive or negative," says Wolf, director of the Head and Neck Cancer Specialized Program of Research Excellence at the U-M Comprehensive Cancer Center.

Previous studies have shown that HPV-positive head and neck cancers tend to be more responsive to current treatments, and these patients overall tend to have better outcomes than patients with HPV-negative tumors.

The researchers suggest that these new findings could help them devise strategies to boost the immune system of HPV-negative patients and improve the success rate of current therapies.

"We're actively pursuing how we can capitalize on this information and devise better immunotherapy approaches to head and neck cancer that would be less toxic than surgery or intensive radiation and hopefully cure more patients," Wolf says.

Head and neck cancer statistics: 35,720 Americans will be diagnosed with head and neck cancer this year and 7,600 will die from the disease, according to the American Cancer Society

Warthin tumour - Lymphatic vessels - Podoplanin - D2-40 - Immunohistochemistry

2010-04-26T01:26:00.000-07:00

Warthin tumour is the second most common benign tumour of the parotid gland. This study was designed to investigate the lymphatic vessels in Warthin tumours in an effort to understand better its pathogenesis.

Materials and methods

Tissue specimens of 31 patients (19 men and 11 women; mean age 57 years, median size of the tumours 2.86 cm) were analysed by means of immunohistochemistry applying the monoclonal antibody D2-40.

Results

We found numerous D2-40-positive sinus-like vessels particularly at the inner layer of the capsule.

Conclusions

Since subcapsular sinuses are a major morphological feature of lymph nodes in general, the finding of podoplanin expression in the large majority of subcapsular vessels in Warthin tumours confirms the view that this tumour has its origin in regional lymph nodes.

Acute suppurative thyroiditis,Thyroid Abscess

2010-04-20T02:10:00.000-07:00

Acute suppurative thyroiditis is not a common complication in multinodular goiter.
The source of infection was not known.

Thyroid abscesses may be from surrounding sepsis or anatomic abnormality or spread from a distant site.
Bacteria may spread via a hematogenous route and can produce positive blood cultures.
The management may include complete excision of the suppurated tissues along with underlying thyroid pathology.

Eardrum Cholosteatoma

2010-04-20T02:08:00.000-07:00

http://www.entusa.com/surgery_videos_flash/ear-1-keratin-pearl/tympanoplasty-cholesteatoma_flv0.htm

Vocal Fold Bowing

2010-04-20T02:03:00.001-07:00

Treatment of Vocal Fold Bowing Using Neuromuscular Electrical Stimulation
Lisa A. LaGorio, MS; Giselle D. Carnaby-Mann, PhD; Michael A. Crary, PhD
Arch Otolaryngol Head Neck Surg. 2010;136(4):398-403.
Objective  To investigate the clinical effectiveness andsafety of a novel behavioral voice therapy program combiningstructured vocal exercise with adjunctive neuromuscular electricalstimulation for rehabilitating dysphonia secondary to vocalfold bowing.
Design  Prospective interventional clinical case serieswith a 3-month follow-up.
Setting  Outpatient speech and hearing clinic in an academicmedical center.
Patients  Convenience sample of 7 patients diagnosed byan otolaryngologist as having chronic dysphonia for at least3 months due to bilateral vocal fold bowing.
Intervention  A novel voice therapy program incorporatingexercise principles and sustained phonations of increasing length,volume, and pitch paired with concurrent transcutaneous neuromuscularelectrical stimulation.
Main Outcome Measures  Change in maximum phonation time,highest attainable pitch, glottal closure, supraglottic compression,and Voice Handicap Index.
Results  Maximum phonation time for /i/ increased significantly(z = –2.201, P < .03), with a modesteffect demonstrated (Hedges g, 0.65; 95% confidence interval,–0.56 to 1.75). Voice Handicap Index trended toward significance(z = –1.787, P < .07). Glottalclosure during phonation improved, and supraglottic compressiondecreased. Improvements were maintained or enhanced at the 3-monthfollow-up. Analysis of highest attainable pitch data was limitedowing to aperiodicity in the baseline evaluations.
Conclusions  Behavioral voice therapy with adjunctive neuromuscularelectrical stimulation reduced vocal fold bowing, resultingin improved acoustic, laryngeal, and patient-centered outcomes.Maximum phonation time and glottal closure results imply increasedvocal fold tension secondary to enhanced thyroarytenoid or cricothyroidmuscle function after voice therapy.

Carotid-Cavernous Fistula

2010-04-20T02:01:00.000-07:00

http://www.ajronline.org/cgi/reprint/106/2/376.pdf

Thyroid Nodules

2010-04-16T07:35:00.001-07:00

Thyroid nodules are solid or fluid-filled lumps that form within the thyroid which is a small gland located at the base of the neck. They often go unnoticed but sometimes they can be felt as a lump in the throat.

When they are large or when they occur in very thin individuals, they can even sometimes be seen as a lump in the front of the neck.

The great majority of thyroid nodules are noncancerous and do not cause symptoms. Only a small percentage of thyroid nodules are cancerous.

Thyroid nodules are extremely common in young adults and children. Almost 50 percent of people have had one, but they are usually only detected during the course of a health examination. Some thyroid nodules, however, may become large enough to press on the windpipe, making it uncomfortable or difficult to swallow.

Treatment options depend on the type and size of the thyroid nodule.

What are the signs and symptoms of thyroid nodules?

A symptom is something the patient senses and describes, while a sign is something other people, such as the doctor notice. For example, drowsiness may be a symptom while dilated pupils may be a sign.

Most thyroid nodules do not cause any signs or symptoms. Occasionally, however, some nodules become so large that they can be felt or they can be detected by the swelling at the base of the neck.

They may become apparent when going about everyday activities such as shaving or putting on makeup, or tightening a shirt collar.

In some cases, thyroid nodules produce too much thyroxine, a hormone normally secreted by the thyroid gland. The extra thyroxine can cause problems such as:

Nervousness
Rapid or irregular heartbeat
Sudden, unexplained weight loss

Only a small percentage of lumps in the neck are malignant, and most thyroid nodules are benign.

A nodule is more likely to be cancerous if it:

Grows quickly
Feels hard
Causes the person to become hoarse
Causes trouble swallowing
Causes difficulty in breathing
Causes enlarged lymph nodes under the jaw or in the neck

Most thyroid nodules are noncancerous and do not cause problems but it is recommended to seek medical advice and evaluation of any unusual swelling in the neck. This is important if trouble in breathing or swallowing is experienced.

Seek medical care if signs and symptoms of hyperthyroidism are developing, such as:

A pounding heart
Irritability
Muscle weakness
Nervousness
Sudden weight loss even though appetite is normal or has increased
Trouble sleeping

Thyroid disease: A person who has overactive thyroid (hyperthyroidism) may experience unusual nervousness, restlessness, anxiety and irritability. On the other hand, a person who has underactive thyroid (hypothyroidism) may experience mild to severe fatigue and depression.

However, it is unlikely that emotional symptoms such as anxiety or depression would be the only evidence of thyroid disease. Thyroid disease is usually associated with signs and symptoms such as weight gain or loss, sensitivity to hot or cold, bowel movement changes and menstrual irregularities.

The thyroid gland

The thyroid gland consists of two lobes. It takes up iodine from the food that is eaten. It uses it to manufacture two main hormones:

thyroxine (T4)
triiodothyronine (T3).

These hormones maintain the rate at which the body uses fats and carbohydrates. They help control body temperature, influence heart rate, and help regulate the production of protein. The thyroid gland also produces calcitonin which is a hormone that regulates the amount of calcium in the blood.

What are the causes of thyroid nodules?

In most cases, what causes most nodules to develop is unclear. But the following factors are sometimes involved:

A lack of iodine in the diet.
A genetic defect of receptors that release thyroid-stimulating hormone (TSH).
Hashimoto's disease. It is an autoimmune disorder that causes chronic inflammation and underactivity (hypothyroidism) of the thyroid gland.
Radiation treatments to the head or neck in childhood.

Several types of nodules can develop in the thyroid gland:

Colloid nodule. The majority of thyroid nodules are colloid nodules. They are noncancerous overgrowths of normal thyroid tissue. There can be one colloid nodule or many. These nodules may grow larger. However, they do not spread beyond the thyroid gland.

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Follicular adenoma. Another type of benign nodule.
Thyroid cyst. They are fluid-filled areas of the thyroid. They can range in size from less than 1/3-inch (about 8 millimeters) in diameter to 1 inch (25 millimeters) or more. Many thyroid cysts are entirely filled with fluid. But some cysts, called complex cysts, also have solid components.

Fluid-filled cysts are usually benign. Complex cysts are sometimes malignant.
Inflammatory nodule. This occasionally develops as a result of chronic inflammation of the thyroid gland (thyroiditis).

Subacute thyroiditis is a rare type of thyroiditis that causes severe pain in the thyroid gland. Other types are painless and sometimes occur after pregnancy and are known as postpartum thyroiditis.
Multinodular goiter. "Goiter" is a term used to describe any enlargement of the thyroid gland. Several factors can lead to a goiter, including the presence of a number of thyroid nodules. This condition, called multinodular goiter, can cause a tight feeling in the throat and difficulty breathing or swallowing.
Hyperfunctioning thyroid nodule. Including:
- toxic adenoma
- toxic multinodular goiter
- Plummer's disease
These nodules grow and produce thyroid hormones independent of the influence of TSH which is a substance released by the pituitary gland. It normally regulates the production of the thyroid hormones.

Hyperfunctioning thyroid nodules cause high blood levels of thyroxine along with low or nonexistent levels of TSH. A genetic defect of the TSH receptors may play a role in the overactivity of these nodules.
Thyroid cancer. There is a small possibility that a nodule is malignant. There is higher risk for people who:
- have a family history of thyroid or other endocrine cancers
- are younger than 30 or older than 60
- are male
- have a history of head or neck radiation
Malignant nodules are usually large and hard and may cause neck discomfort or pain.

There are many factors to consider when diagnosing a malignant lump. If the patient is a smoker, the chances of malignancy are considerably higher. When there is difficulty swallowing or breathing, this may be a symptom of a serious condition and requires speedy medical attention. Needle aspiration biopsy can be used to assess the condition.

What are the risk factors of thyroid nodules?

A risk factor is something which increases the likelihood of developing a condition or disease. For example, obesity significantly raises the risk of developing diabetestype 2. Therefore, obesity is a risk factor for diabetes type 2.

Thyroid nodules are common. Almost 50 percent of people may have one. The exact cause of most thyroid nodules is unknown.

Certain factors appear to increase the risk:

Age. The likelihood of developing thyroid nodules increases with age. Some changes in thyroid tissue may occur as a normal part of aging.
Certain thyroid conditions. Nodules are more likely to form in people who have or have had thyroiditis which is a chronic inflammation of the thyroid gland.
Gender. Women are more likely to develop thyroid nodules than men are.
Heredity. People who have a parent or sibling with thyroid nodules have a greater chance of developing them as well.
Radiation exposure. Back in the 1940s and 1950s, children, teenagers and even newborns were often treated with radiation for benign conditions, such asacne or enlarged tonsils. Radiation therapy to the neck or head for conditions such as acne increases the risk of developing thyroid nodules.

There is also an increased risk for people who were exposed to radioactive particles released into the air during atomic weapons testing or in nuclear power plant accidents.

What are the complications of thyroid nodules?

Most thyroid nodules are noncancerous. However, they sometimes can cause serious complications:

Large nodules or a multinodular goiter that can interfere with swallowing or breathing.
Serious problems can occur when a nodule or goiter produces thyroid hormone, leading to hyperthyroidism.

Hyperthyroidism

In addition to signs and symptoms such as unintended weight loss, muscle weakness, heat intolerance, and anxiousness or irritability, can also cause the following complications:

Heart-related complications. These can include

rapid heart rate
atrial fibrillation which is a heart rhythm disorder
congestive heart failure which is a condition in which the heart becomes too weak to circulate enough blood to meet the needs of the body

Osteoporosis (weak, brittle bones). The strength of the bones depends, in part, on the amount of calcium and other minerals they contain. An excess of thyroid hormone interferes with the body's ability to incorporate calcium into the bones. Actually, hyperthyroidism often affects the bones before any other signs or symptoms of the disorder are experienced. This is especially true of postmenopausal women who are already at high risk of osteoporosis.

Thyrotoxic crisis. This is a sudden and potentially life-threatening intensification of the signs and symptoms. It requires immediate medical care.

How are thyroid nodules diagnosed?

Sometimes thyroid nodules may be felt, usually just below and to the right or left of the Adam's apple. Most are discovered during a routine medical exam. During examination, the patient will be asked to swallow because a nodule in the thyroid gland will usually move up and down during swallowing. A nodule that forms in other parts of the neck will not move.

Sometimes, a thyroid nodule is detected during an imaging test such as an ultrasound, computerized tomography (CT) or magnetic resonance imaging (MRI) scan to evaluate another condition in the head or neck. Nodules detected this way are usually smaller than those found during a physical exam.

Additional tests

Once a nodule is discovered, it is necessary to determine whether it is malignant or associated with thyroid dysfunction. The following tests may be recommended:

Thyroid function tests. The thyroid gland produces two main hormones, thyroxine and triiodothyronine. The rate at which these hormones are released is part of a carefully controlled system involving the thyroid gland, the pituitary gland and the hypothalamus (an area at the base of the brain that acts as a thermostat for this system). Tests can measure blood levels of thyroxine, triiodothyronine and thyroid-stimulating hormone which are released by the pituitary gland. The results can indicate whether the thyroid is producing too much thyroxine (hyperthyroidism) or too little (hypothyroidism). However, these tests cannot determine if a nodule is benign or malignant.
Fine-needle aspiration (FNA) biopsy. This test helps distinguish between benign and malignant thyroid nodules. During the procedure, a thin needle is inserted in the nodule and a sample of cells is removed. The procedure, which is carried out in the doctor's office, takes about 20 minutes and has few risks. Several samples will be taken from a single nodule. If there is more than one nodule, samples will be taken from all of them. In some cases, an ultrasound will be used to help guide the placement of the needle. The samples are then sent to a laboratory and analyzed under a microscope.

Most nodules diagnosed using FNA biopsy are benign. These nodules may grow. But they are not cancerous and will not spread beyond the thyroid gland.

A small percentage of biopsied nodules are cancerous. This diagnosis is based on the characteristics of individual cells and patterns in clusters of cells that are different from normal thyroid tissue. In some cases, a pathologist can determine specific types of cancer from an FNA biopsy sample.

Sometimes there may not be enough cells in a sample to accurately determine whether a nodule is benign or malignant. In that case, the test is likely to be repeated.

In some FNA biopsies, the test results are considered suspicious or indeterminate. This means there is no definitive way to tell from the biopsy sample whether the nodule is cancerous. Repeat biopsies usually are not helpful in those suspicious cases. The next step may be surgery to remove the nodule for a definitive diagnosis.
Ultrasonography. This imaging technique uses high-frequency sound waves to produce images. It provides the best information about the shape and structure of nodules. It may be used to distinguish cysts from solid nodules, to determine if multiple nodules are present and as a guide in performing an FNA biopsy.
Thyroid scan. Sometimes a thyroid scan can help evaluate thyroid nodules. During this test, an isotope of radioactive iodine is injected into the vein on the inside of the elbow. A special camera produces an image of the thyroid on a computer screen.

Hot nodules are nodules that produce excess thyroid hormone. They show up on the scan because they take up more of the isotope than normal thyroid tissue does. Cold nodules are nonfunctioning and appear as defects or holes in the scan. Hot nodules are almost always noncancerous. About 5 percent of cold nodules are cancerous.

The disadvantage of a thyroid scan is that it cannot distinguish between benign and malignant cold nodules. During a thyroid scan, the patient will be exposed to a small amount of radiation.

What are the treatments options for thyroid nodules?

The treatment options depend on the type of thyroid nodule. They may include:

Watchful waiting. If a biopsy shows that the thyroid nodule is benign, your doctor may suggest simply watching the condition. This usually means having a physical exam and thyroid function tests at regular intervals. If the nodule grows larger another biopsy is likely to be performed. If a benign thyroid nodule remains unchanged, there may never be need for treatment beyond careful monitoring. Discuss this approach with your doctor or get more information on other options.
Thyroid hormone suppression therapy. This involves treating a benign nodule with levothyroxine (Levoxyl, Synthroid), a synthetic form of thyroxine that is taken in pill form. The supply of additional thyroid hormone will signal the pituitary to produce less TSH, the hormone that stimulates the growth of thyroid tissue.

There is some debate on levothyroxine therapy. There is no clear evidence that the treatment consistently shrinks nodules or even that shrinking small, benign nodules is necessary.
Radioactive iodine. Doctors often use radioactive iodine to treat hyperfunctioning adenomas or multinodular goiters. Taken as a capsule or in liquid form, radioactive iodine is absorbed by the thyroid gland. This causes the nodules to shrink and signs and symptoms of hyperthyroidism to drop, usually within two to three months.

The thyroid hormone is released into the bloodstream as the nodules are destroyed. In rare cases, symptoms may worsen for a few days or weeks after therapy. Neck tenderness or a sore throat may be experienced. In addition, because this treatment eventually causes thyroid activity to slow considerably, the patient may develop hypothyroidism.
Surgery. Surgical removal is the usual treatment for malignant nodules. The majority of thyroid tissue is also removed during a procedure called near-total thyroidectomy. Occasionally, a nodule that is clearly benign may require surgery, especially if it is so large that it makes it hard to breathe or swallow. Surgery is also considered the best option for people with large multinodular goiters, particularly when the goiters constrict airways, the esophagus or blood vessels.

Nodules diagnosed as indeterminate or suspicious by a biopsy also need surgical removal so they can be examined more thoroughly for signs of cancer.

Risks of thyroid surgery include:
- Damage to the nerve that controls the vocal cords.
- Damage to the parathyroid glands which are four tiny glands located on the back of the thyroid gland that help control the level of calcium in the blood.
After thyroidectomy, patients need lifelong treatment with levothyroxine to supply their body with normal amounts of thyroid hormone.

Cystic Fibrosis

2010-04-16T07:32:00.001-07:00

Pure tone audiometry and analysis of the distortion product otoacoustic emissions revealed that there was a high prevalence of hearing loss, which makes cystic fibrosis patients a high-risk group which needs periodic assessment by an otorhinolaryngologist.

Comparison of the groups with and without aminoglycosides use showed that there was no statistically significant difference among them in analyses, which suggests that the use of aminoglycosides is not the only causal factor for hearing loss in cystic fibrosis.

Matrix Metallo-Proteinases and Chronic Tonsillitis

2010-04-16T07:31:00.000-07:00

The presence of MMPs in tonsil tissue consolidates the involvement of degraded extracellular matrix proteins in the pathophysiology of chronic tonsillitis.

MMPs activity showed diffuse dissemination in the tonsillar tissue and especially MMP-9 and MMP-7 are the main promoters of the extracellular matrix that responded to inflammatory changes in the tonsillar tissue.

Further studies are needed concerning the possible efficiency of selective MMP inhibitors on tonsillar tissue.

Muckle–Wells syndrome

2010-04-16T07:26:00.000-07:00

Muckle–Wells syndrome (MWS), also known as urticaria-deafness amyloidosis (UDA), is a rare autosomal dominant disease which causes
sensorineural deafness,
recurrent hives, and can lead to
amyloidosis.

Individuals with MWS often have episodic fever, chills, and painfuljoints.

As a result, MWS is considered a type of periodic fever syndrome.

MWS is caused by a defect in the CIAS1 gene which creates the protein cryopyrin. MWS is closely related to two other syndromes, familial cold urticaria and neonatal onset multisystem inflammatory disease— in fact, all three are related to mutations in the same gene and subsumed under the term cryopyrin-associated periodic syndromes (CAPS).

Johanson-Blizzard Syndrome

2010-04-16T07:24:00.001-07:00

Johanson-Blizzard Syndrome (JBS) is an extremely rare inherited disorder characterized by an unusually small nose that appears "beak shaped" due to absence (aplasia) or underdevelopment (hypoplasia) of the nostrils (nasal alae); abnormally small, malformed primary (deciduous) teeth and misshapen or absent secondary (permanent) teeth; and/or unusually sparse, dry, coarse scalp hair that tends to have a distinctive "upsweep" in the forehead area. In addition, affected infants may have a low birth weight, demonstrate signs of insufficient intestinal absorption (malabsorption) of fats and other nutrients due to abnormal development of the pancreas (exocrine pancreatic insufficiency), and fail to grow and gain weightat the expected rate (failure to thrive) during the first years of life, contributing to short stature.

Approximately one third of infants with Johanson-Blizzard Syndrome also demonstrate abnormally decreased activity of the thyroid gland and underproduction of thyroid hormones (hypothyroidism), causing generalized weakness and contributing to growth retardation as well as abnormal delays in the acquisition of skills requiring the coordination of mental and physicial activity (psychomotor retardation). In many cases, affected infants may also exhibit hearing impairment of both ears at birth due to abnormalities of the inner ear (congenital bilateral sensorineural hearing loss) and may experience associated, severe speech impairment. In addition, approximately 60 percent of affected children have moderate mental retardation; however, others may have normal intelligence or mild retardation. In many cases, additional abnormalities may also be present. The range and severity of symptoms may vary greatly from case to case. Johanson-Blizzard Syndrome has autosomal recessive inheritance.

Rosai-Dorfman disease,Sinus histiocytosis with massive lymphadenopathy

2010-04-16T06:59:00.000-07:00

Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare, benign disorder of unknown etiologythat is characterized by the overproduction of histiocytes, which accumulate in lymph nodes throughout the body.
Lymphadenopathy of the neck is the most common place of histiocyte accumulation, although accumulation outside of lymph nodes may occur, as well. The most common sites of accumulation outside of the lymph nodes are skin, upper respiratory tract, and the sinuses.
The symptoms of this disease vary with the site of accumulation. This condition has been named after Ronald F. Dorfman and Juan Rosai. A synonym of this condition is known as Destombes-Rosai-Dorfman syndrome, part of which is named after Pierre-Paul Louis Lucien Destombes

Pelizaeus-Merzbacher Disease

2010-04-16T06:56:00.000-07:00

Table of Contents (click to jump to sections)

What is Pelizaeus-Merzbacher Disease?
Is there any treatment?
What is the prognosis?
What research is being done?
Clinical Trials
Organizations

What is Pelizaeus-Merzbacher Disease?

Pelizaeus-Merzbacher disease (PMD) is a rare, progressive, degenerative central nervous system disorder in which coordination, motor abilities, and intellectual function deteriorate.

The disease is one of a group of gene-linked disorders known as the leukodystrophies, which affect growth of the myelin sheath -- the fatty covering that wraps around and protects nerve fibers in the brain.

The disease is caused by a mutation in the gene that controls the production of a myelin protein called proteolipid protein-1 (PLP1). PMD is inherited as an X-linked recessive trait; the affected individuals are male and the mothers are carriers of the PLP1 mutation.

Severity and onset of the disease ranges widely, depending on the type of PLP1 mutation.

PMD is one of a spectrum of diseases associated with PLP1, which also includes Spastic Paraplegia Type 2 (SPG2). The PLP1-related disorders span a continuum of neurologic symptoms that range from severe central nervous system involvement (PMD) to progressive weakness and stiffness of the legs (SPG2).

There are four general classifications within this spectrum of diseases. In order of severity, they are:

Connatal PMD, which is the most severe type and involves delayed mental and physical development and severe neurological symptoms;
Classic PMD, in which the early symptoms include muscle weakness, involuntary movements of the eyes (nystagmus), and delays in motor development within the first year of life;
Complicated SPG2, which features motor development issues and brain involvement, and,
Pure SPG2, which includes cases of PMD that do not have neurologic complications.

Noticeable changes in the extent of myelination can be detected by MRI analyses of the brain. Additional symptoms of PMD may include slow growth, tremor, failure to develop normal control of head movement, and deteriorating speech and mental function.

Is there any treatment?

There is no cure for Pelizaeus-Merzbacher disease, nor is there a standard course of treatment. Treatment is symptomatic and supportive and may include medication for movement disorders.

What is the prognosis?

The prognosis for those with the severe forms of Pelizaeus-Merzbacher disease is poor, with progressive deterioration until death. On the other end of the disease spectrum, individuals with the mild form, in which spastic paraplegia is the chief symptom, may have nearly normal activity and life span.

What research is being done?

NINDS supports research on gene-linked disorders, including the leukodystrophies. The goals of this research are to increase scientific understanding of these disorders and to find ways to prevent, treat, and ultimately cure them.

enign paroxysmal vertigo, Cervical vestibular-evoked myogenic potential (cVEMP), Migraine equivalent, Ocular vestibular-evoked myogenic potential (oVEMP), Stabilometry

2010-04-16T06:53:00.001-07:00

Objective

This study utilized a combined ocular vestibular-evoked myogenic potential (oVEMP) and cervical VEMP (cVEMP) test in children with benign paroxysmal vertigo (BPV) to investigate whether the upper or lower brainstem is more frequently affected in BPV children.

Methods

Fifteen BPV children aged 4–14 years, and 15 age- and sex-matched healthy children were enrolled. All subjects underwent pure tone audiometry, stabilometry, and a combined oVEMP and cVEMP test using acoustic stimulation.

Results

All BPV patients displayed normal hearing and clear oVEMPs. However, 11 (73%) of 15 BPV patients had delayed cVEMPs, showing significant difference when compared with 100% normal cVEMPs in healthy children. The sway path and sway area in stabilometry were significantly different between BPV and healthy children, regardless of whether their eyes were open or closed. However, neither the sway path nor sway area correlated significantly with cVEMP results.

Conclusion

Normal oVEMPs in BPV children indicate an intact vestibulo-ocular reflex pathway, which travels through the upper brainstem. In contrast, delayed cVEMPs in BPV children reflect a retrolabyrinthine lesion along the sacculo-collic reflex pathway, which descends via the lower brainstem. Hence, the lower brainstem is more frequently affected than the upper brainstem in children with BPV.

Keywords: Benign paroxysmal vertigo, Cervical vestibular-evoked myogenic potential (cVEMP), Migraine equivalent, Ocular vestibular-evoked myogenic potential (oVEMP), Stabilometry

Piezoelectric device in revision surgery for chronic otitis media.

2010-04-13T00:20:00.000-07:00

For otologic revision surgery, the advantage of the piezoelectric device appears real because it is possible to perform a 'blind' cutting of bone with fewer precautions necessary for soft tissues such as the facial nerve, lateral sinus, and dura mater.

Objectives: The aim of this study was to determine the efficiency of the piezoelectric device in revision surgery for chronic otitis media.

Methods: A total of 30 patients had revision mastoidectomy with previous canal wall up mastoidectomy. The piezoelectric device was used in all intraoperative steps. Before surgery and 1 month and 1 year after surgery, all the patients underwent the following instrumental examinations: pure-tone audiometry, tympanometry, transient-evoked otoacoustic emissions, distortion product otoacoustic emissions, auditory brainstem response, and electronystamographic recording.

Results: The piezoelectric device provided effective cutting, with excellent control and without side effects on the adjacent structures of the middle and inner ear (lateral sinus, facial nerve, and/or dura mater). Postoperatively, all patients had an uneventful recovery with no evidence of audiovestibular deficit or side effects. Among 30 cases followed for 1 year, 29 (97%) maintained a dry and safe ear. Intermittent otorrhea with perforation of the tympanic membrane occurred in one patient (3%).

Piezo-surgery

2010-04-13T00:16:00.001-07:00

The Piezosurgery has been developed to overcome the limits of precision and intraoperatory safety existing in traditional bone cutting instruments. Piezosurgery allows to obtain high predictability and low morbidity in bone surgery.

Piezosurgery® was invented by Prof. Tomaso Vercellotti.

Piezosurgery® Device has been invented, engineered and produced byMectron Medical Technology.

8 years of experience in scientific research and clinical studies

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Via Loreto, 15/A16042 Carasco (GE) - ITALY
Tel. +39 0185 35361
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www.mectron.com
e-mail: mectron@mectron.com

Sinus Lift Procedure Works

2010-04-13T00:14:00.001-07:00

http://www.youtube.com/watch?v=fKP-XoL06Q8&feature=related

Upper level and lower level obstructive apneas

2010-04-13T00:11:00.000-07:00

This study demonstrates that there is a significant difference in sound spectrum between first snoring sound after upper level and lower level obstructive apneas, and it is possible to use sound spectrum analysis as a supplementary method to determine the obstructive site of a patient with obstructive sleep apnea syndrome (OSAS).

Objectives:
To investigate the difference in sound spectrum between the first snoring sounds after upper level (above the free margin of soft palate) obstructive apneas and lower level (below the free margin of soft palate) obstructive apneas in patients with OSAS.

Methods:
Thirty male patients with OSAS were included in this study. Overnight snoring sound was recorded and continuous upper airway pressure measurement was performed simultaneously on each subject. The first snores after upper level and lower level obstructive apneas were identified and analyzed acoustically.

Results:
The mean of peak frequencies, central frequencies, and proportions of energy

from 800 Hz to 2000 Hz and above 2000 Hz of the first snoring sounds after lower level obstructive apneas were higher
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and the proportion of energy

below 800 Hz was lower than those after upper level obstructive apneas.
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The differences of all the parameters were of significance. The power spectrum of the two types of snoring sounds also exhibited different characters.

Papillary thyroid microcarcinoma.

2010-04-13T00:04:00.000-07:00

Objective: To discuss the prognostic factors and outcomes of treatment in patients diagnosed with papillary thyroid microcarcinoma.

Design: Retrospective observational case review.

Setting: Department of Otorhinolaryngology of the tertiary referral teaching hospital of Parma.

Participants: Ninety-seven patients diagnosed with PTMC and surgically treated between January 1998 and December 2007.

Main outcome measures: Clinical and histopathological characteristics of the study group were identified and statistically analysed.

Results: No cancer-related deaths were registered. Incidence of recurrent disease was 9% after a 43 month mean follow-up (range 12–120). Univariate analysis detected that metastases in neck lymph nodes at diagnosis (p = 0.025), a tumour >5 mm in size (p = 0.011),

the presence of bilateral tumoural foci (p = 0.007),

the presence of capsular invasion (p = 0.001), and

the presence of vascular invasion (p = 0.004) were related to recurrent disease.

On multivariate analysis, the presence of bilateral tumoural foci (p = 0.030), and the presence of capsular invasion (p = 0.005) were significantly related to tumour recurrence.

Conclusion: The prognosis for patients with papillary thyroid microcarcinoma in this series was excellent, with a 100% survival rate and minimal surgical-related morbidity. Nevertheless, approximately 10% of patients developed recurrent disease; aggressive treatment may be justified depending on the presence or absence of prognostic risk factors.

Barotitis

2010-04-12T02:55:00.001-07:00

Middle ear problems due to changing atmospheric pressures, as when a plane descends to land.

The problems include ear pain, ringing, diminished hearing and, sometimes, dizziness.

The word barotitis (pronounced bar-o-tit-is) is compounded from bar- as in barometer + otitis meaning inflammation of the ear. Alternate terms include aerotitis, aerotitis media, barotitis media, and otic barotrauma.

Magnetic resonance imaging cine - Dynamic MRI - Swallowing physiology - Deglutition disorders

2010-04-12T02:47:00.000-07:00

With the advent of dynamic fast MRI sequences the act of deglutition can be dynamically visualized in cine-mode. T

wenty-three healthy volunteers were enrolled in this study to define the morpho-functional patterns of oral and pharyngeal deglutition using new dynamic MRI techniques. All subjects were previously submitted to video endoscopic assessment, to exclude swallowing abnormalities. As contrast material a combination of yogurt mixed with gadolinium-diethylene diamine pentaacetic acid was used.

The protocol was divided into three parts:

(a) preliminary assessment of the oral cavity, pharynx and laryngeal structures;

(b) morphologic assessment of tongue, soft palate, pharynx, epiglottis and larynx-hyoid bone;

(c) dynamic assessment of swallowing without administrating any contrast media and, in subsequent phase, by injecting 5 ml of yogurt-based contrast medium in the patient’s mouth.

The time resolution was 3–4 images/s.

The MR protocol revealed to be effective in the evaluation of normal motility patterns of the structures involved in swallowing.

Moreover, the evaluation of the bolus progression, slowdown or stagnation, was possible. On the contrary problems were encountered in calculating precisely the bolus progression time, because of the insufficient temporal resolution. However, more energy should be invested to optimize the spatial and temporal resolution of turbo-FLASH sequences, to obtain a better dynamic representation of a complex function such as deglutition.

Serous Otitis Media Causing Autistic Symptoms

2010-04-09T02:17:00.001-07:00

My three year old daughter was diagnosed with developmental delay when she was 9 months old. When she was two and a halfÂ and she still wasn't walking I asked an ENT doctor to look into her ears. What he found was undiagnosed serous otitis media also called otitis media with effusion (OME). This means that there was fluid in her middle ear space. She had never had an ear infection, the fluid wasn't infected rather her adenoidswere enlarged and preventing her Eustachian tube from draining. This affected her hearing and balance. One week after the fluid was removed she started walking.

We noticed a lot of improvements right after her myringotomy and adenoidectomy but she still didn't come close to developing like a normal child her age. At this point (about 9 months after surgery), she attends a school for autistic children which specializes in ABA therapy. She has not been diagnosed with autism, but experts agree that she qualifies for a minimum diagnosis of PDD-NOS. While she does occasionally stim, her deficits are almost primarily communicative. While I know that she can hear at least some of the time she still has no startle reflex and I often have to call her multiple times before she will acknowledge me.

Just when I think I've come to terms with the idea that my daughter has autism I come across something like this study that makes me stop and think again. According to thearticleÂ in Medical News Today scientists have discovered that a temporary hearing deficit, (such as is often caused by OME), at an early age can affect your child's hearing permanently, even after the problem should have resolved. According to researchers the auditory cortex must be stimulated at specific times (critical periods in a child's development) to develop properly and if it is not it can lead to long term problems. Impaired hearing makes it very difficult for children to learn how to talk, so the period of hearing loss leads to multiple communication deficits.

A quote from the article I'd like to share: ". . . children commonly experience a buildup of viscous fluid in the middle ear cavity, called otitis media with effusion, which can degrade the quality of acoustic signals reaching the brain and has been associated with long-lasting loss of auditory perceptual acuity," explains senior study author, Dr. Daniel Polley from the Massachusetts Eye and Ear Infirmary."

I don't know when or how I'll discover what really happened with my daughter but I encourage other parents to be aware of OME and to be extra vigilant if they have a child with development delays. I say, "extra vigilant," because I was aware of OME but it was ruled out by my regular pediatrician and two audiologists who had done tympanometrytesting on my daughter before it was finally discovered by our ear, nose and throat doctor. Fluid in the ear can be difficult to diagnose, it takes an experienced specialist to notice subtle changes in the ear drum. Also, remember, that the inner ear controls balance so gross motor delays are common among children with OME.

Lateral semicircular canal dehiscence,Tullio phenomenon,Sound-induced vertigo

2010-04-09T02:02:00.000-07:00

Dehiscence of the lateral semicircular canal (LSCD) has been reported much but mainly in association with cholesteatoma and canal wall down mastoidectomy, while idiopathic LSCD was rarely reported.

Bassim reported one case with lateral semicircular canal dehiscence, but presented no vestibular or auditory symptoms.

The patient in this study complained significant sound-induced vertigo and autophony in his right ear.

The axis of nystagmus was orthogonal to the lateral semicircular canal, and no torsional or vertical motions were observed, so pathology of the lateral semicircular canal was preferentially considered.

Benign paroxysmal positional vertigo was excluded since vertigo attacks had no relation to the change of head position.

The dehiscence of the right lateral semicircular canal was then confirmed through the high-resolution temporal bone computer tomography scan and the reconstructed images.

The cause of the LSCD is poorly understood, since no history of head trauma, otological infection or surgery was documented.

Puffed-cheek computed tomography technique,Pneumoparotitis

2010-04-08T00:20:00.000-07:00

Pneumoparotitis is a rare etiology of symptomatic parotid gland enlargement that is often misdiagnosed clinically. Pneumoparotitis results from air refluxing into the parotid ductal system via an incompetent Stensen duct orifice. We report a protracted case of pneumoparotitis evaluated with the puffed-cheek computed tomography technique to help confirm the diagnosis.

Vestibular Neuritis and Corticosteroids

2010-04-07T01:18:00.001-07:00

Goudakos et al (2010) published their meta-analysis that reviewed controlled trials of placebo versus corticosteroids with regard to treating patients with vestibular neuritis. They reported that vestibular neuritis (VN) is among the three most common causes of peripheral vestibulopathy, and VN has an incidence of 3.5 per 100,000.

Symptoms of VN include acute (or sub-acute) sustained rotatory vertigo, gait imbalance, postural imbalance and may include falls, vomiting and nausea. The natural course of VN is characterized by incomplete recovery facilitated through proprioceptive and visual substitution, as well as central compensation for the unilateral vestibular deficit.

The authors identified 157 publications, 34 of the 157 were potentially eligible, but eventually only four publications had enough data and information for inclusion in the meta-analysis. The four were published between 1990 and 2008 and included data for 159 patients. Of the 159 patients, 78 were in the corticosteroids group, 81 were in the placebo group.

They reported at one month post VN onset, that there were no differences in clinical symptoms between the corticosteroid and placebo groups. However, with regard to caloric recovery, there was a difference at one month post VN onset. Specifically, subjects receiving corticosteroid treatments demonstrated caloric recovery evident at one month and at 12 months. However, the present meta-analysis confirmed the (previously reported) lack of correlation between clinical recovery from VN and caloric results/improvements.

Goudakos et al note that the benefit of administering corticosteroids to VN patients appears limited to improving the caloric extent and recovery of canal paresis in VN patients and based on the present meta-analysis, clinical recovery does not seem enhanced via corticosteroids.

Killian's triangle • Killian's dehiscence • Zenker's diverticulum • inferior pharyngeal constrictor muscle • cricopharyngeal muscle

2010-04-07T01:14:00.000-07:00

Objectives/Hypothesis:

To determine the prevalence and the morphometric characteristics of Killian's triangle and to examine their relationship with the anthropometric features of the person.

Study Design:

Prospective.

Methods:

The hypopharyngeal wall was examined on 47 human cadavers. After a standardized method of dissection, the muscular structures of the hypopharyngeal wall were demonstrated and the existence or absence of Killian's triangle was determined. The triangle was examined in regard to its morphometric characteristics. In addition, anthropometric examinations of the whole body and of the head and neck regions on these cadavers were done, and the results were compared with the features of Killian's dehiscence.

Results:

A triangle of Killian was present on 60% (9/15) of the males and on 34% (11/32) of the females. The mean height for the males was 7 mm and for the females 4 mm, and the width was on average 16 mm and 12 mm, respectively. The dimensions of the triangle were correlated significantly with the dimensions of the body and with the length and the descensus of the larynx.

Conclusions:

The frequency and the dimensions of Killian's triangle appear to be greater in males than in females. Furthermore, the morphometric characteristics of the dehiscence show a correlation with the anthropometric features of the person. These findings can be an explanation for the higher incidence of Zenker's diverticulum with men and for the geographical differences of the diverticulum's frequency.

Presbyphonia

2010-04-07T01:12:00.000-07:00

Objectives/Hypothesis:

Age, vocal fold atrophy, glottic closure pattern, and the burden of medical problems are associated with voice therapy outcomes for presbyphonia.

Study Design:

Retrospective.

Methods:

Records of patients seen over a 3-year period at a voice center were screened. Inclusion criteria consisted of age over 55 years, primary complaint of hoarseness, presence of vocal fold atrophy on examination, and absence of laryngeal or neurological pathology. Videostroboscopic examinations on initial presentation were reviewed. Voice therapy outcomes were assessed with the American Speech-Language-Hearing Association National Outcomes Measurement System scale. Statistical analysis was performed with Spearman rank correlation and ² tests.

Results:

Sixty-seven patients were included in the study. Of the patients, 85% demonstrated improvement with voice therapy. The most common type of glottic closure consisted of a slit gap. Gender or age had no effect on voice therapy outcomes. Larger glottic gaps on initial stroboscopy examination and more pronounced vocal fold atrophy were weakly correlated with less improvement from voice therapy. A weak correlation was also found between the number of chronic medical conditions and poorer outcomes from voice therapy.

Conclusions:

The degree of clinician-determined improvement in vocal function from voice therapy is independent of patient age but is influenced by the degree of vocal fold atrophy, glottic closure pattern, and the patient's burden of medical problems.

Narrow-band imaging - High-definition television - Head and neck cancer

2010-04-07T01:10:00.000-07:00

Narrow-band imaging (NBI) is an endoscopic technique enhancing mucosal vasculature and better identifying superficial carcinomas due to their neo-angiogenic pattern.

NBI accuracy is increased by combination with a high-definition television (HDTV) camera.

* * *

The aim of this report was to evaluate the diagnostic improvement of NBI ± HDTV in the evaluation of head and neck squamous cell cancer (HNSCC) previously treated by chemo-radiotherapy (CHT-RT) or RT.

A total of 390 patients affected by HNSCC were prospectively evaluated by NBI and white light (WL) endoscopy ± HDTV between April 2007 and April 2009 at a single academic institution.

Among them, we focused on 59 (15%) patients who received CHT-RT or RT as part of their treatment.

Of 59 patients, 13 (22%) showed adjunctive preoperative NBI findings when compared to the standard WL examination. These findings were always confirmed by intraoperative HDTV NBI, while only eight (62%) were visible with HDTV WL. Of 13 lesions, 12 received histopathologic confirmation (from carcinoma in situ to invasive carcinoma).

The sensitivity of flexible NBI, HDTV WL, and HDTV NBI was 100, 66 and 100%, respectively.

The specificity was 98, 100, and 98%. The positive predictive value was 92, 100, and 92%. The negative predictive value was 100, 94, and 100%. The accuracy was 98, 91, and 98%. NBI ± HDTV after CHT-RT or RT was of value in detecting tumor persistence (n = 2), early recurrences (n = 6), and metachronous tumors (n = 4). By contrast, only 1 of 59 (2%) patients was found to be false positive.

Warthin tumor • adenolymphoma • fine-needle aspiration cytology •

2010-03-29T08:05:00.000-07:00

Background

Our aim was to evaluate the diagnostic accuracy of fine-needle aspiration cytology (FNAC) for Warthin tumors of the parotid gland.

Methods

All cytologic diagnoses of Warthin tumor between 1990 and 2007 were correlated with available histology. In addition, our results were compared to current literature.

Results

In 310 cases, Warthin tumor was diagnosed by FNAC. In 133 cases, (43%) both cytology and histology were available. In 127 of these 133 cases (95.5%), the diagnosis Warthin tumor was confirmed by histology. In 4 cases (3%), a benign lesion was diagnosed and 2 (1.5%) revealed a malignant lesion. On review, those cytologic diagnoses were not certain. In the literature, 11 missed malignancies (5.4%) in 202 cases were reported.

Conclusion

The diagnostic accuracy of FNAC for the diagnosis of Warthin tumor is high and the percentage of missed malignant tumors is very low. Our results imply that a cytologic diagnosis of Warthin tumor may justify conservative treatment.

Necrotizing fasciitis of the head and neck

2010-03-29T07:45:00.000-07:00

Necrotizing fasciitis of the head and neck is a rapidly progressing and life-threatening condition. The purpose of this study was to describe the patients with a focus on clinical presentation, microbiology, treatment, and prognosis.

Patients and Results

Seventeen patients (10 men; median age, 54 years) were included. Nine patients underwent minor head and neck surgery immediately prior to necrotizing fasciitis.

The typical course was
a quickly spreading erythema,
pronounced tenderness, and
severe pain.

Imaging demonstrated
diffuse swelling of the soft tissue,
poorly differentiated dilated fat layers, and
subcutaneous gas.

All patients underwent surgical debridement within 2 days, and received broad-spectrum antibiotics and hemodynamic support, hyperbaric oxygen, and immunoglobulin. All patients survived, although 12 of 17 suffered sequelae.

Conclusions

Early diagnosis is of utmost importance. Quickly spreading erythema and extreme pain in the affected area serve as red flags. With the current intensive multimodality regimen, the mortality was zero, although 70% suffered sequelae.

Occlusal treatments for temporomandibular disorders (TMDs), habitual occlusal position (HOP), bite plate-induced occlusal position (BPOP)

2010-03-29T07:42:00.000-07:00

Background

Many researchers have not accepted the use of occlusal treatments for temporomandibular disorders (TMDs).

However, a recent report described a discrepancy between the

habitual occlusal position (HOP) and the

bite plate-induced occlusal position (BPOP)

and discussed the relation of this discrepancy to TMD.

Therefore, the treatment outcome of evidence-based occlusal adjustments using the bite plate-induced occlusal position (BPOP) as a muscular reference position should be evaluated in patients with TMD.

Methods

The BPOP was defined as the position at which a patient voluntarily closed his or her mouth while sitting in an upright posture after wearing an anterior flat bite plate for 5 minutes and then removing the plate. Twenty-one patients with TMDs underwent occlusal adjustment using the BPOP. The occlusal adjustments were continued until bilateral occlusal contacts were obtained in the BPOP. The treatment outcomes were evaluated using the subjective dysfunction index (SDI) and the Helkimo Clinical Dysfunction Index (CDI) before and after the occlusal adjustments; the changes in these two indices between the first examination and a one-year follow-up examination were then analyzed. In addition, the difference between the HOP and the BPOP was three-dimensionally measured before and after the treatment.

Results

The percentage of symptom-free patients after treatment was 86% according to the SDI and 76% according to the CDI. The changes in the two indices after treatment were significant (p<0.001). The changes in the mean HOP-BPOP differences on the x-axis (mediolateral) and the y-axis (anteroposterior) were significant (p<0.05), whereas the change on the z-axis (superoinferior) was not significant (p>0.1).

Conclusion

Although the results of the present study should be confirmed in other studies, a randomized clinical trial examining occlusal adjustments using the BPOP as a reference position appears to be warranted.

Epidermal growth factor receptor

2010-03-29T07:40:00.000-07:00

Background

Cumulative evidence implicates the epidermal growth factor receptor (EGFR)
as an important therapeutic target in head and neck squamous cell carcinoma (HNSCC).

The basis for the lack of correlation between EGFR expression in the HNSCC tumor and clinical responses to EGFR inhibitors is incompletely understood.

Although a variety of mechanisms likely contribute to the effectiveness of EGFR blockade, this review focuses on the biologic implications of known EGFR variations and the role of the immune system in mediating clinical responses to EGFR inhibitors.

Methods

A Medline review of articles published in the last 10 years (1999-present) on EGFR in HNSCC was performed in combination with preliminary data from our laboratories.

Results

Studies published to date suggest no association between the expression of EGFR on HNSCC tumors and clinical responses to EGFR inhibitors. Several mechanisms have been proposed to mediate clinical response to EGFR inhibitors in HNSCC. Cumulative results from our laboratories support the role of several mechanisms, including cellular immune activation and mutated EGFR variants, in contributing to the discrepancy between level of EGFR expression and clinical response to EGFR inhibitors.

Conclusion

The efficacy of EGFR targeted therapies may be mediated, at least in part, by the immune system and the presence of the truncated EGFR variant, EGFRvIII, among other factors. Criteria to identify the subset of patients likely to be responsive to EGFR targeted therapies are needed.

Facial basal-cell carcinoma

2010-03-29T07:38:00.000-07:00

Following surgical BCC treatment,

age and tumor location in the area of the eyes, nose, lips, and ears were independent predictors of wound healing disorders.

Tumor location in the area of the eyes, nose, lips, and ears, subtype and class were independent predictors of recurrence.

Female gender and location in the area of the eyes, nose, lips, and ears were independent predictors of esthetic impairment.

Micrographic surgery and distant reconstruction technique were management-related predictors of wound healing disorders and esthetic outcome, respectively.

Conclusions

The identified negative predictors of treatment outcome should be included in the informed consent to objectify the patient's preoperative expectations.

Nevoid Basal Cell Carcinoma Syndrome (NBCCS)

2010-03-26T09:43:00.000-07:00

The Nevoid Basal Cell Carcinoma Syndrome (NBCCS) (also known as Basal Cell Nevus Syndrome, Multiple Basal Cell Carcinoma Syndrome, Gorlin syndrome, or Gorlin-Goltz syndrome) is an inherited medical condition involving defects within multiple body systems such as the skin, nervous system, eyes, endocrine system, and bones.

Although primarily being known as Nevoid Basal Cell Carcinoma Syndrome, 10% of people with the condition do not develop basal cell carcinomas (BCCs). It is now greatly known as Gorlin Syndrome, after the late Professor Robert J. Gorlin (1923-2006) whose extensive research contributed greatly to what is now known about the condition.

First described in 1960, NBCCS is an autosomal dominant condition that can cause unusual facial appearances and a predisposition for basal cell carcinoma, a malignant type of skin cancer. The prevalence is reported to be 1 case per 56,000-164,000 population. Recent work in molecular genetics has shown NBCCS to be caused by mutations in the PTCH (Patched) gene found on chromosome arm 9q. If a child inherits the defective gene from either parent, he or she will have the disorder.

Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin-Goltz syndrome, is a dominant autosomal condition with complete penetration and variable expression. It is caused by mutations to Patched (PTCH), which is a tumor suppressor gene located on chromosome 9q22,3-q31. Estimates of the prevalence of this syndrome vary from 1 in 57,000 to 1 in 164,000, depending on the country, although it is generally accepted that its prevalence is approximately 1 in 60,000 people.

Palatal Tori , Fasciocutaneous free flaps

2010-03-26T09:39:00.001-07:00

Tori and exostoses are nodular protuberances of mature bone, classified according to anatomic location. Torus palatinus (TP) is a sessile, nodular mass of bone that occurs along the midline of the hard palate. Morphologically, TP is classified as flat, spindle, nodular, and lobulated.

After oncologic resection, the palate can be reconstructed by use of fasciocutaneous free flaps, locoregional pedicled flaps, or local flaps, depending on the size and site of the defect. Although microsurgical free flaps are currently the first choice for reconstructing several head and neck defects, palate resections smaller than 8 to 10 cm can easily be restored by use of a local or locoregional flap, reducing the donor-site morbidity and lengths of surgery and hospitalization. However, the use of locoregional flaps such as a temporalis myocutaneous pedicled flap or pedicled temporoparietal fascial flap is limited by postoperative contracture, which can limit mouth opening or even lead to trismus.

Phialemonium curvatum

2010-03-26T09:37:00.001-07:00

Sphenoid sinusitis with intracranial extension produced by an emergent fungus.

This is a case of fungal sphenoid sinusitis in a diabetic patient with non-specific symptoms and bone erosion radiological findings in the superior and posterior sphenoid walls. Surgical treatment was performed by transnasal endoscopic approach and voriconazole orally thereafter. The histopathological study found fungus hyphal without mucosa invasion and the molecular study determined DNA to be Phialemonium curvatum, an unusual pathogen.

Lethal midline granuloma

2010-03-26T09:36:00.000-07:00

NK/T-cell lymphoma is a rare condition with an aggressive course and poor prognosis.

Historically known as <>, it generally appears in a midfacial location.

We describe the case of a 22-year-old woman with laryngotracheal affectation, presenting with hoarseness and hemoptysis.

CT scan and MRI showed severe laryngeal and tracheal destruction.

The biopsy showed a polymorphic, lymphoid cell infiltrate with angiocentric and angiodestructive pattern.

The immunohistochemical study confirmed the immunophenotype of the NK/T-cells: CD2+, CD56+ and cytoplasmic CD3+.

The in situ hybridization and flow cytometry findings were: EBER+, TIA-1+ and perforin+.

The patient died from complications of her disease, before undergoing oncologic treatment.

Canalithiasis and cupulolithiasis subtypes of benign paroxysmal positional vertigo (BPPV)

2010-03-26T09:34:00.000-07:00

Although computational models suggest the existence of canalithiasis and cupulolithiasis subtypes of benign paroxysmal positional vertigo (BPPV), these subtypes cannot be distinguished from each other based on characteristics of nystagmus.

Therefore, although the subtypes probably exist more information is needed from each patient than is available without invasive procedures.

Also, some patients may have clinical syndromes that include both canalithiasis and cupulolithiasis subtypes.

Objective: To determine if the parameters of nystagmus provide sufficient information to determine the subtype of nystagmus in a patient with BPPV.

Methods: Patients (n = 118) had unilateral BPPV of the posterior canal; 15 patients also had BPPV of the lateral canal. The main outcome measures were parameters of nystagmus in response to the Dix-Hallpike maneuver: latency to onset of nystagmus, maximum slow phase velocity, and maximum duration.

Results: Correlations between pairs of variables showed minimal or no relationships. Also, cluster analyses showed no significant subtypes. The contralateral eye moved significantly faster than the ipsilateral eye.

Triangular Tracheostomy

2010-03-26T09:31:00.001-07:00

Stenosis of a permanent tracheostoma after total laryngectomy lowers postoperative quality of life (QOL), and its prevention is clinically important.

Methods

From April 2003 to March 2009, the authors performed 87 permanent tracheostomies. For the purpose of prevention of tracheostomal stenosis, we had applied new technique from October 2005.

Results

The incidence of the tracheostomal stenosis was retrospectively reviewed. Until September 2005, conventional permanent tracheostomy was applied for 33 cases and tracheostomal stenosis developed in 6 cases (18.2%). On the other hand, stenosis did not develop in any of the 54 cases in which the new technique was used. The triangular method was significantly superior to the conventional method in preventing stenosis. Stomal recurrence did not develop in either technique.

Conclusion

The key point of the new technique is as follows: at the upper end of trachea, the posterior part of tracheal cartilage is preserved and the anterior edge of the tracheostoma is made much lower. The shape of the tracheostoma approximates a triangle, and the area is greater than with other methods. From our experience, this technique is safe and effective for the prevention of tracheostomal stenosis.

Tonsils and IgA nephropathy

2010-03-26T09:29:00.001-07:00

Relationship between tonsils and IgA nephropathy as well as indications of tonsillectomy. Although there are many papers about IgA nephropathy (IgAN) and tonsils, respectively, reviews about the relationship between tonsils, tonsillitis, tonsillectomy, and IgAN are limited.

In this review, we introduced the structure, development, and function of tonsils, difference of tonsils with and without IgAN, consistency of both tonsillar IgA and glomerular IgA, the effect of tonsil stimulation, tonsil infection, and tonsillectomy on IgAN showed some evidences in which tonsils were closely related to IgAN and polymeric IgA1 deposited in glomerular mesangium were at least in part of tonsillar origin.

Tonsillectomy can improve the urinary findings, keep stable renal function, improve mesangial proliferation and IgA deposit, have a favorable effect on long-tern renal survival in some IgAN patients, and do not cause significant immune deficiency and do not increase incidence of the upper respiratory tract infections, and can be used as a potentially effective treatment. The indications of tonsillectomy in patients with IgAN include mainly the deterioration of urinary findings after tonsillar infection, mild or moderate renal damage. However, tonsillectomy may not be enough and may not change the prognosis in IgAN patients with marked renal damage.

Tonsils Provocation Test

2010-03-26T09:27:00.000-07:00

There have been still many unclarified points concerning the functional mechanisms on provocation test of tonsils.

Following the ultrashort provocation test of tonsils changes of the components of leucocytes were studied.

A comparative study by monoclonal antibody method was made on the lymphocyte subsets
(OKT4,OKT8,and OKIa1)
of peripheral blood and intratonsillar tissues before and after the provocation and on those of intratonsillar tissues of the provocated tonsil on one side and the nonprovocated on the other.

The subjects were classified into three groups, namely, habitual angina, focal infection and simple hypertrophy of tonsils.

Changes of peripheral blood components except for OKIa1 in the group below 20 years revealed significant increase in habitual angina, focal infection and simple hypertrophy of the tonsil in that order.

As regards intratonsillar changes, no significant change was observed, but OKT4 values decreased in nonprovocated tonsils of adult group.

Monoclonal antibody peroxidase stain revealed that the intratonsillar distribution of OKT4 in provocated tonsil decreased, compared with the nonprovocated. I

n conclusion, significant changes of the lymphocyte subsets of peripheral blood and tonsillar tissues were observed in our studies. These immunological observations following provocation test of tonsils may be valuable for explaining the functional mechanisms of tonsillitis. The increase in lymphocytes of peripheral blood may be the most helpful indicator for tonsillectomy.

Synovitis, acne, palmoplantar pustulosis (PPP), hyperostosis, and osteitis (SAPHO)

2010-03-26T09:21:00.000-07:00

Synovitis, acne, palmoplantar pustulosis (PPP), hyperostosis, and osteitis (SAPHO) are the characteristic features of SAPHO syndrome.

A 53-year-old woman had been treated for PPP for 2 years.

She complained of hearing loss in the right ear, and otitis externa was diagnosed.

The pure-tone audiogram (PTA) indicated mild hearing loss in the right ear, and her hearing continued to deteriorate despite recovery from inflammation.

Her tympanogram was of the As-type, and acoustic reflex was absent in the right ear.

A computed tomography (CT) scan revealed bilateral normal ossicles and cochleas.

Bone scintigraphy revealed tracer uptake in the bilateral sternoclavicular joints, glenohumeral joints, and the capital humerus.

She was hospitalized for arthralgia, and the pain was controlled with steroid therapy.

Her right hearing deteriorated soon after the tapering of the steroid; her hearing recovered after cyclosporine therapy was initiated.

The first tonsil provocation test showed increased blood cells in the urine, and the second test showed exacerbation of pustulosis.

Despite immunosuppressant therapy, the arthritis attacks and hearing loss persisted; therefore, tonsillectomy was performed, which improved PPP.

However, her hearing remained unchanged after the operation.

We considered that irreversible changes might have already developed in the ossicular joints, and ossicular reconstruction was performed.

Thereafter, her hearing and earache improved.

Keywords: SAPHO syndrome, Mixed-type hearing loss, Palmoplantar pustulosis

Arteriovenous malformation (AVM)

2010-03-26T09:18:00.001-07:00

Arteriovenous malformation (AVM) is an uncommon vascular anomaly usually present in intracranial location, however may be present in other sites of the body, mostly in the head and neck region.

The lesion may be present since birth or caused by trauma, but may become obvious during puberty or pregnancy.

The diagnosis can be confirmed by selective angiography.

Treatment usually includes super selective embolization followed by surgical excision within 48h.

We hereby present a rare case of a grotesquely disfiguring congenital auricular arteriovenous malformation in a 77-year-old elderly man, which has recently been complicated by ulceration, infection and hemorrhage in the past 6 months. The pinna showed a 15cm×8cm pulsatile, erythematous, ulcerative hemorrhagic swelling which on angiography revealed tortuous superficial temporal and occipital arteries supplying the lesion. Complete auricular excision with split skin grafting was carried out. The option of preoperative embolization was not considered due to his renal dysfunction and the high dye load required for embolizing the fast flow arteriovenous malformation.

Keywords: Arteriovenous malformation, Elderly man, Pinna

Meningeal carcinomatosis (MC) , Progressive SNHL.

2010-03-26T09:17:00.000-07:00

Meningeal carcinomatosis (MC) is an uncommon form of metastasis of solid tumors.

In the absence of clinical meningeal or parenchymal involvements, the sensorineural hearing loss (SNHL) as the starting symptom of MC is very infrequent.

We report the history of two patients affected by MC who presented first with progressive SNHL. In both cases the early magnetic resonance imaging (MRI) finding mimicked bilateral masses in the cerebellopontine angle (CPA). Only the histopathologic result and surgical biopsy in cases 1 and 2, respectively, identified masses secondary to occult malignancy. However the available investigations could not discover the primary site of metastatic carcinoma. Despite the poor prognosis, because of the rarity and severity of MC we consider important to make known our experience in order to consider metastatic tumors in the differential diagnosis for sudden SNHL.

Keywords: Sudden sensorineural hearing loss, Meningeal carcinomatosis, Occult cancer

Syringoid eccrine carcinoma (SEC)

2010-03-26T09:15:00.000-07:00

First described in 1969, syringoid eccrine carcinoma (SEC) is a rare cutaneous tumor with some controversy regarding its correct definition.

It consists of solid nests and small cords in a dense fibrocollagenous stroma.

As it is rare, its clinical appearance is not well characterized and its biological behaviour is not defined.

It usually affects skin of the scalp, extremities and more rarely, other sites.

It behaves as locally aggressive tumor but metastases are rare. Although there have been some previous reports describing clinical presentation and management of SEC in the skin, there has been no previous reports describing clinical findings and management of this tumor in the external auditory canal.

We report a case of a 57-year-old female with small solitary mass in left external auditory canal associated with discharge, severe itching and bleeding on manipulation. Complete local excision is the recommended method for diagnosis and treatment of this tumor in the external auditory canal. This extremely rare case serves as a springboard for the diagnosis as well as the management of SEC in external auditory canal.

Keywords: Auditory, Eccrine, External ear, Syrigoid carcinoma

Ancient schwannoma, Neurilemmoma,Sinonasal ancient schwannoma.

2010-03-26T09:13:00.000-07:00

Schwannoma is a benign tumor arising from the sheath of myelinated nerve fibers and may occur in any part of the body.

Ancient schwannoma, a variant of schwannomas, in the head and neck region is uncommon, and its occurrence in the nasal cavity and paranasal sinuses is extremely rare with only one case reported to date.

Herein, we describe a rare case of sinonasal ancient schwannoma. The tumor arose from the nasal septum and extended to the sphenoid sinus. It was successfully treated by endoscopic sinus surgery, and the patient showed no evidence of recurrence 24 months postoperatively.

Keywords: Ancient schwannoma, Neurilemmoma, Nasal septum, Endoscopic sinus surgery

Carotid artery disease (CAD)

2010-03-22T10:15:00.001-07:00

Heart disease is common, affecting millions of adults and causing 26 percent of deaths annually. Yet there’s another silent killer lurking nearby that often goes unnoticed and untreated until it’s too late.

What is CAD?

Carotid artery disease (CAD) is a serious vascular condition that shows no symptoms in 70 percent of individuals evaluated. It is also a leading cause of strokes.

The carotid arteries, the main source of blood flow to the brain and face, are located on each side of the neck and extend from the aorta in the chest to the base of the skull. A healthy artery is open and allows the blood to flow to the brain efficiently. Blood flow can become partially or totally blocked by fatty material called plaque. As more plaque forms, the artery becomes narrower and the walls become irregular, which can cause blood clots to form on the plaque.

“Carotid artery disease is prevalent in many people, especially those who also have peripheral vascular or coronary heart disease,” says Luis Sanchez, MD, Washington University vascular surgeon at Barnes-Jewish Hospital. “If someone has signs of another vascular condition, doctors often look for CAD to prevent more serious conditions.”

Carotid Artery Stenosis

A partial blockage or narrowing of the artery is called carotid artery stenosis. Blockage can reduce the blood supply to the brain and lead to a stroke. A stroke can also result from an embolism, a blockage caused when a clot breaks loose and travels to the brain. If a small blood vessel is blocked, this causes a mini-stroke (transient ischemic attack, or TIA) and is often a warning sign that a larger stroke may soon occur.

As the degree of narrowing increases, so do the risks of stroke and embolization. Patients with high blood pressure, diabetes and high cholesterol are at risk, as are patients who are overweight or smoke.

“There is often a noise in the neck on a routine test or something small that makes us then test for CAD,” Sanchez says. “If symptoms do arise, patients may have a small mini-stroke due to a carotid lesion. A TIA patient can have speaking difficulties, numbness in the extremities, weakness or vision problems. Any or all of the symptoms can either last just a few seconds or repeatedly for many minutes, so some patients don’t realize the seriousness.”

How CAD Stenosis is Treated

Not all patients need surgery right away. Drug therapies are often the first line of defense. More serious treatment for carotid stenosis is needed in patients who have had a prior stroke, prior TIA, or severely blocked carotid arteries. Once the diagnosis of significant carotid stenosis is made, meaning there is a 70 percent blockage of the artery, the most appropriate therapy is vascular surgery. This can be one of two procedures, the more common carotid endarterectomy or the newer carotid artery stenting.

Washington University heart and vascular surgeons offer both options with great success.

Carotid Angioplasty and Stenting (CAS)

In the past 15 years, surgeons have developed a minimally invasive stenting procedure to combat severe CAD. In the procedure, the surgeon introduces a needle into the artery in the groin after injecting numbing medicine. A catheter, a flexible plastic tube, is inserted into the artery and carefully guided up to the neck where the carotid artery blockage is located. Live X-rays, called fluoroscopy, are taken to see the artery during the procedure.

The surgeon then passes a guidewire through the catheter to the blockage. Once a filter is placed above the blockage to prevent pieces of plaque from breaking off and causing a stroke, a small balloon at the end of the wire is then inflated and pressed against the inside walls of the artery to open the artery and allow better blood flow to the brain.

Finally, a wire mesh stent is placed across the blocked area. The stent is inserted to help keep the artery open after balloon treatment.

Because the procedure is still new, stenting is more commonly preformed when patients are at high risk, are symptomatic, or have had previous surgeries in the area. It is beneficial, however, because patients are treated under local anesthesia while awake. Staying awake allows the surgeon to monitor the brain’s reaction to decreased blood flow. Patients are usually treated on an outpatient basis and only held overnight for surveillance on the medication.

Stenting Best Option for Severe Cases

“Outcomes at Barnes-Jewish are the same as those with the more traditional surgery, and it is the best option for the most severe cases,” says Gregorio Sicard, MD, vice chair for the department of surgery.

“Stenting can be better for patients because there are no pulmonary complications and it puts less stress on the body overall,”

Bichat's buccal fat pad for reconstruction of posterior oral cavity defects.

2010-03-22T10:11:00.000-07:00

Some losses of matter in the posterior area of the oral cavity require reconstruction with vascularized tissue to prevent retraction and a limited mouth opening.

Bichat's fat pad or Bichat's fat ball is rarely used for oral mucosal reconstruction despite its proximity, easy availability, plasticity and simple use as a flap. Bichat's fat pad is resistant to lipolysis even in case of major weight-loss.

It usually herniates in the exeresis site of a jugal tumor or intermaxillary commissure. When this is not the case, it can be obtained by a horizontal vestibular incision.

The fat pad is unfolded and sutured without tension to the margin of the defect. The flap may be protected by a Bourdonnet dressing.

Epithelialization begins 8 to 10 days after surgery.

It is completed in the second or third week. The fat volume is variable, thus filling can only be applied to areas with loss of matter under 6cm.

The technique is simple and quick.

Postoperative evolution is usually uneventful; healing is quick as well as recovery of normal feeding function.

Acute idiopathic sensorineural hearing impairment at a frequency exceeding 8 kHz

2010-03-22T10:09:00.000-07:00

Acute idiopathic sensorineural hearing impairment at a frequency exceeding 8 kHz (high-frequency range) was recognized in patients suffering from acute tinnitus without hearing loss.

The cases in this study may contribute to clarifying the pathogenesis of tinnitus without hearing loss and evaluating its response to steroid therapy. Objectives. The aim of this study was to demonstrate the existence of acute idiopathic sensorineural hearing impairment only in the high-frequency range and to investigate its relation to tinnitus without hearing loss.

Methods. Five patients aged 29 years or younger who consulted a hospital within a few days after the onset of unilateral tinnitus without hearing loss were studied. We conducted audiometry involving the high-frequency range on first medical examination and on improvement in tinnitus, and investigated the association between the hearing findings in the high-frequency range and a tinnitus prognosis.

Results. All five patients showed abnormalities in the threshold in the high-frequency range on the affected side. In the three cases given prednisolone, tinnitus and the threshold abnormalities were reduced within 20 days. In contrast, tinnitus and the threshold abnormalities showed little change in two patients who were not treated.

PHR1 • PLEKHB1 • olfaction • maturity • pleckstrin homology • sensory neurons

2010-03-21T03:23:00.000-07:00

ABSTRACT

Objectives/Hypothesis.

To characterize the role of Phr1, a gene highly expressed in primary sensory neurons where it encodes an integral membrane protein with an N-terminal pleckstrin homology domain and a C-terminal transmembrane domain, in the olfactory system.

Methods.

We studied the immunelocalization of the PHR1 protein in mouse olfactory epithelium both at steady state and during regeneration following methyl bromide (MeBr) exposure using scanning confocal microscopy. Additionally, we examined the electrophysiologic role of Phr1 in olfaction and short-term olfactory adaptation.

Results.

We found that PHR1 is abundantly and specifically expressed in olfactory neurons. It is widely distributed in punctate, vesiculated organelles throughout the cell bodies, axons, and glomeruli of primary olfactory neurons but is specifically excluded from the olfactory cilia. In the regenerating olfactory epithelium, PHR1 expression appears at 14 days following MeBr ablation coinciding with the onset of olfactory neuron maturity. Despite the abundant and specific expression throughout the olfactory neurons, mice lacking Phr1 did not exhibit differences in the distribution of the components of olfactory signal transduction system, the rate of olfactory regeneration following MeBr exposure, olfactory function, or short-term adaptation to odors.

Conclusions.

Phr1 is widely and abundantly expressed throughout mature olfactory neurons and other primary sensory neurons, but its absence does not appear to affect olfactory morphology, regeneration, sensory function, or adaptation. The exact function of Phr1 remains to be discovered.

Synkinesis • facial nerve • misdirection • regeneration • facial nucleus • fluorescent retrograde tracer • Level of Evidence: 2a.

2010-03-21T03:22:00.000-07:00

Objectives/Hypothesis:

The anatomical configuration of the facial nerve differs greatly between the intratemporal and extratemporal portions. The purpose of this study was to investigate the incidence of facial synkinesis and misdirection on clamping the facial nerve at the intratemporal or extratemporal portion of the facial nerve in guinea pigs.

Study Design:

Experimental study.

Methods:

In 16 guinea pigs, the facial nerve was clamped with microsurgical needle forceps at either the extratemporal (group A) or intratemporal (group B) segment. Facial nerve function was evaluated 1 week postoperatively using electroneurography (ENoG), and the incidence of facial synkinesis was evaluated 15 weeks postoperatively using an evoked blink reflex test. Fifteen weeks postoperatively, two retrograde fluorescent tracers (Dil [1-1-dioctodecyl-3,3,3,3-tetramethyl-indocarbocyanine perchlorate] and True Blue) were injected into the facial muscles to observe reorganization of the facial nucleus.

Results:

No significant difference in the ENoG threshold was observed between groups A and B. In group A, none of the animals developed facial synkinesis and the somatotopic organization of the facial nucleus was not disturbed. In contrast, synkinesis occurred and the somatotopic organization was disturbed in group B.

Conclusions:

A lack of funicular structure within the intratemporal facial nerve increases the possibility of misdirected regenerating axons and synkinesis.

Langerhans cell histiocytosis (LCH)

2010-03-21T03:20:00.000-07:00

Langerhans cell histiocytosis (LCH) most commonly occurs as a localized solitary bone lesion and appears predominantly in pediatric patients.

LCH is characterized by the proliferation and accumulation of Langerhans cells which may cause pain and adjacent soft-tissue swelling.

This disease is of clinical importance to dental professionals because LCH commonly involves the oral and maxillofacial region and early symptoms can be manifested in the jaw and, when overlooked, may result in extensive destruction of the involved structures. Considering the fact that this is a relatively rare entity, close investigation of the cases that are encountered are warranted. Herein we report a case of LCH occurring in the left condyle of an 11-year-old boy that initially showed clinical symptoms mimicking an abscess of the temporomandibular joint. The clinical and radiographic features, differential diagnosis, treatment, and long-term follow-up of this patient with indomethacin are presented.

Benign lymphoepithelial lesions of the parotid

2010-03-21T03:19:00.000-07:00

Background

Benign lymphoepithelial lesions of the parotid include a spectrum of disorders ranging from lymphoepithelial sialadenitis (LESA) of Sjögren syndrome to lymphoepithelial cysts (LEC) and both human immunodeficiency virus (HIV)–related and –unrelated cystic lymphoid hyperplasia (CLH). They share a common microscopic appearance characterized by epimyoepithelial islands and/or epithelial lined cysts in a lymphoid stroma. However, they differ greatly regarding their etiology, clinical presentation, and management.

Objective

The purpose of this study was to establish specific immunophenotypic profiles for these diverse disease entities.

Study design

Four cases of HIV+ CLH, 5 cases of HIV− CLH, 3 cases of LESA of Sjögren syndrome, and 3 cases of sporadic LEC were quantitatively analyzed for distribution of lymphoreticular cell subpopulations, using antibodies against CD20, CD45RO, CD4, CD8, CD57, and CD68.

Results

The cystic lesions in both the HIV+ and HIV− cases were microscopically analogous. However, a marked decrease in the interfollicular CD4:CD8 ratio was observed in all HIV+ CLH cases, which was statistically significant when compared with the HIV− cases (P = .02) and cases of LESA of Sjögren syndrome (P = .03). No significant differences regarding the distribution of CD20+ B lymphocytes in epithelial cyst lining or the interfollicular or follicular distribution of CD20+, CD45RO+, CD57+, and CD68+ cells were present among the different groups.

Conclusion

Analysis of the interfollicular CD4:CD8 ratio may offer a simple immunophenotypic approach in the distinction of HIV+ from other lymphoepithelial lesions of the parotid gland, when HIV status is unknown and p24 immunohistochemistry is not readily available.

Odontogenic cysts include dentigerous cysts, periapical or radicular cysts, and the keratocysts—nowadays declared as keratocystic odontogenic tumor.

2010-03-21T03:18:00.000-07:00

Malignant tumors in the oral cavity are relatively rare.
About 5% of all malignant growths in the body are localized in the oral cavity.
The vast majority of oral malignancies are primary tumors with squamous cell carcinoma being the most frequent and sarcomas occurring very seldom.
Secondary tumors caused by hematogenous spread arising from a tumor localized elsewhere in the body are extremely rare.
About 1% of all oral cancers are metastases to the jawbones and the surrounding soft tissues.

Metastases to the jaws are mainly caused by malignant tumors of the breast, lung, kidney, bone, and colon.

They occur in the late state of the disease and are regularly detected by staging examinations including scintigraphy.

Even more rare are metastases into odontogenic cysts. Odontogenic cysts include dentigerous cysts, periapical or radicular cysts, and the keratocysts—nowadays declared as keratocystic odontogenic tumor.

The incidence of odontogenic cysts is about 10% to 15% of all oral biopsies and therefore general dentists are frequently faced with these types of lesions. The aim of this study was to review the literature regarding metastasis into odontogenic cysts and to further highlight this rare entity with the help of a clinical case.

Alveolar soft-part sarcoma (ASPS

2010-03-21T03:16:00.001-07:00

Alveolar soft-part sarcoma (ASPS) is a rare malignant neoplasm with uncertain histogenesis and with a distinctive morphology.

It has been described in the oral cavity, but this is the first report of ASPS metastasizing to the maxillary tuber region.

A 27-year-old male patient, who was under chemotherapy treatment for ASPS of the thigh, presented in our dental clinic with a painless and pedunculated nodule on the right tuber maxillae. The nodule was erythematous with smooth and lobular surface, measuring 3 cm in maximum diameter. An incisional biopsy was performed and the diagnosis of metastatic ASPS was made. Histologically, the tumor was characterized by a proliferation of polyhedral cells in pseudoalveolar pattern. Tumor cells were large, showing granular cytoplasm, periodic acid–Schiff positive diastase-resistant intracytoplasmic material, and vesicular nuclei with prominent nucleoli. Unfortunately, the patient died 2 months after the diagnosis of the oral metastasis. Metastases of ASPS to the mouth are very rare and indicate a poor prognosis.

Metastatic clear cell renal cell carcinoma (CCRCC) should be considered in differential diagnosis of intraoral clear cell tumors, including mucoepidermoid carcinoma (MEC).

2010-03-21T03:15:00.000-07:00

Objective and study design

We compared the clinical, histologic, histochemical, and immunohistochemical characteristics of 9 oral metastatic CCRCCs and 8 intraoral clear cell MECs.

Results

Oral metastatic CCRCC affected salivary-gland containing tissues in 7 cases (78%). Microscopically, oral metastasis revealed a proliferation of neoplastic clear cells arranged in an alveolar pattern with central blood vessels, features that were not seen in any intraoral clear cell MEC. Mucicarmine staining was positive only in clear cell MEC. Immunohistochemistry showed similarities in cytokeratin expression; vimentin and CD10 were expressed in all oral metastatic CCRCCs but in only 1 clear cell MEC each.

Conclusions

Besides clinical history, the alveolar pattern, vessel distribution, absence of mucicarmine staining, and vimentin and CD10 immunoexpression are useful in histologic differential diagnosis of CCRCC and clear cell MEC.

Combined neuroendocrine carcinoma with squamous cell carcinoma, occurring at the buccal gingiva

2010-03-21T03:14:00.000-07:00

Neuroendocrine carcinoma is a rare neoplasm that occurs widely in various organs and tissues.

The biological behavior of this tumor in the oral region remains poorly understood.

We encountered an extremely rare case of combined neuroendocrine carcinoma with squamous cell carcinoma, occurring at the buccal gingiva in a 62-year-old woman.

Left partial maxillectomy was performed. Histological examinations revealed solid nests with extensive necrosis and nuclear palisading at the periphery.

The tumor also showed areas of stratified neoplastic squamous differentiation. Immunohistochemically, tumor nests stained positive for synaptophysin, chromogranin, N-CAM (CD56), and neuron-specific enolase.

Strong positivity was seen for K14 and K17 in the squamous component and for K7 in the neuroendocrine component.

Both components showed K19 staining. Cells with squamous differentiation and K14 staining occasionally expressed p63. The patient showed no evidence of disease as of 23 months postoperatively. Given the aggressive characteristics of neuroendocrine carcinoma, strict follow-up has been performed.

The calcifying odontogenic cyst (COC), The odontogenic keratocyst (OKC)

2010-03-21T03:12:00.000-07:00

The calcifying odontogenic cyst (COC), first identified as a separate and distinct lesion by Gorlin et al. in 1962, is an uncommon benign lesion, consisting of a proliferation of odontogenic epithelium and scattered nests of ghost cells and calcifications that may form the lining of a cyst or present as a solid mass.

The COC occurs alone or occasionally with odontomas or other odontogenic tumors, and it is this variable histology and clinical behavior that has raised the question of whether or not it is a cyst or a true neoplasm.

The odontogenic keratocyst (OKC) is a locally aggressive odontogenic cyst lined by parakeratinizing epithelium that also exhibits characteristics of a neoplasm, including rapid growth, a high rate of recurrence when treated conservatively, and the presence of a gene mutation.

We describe a patient diagnosed with an OKC of the mandible that arose simultaneously with a COC of the anterior maxilla.

The occurrence of 2 “cystic neoplasms” in the same patient is an occurrence which to our knowledge has not been previously reported.

Barodontalgia

2010-03-21T03:10:00.000-07:00

This article reviews the current knowledge regarding barodontalgia, a barometric pressure–related oral (dental and other) pain. Contemporary classification, prevalence, and incidence, features, etiology, and diagnosis of this entity are presented regarding flight and diving conditions. Summarizing the past decade data, three-fourths of episodes were described as severe, sharp, and localized pain. Barodontalgia affects 11.9% of divers and 11.0% of military aircrews with a rate of 5 episodes/1,000 flight-years. Upper and lower dentitions were affected equally in flight, but more upper than lower dentition were affected in diving. The most prevalent etiologic pathologies for in-flight dental pain were faulty dental restorations (including dental barotrauma) and dental caries without pulp involvement (29.2%), necrotic pulp/periapical inflammation (27.8%), vital pulp pathology (13.9%), recent dental treatment (11.1%), and barosinusitis (9.7%). This review refutes 3 generally accepted conventions: According to the results, the current in-flight barodontalgia incidence is similar to the incidence in the first half of the 20th century, the weighted incidence of barodontalgia among aircrews are similar to the weighted incidence among divers, and the role of facial barotrauma in the etiology of in-flight barodontalgia is minor.

Matrix metalloproteinases likely play a significant role in growth of the cricoid cartilage such that their inhibition leads to marked changes in the shape of the ring.

2010-03-21T03:08:00.001-07:00

Objective

Luminal expansion of the cricoid cartilage appears to be stunted by loss of luminal epithelium (LE) and can be enhanced by transforming growth factor-β3 (TGF-β3). When both the LE and perichondrium are disrupted, matrix metalloproteinase (MMP) levels within adjacent chondrocytes are diminished but can be restored by exogenous TGF-β3. Cricoid growth stunting and luminal expansion that occur in the absence and presence of MMP activity, respectively, suggest that MMPs play an important role in normal subglottal development. The study objective was to determine if MMP inhibition affects cricoid expansion and by what mechanism, which will in turn help to define the mechanism of action of TGF-β3-induced luminal expansion.

Study Design

Ex vivo, in vitro whole organ culture of subglottises grown with and without the presence of an MMP inhibitor.

Setting

Tertiary care facility.

Subjects and Methods

Subglottises from 20 neonatal mice were divided into 10 grown with an MMP inhibitor, GM6001, and 10 grown in basic medium alone. The luminal cross-sectional area, apoptosis levels, cell proliferation rates, and presence or absence of cleaved aggrecan fragments were determined.

Results

Subglottises that were exposed to the MMP inhibitor displayed statistically significant luminal narrowing, accompanied by apparent circumferential thickening of the cricoid ring, relatively decreased apoptosis, increased chondrocyte proliferation, and decreased amounts of aggrecan cleavage fragments in the extracellular matrix.

High jugular bulb causing tinnitus.

2010-03-21T03:06:00.001-07:00

Surgical reconstruction of the middle ear floor under local anesthesia offers valuable treatment for patients with incapacitating tinnitus due to dehiscent middle ear floor.

However, the risk of sigmoid sinus thrombosis should be considered.

To our knowledge, this is the first trial of multilayer reconstruction of the middle ear floor dehiscence to manage high jugular bulb causing tinnitus.

H1N1 influenza A presenting as bacterial tracheitis

2010-03-21T03:04:00.001-07:00

Cases of bacterial tracheitis (BT) occurring early in the 2009 flu season have been isolated in conjunction with the H1N1 strain of influenza A (H1N1). No previous H1N1 cases have presented as BT in the literature to date. We would like to discuss viral coinfection in BT patients and how this new strain may affect the rate and type of presentation encountered. The life-threatening potential of BT and the pandemic proportion of H1N1 highlight a possibly dangerous combination that should be recognized by the otolaryngology community. In hospitalized patients with presumed BT, consideration should be given to routine H1N1 testing and the addition of antiviral medication when indicated as this entity is further investigated

Guillain-Barre syndrome presenting as epiglottitis in a child

2010-03-21T03:03:00.000-07:00

A previously healthy five-year-old boy presented to the emergency department with respiratory distress and drooling. His medical history was unremarkable except for symptoms of an upper respiratory tract infection five days earlier. Upon arrival at the emergency room, the child was lethargic, leaning forward, and in severe distress. Stretch reflexes were absent in the upper and lower extremities. His temperature was normal. The child was immediately taken to the operating room for emergency intubation with a presumptive diagnosis of epiglottitis. Laryngoscopy and bronchoscopy revealed a large pool of secretions in the postcricoid area and pyriforms, bilateral vocal cord paralysis, and tracheobronchial aspiration. Postoperatively, an electromyography (EMG) showed no motor or sensory respons

Renal tubular acidosis (dRTA), hearing loss (HL), and enlargement of the vestibular aqueduct (EVA).

2010-03-21T03:00:00.000-07:00

Novel ATP6V1B1 mutations were found in a patient with
distal renal tubular acidosis (dRTA),
hearing loss (HL), and
enlargement of the vestibular aqueduct (EVA).

The deterioration of HL and vertiginous attacks may be associated with the disruption of the endolymph pH homeostasis.

Objectives:
To study the audiovestibular functions and to identify the causative gene.

Methods:
This study enrolled a Japanese family, where the proband showed type 1 dRTA, early onset HL, and bilateral EVA. A deterioration of HL occurred several times in both ears. Vertiginous attacks were always associated with a deterioration of HL. Audiovestibular examinations included distortion product otoacoustic emissions (DPOAEs), auditory brainstem responses (ABRs), caloric testing, and vestibular evoked myogenic potentials (VEMPs). Direct sequencing was utilized to screen for ATP6V1B1, SLC26A4, and GJB2 mutations.

Results:
The findings of DPOAEs and ABRs indicated cochlear HL. The vestibular function was thought to be mildly impaired according to the caloric responses and VEMP findings. Two novel ATP6V1B1 mutations of a heterozygous 15 base-pair deletion (c.756_770del) in exon 7 and a heterozygous 1 base-pair insertion (c.1242_1243insC) in exon 12 were detected in a compound heterozygous state. No mutation was identified in either SLC26A4 or GJB2.

CRP and Rhinisinusitis

2010-03-21T02:58:00.000-07:00

C-reactive protein (CRP) levels may predict the extent of acute rhinosinusitis disease in the computed tomography (CT) scans, as well as the specific symptom severity.

High levels may direct the physician to change the treatment.

To establish tools to define 'high risk' patients suffering from acute rhinosinusitis.

Methods: Patients suffering from severe unresponsive acute rhinosinusitis filled in health-related quality of life questionnaires and rated their symptoms. Blood tests and CT scans were performed. We examined the value of imaging and inflammatory markers, especially CRP, as predictors of disease severity, defined by subjective and objective means; need for surgery; and occurrence of ocular complications.

Results: Thirty-two patients were prospectively recruited. A significant association was found between CRP levels, imaging scores, and symptoms severity. Neither ocular complications nor the need for surgery were present in the group with low CRP level.

Concentrations of IL-5, IL-6, and IL-10 in nasal secretions

2010-03-16T01:31:00.000-07:00

Concentrations of IL-5, IL-6, andIL-10 in nasal secretions, vURTIs diagnosed by polymerase chainreaction (PCR) detection of upper respiratory tract viruses,and concurrent CLIs diagnosed by parents.

Of 1269 secretion samples, 552 (43.5%) were collectedduring a vURTI (PCR findings positive for an assayed virus [PCR⁺]).A concurrent CLI was diagnosed for 34% of the PCR⁺ samples andfor 18% of the samples found to be negative by PCR analysis(PCR^–). Cytokine concentrations and ratios were highlyvariable and skewed to the lower values. The significance ofthe cytokine concentrations and ratios as discriminators ofgroups defined by the presence or absence of virus and of subgroupsdefined by the presence or absence of a CLI was evaluated usingreceiver operating characteristic curves. All measures weresignificant discriminators of the PCR⁺ vs PCR^– groups,and most were significant discriminators of the paired CLI subgroups.The concentration of IL-6 and the IL-5/IL-6 ratio were the bestdiscriminators across all groups and subgroups. However, thesensitivities and specificities of those discriminators at thebest cutoff values were on the order of 0.7 for the most extremepairwise comparison (PCR⁺CLI⁺ vs PCR^–CLI^–) and lowerfor the other comparison groups.
Conclusion The low sensitivities and specificities forcytokine-based assignment of specimens to the paired groupsand subgroups limit their usefulness for diagnosis of infectionor illness.

Facial Infiltrating Lipomatosis, Multiple endocrine neoplasia type 3, Bannayan-Riley-Ruvalcaba syndrome, Cowden syndrome

2010-03-16T01:29:00.000-07:00

Facial infiltrating lipomatosis is a rare congenital disorder in which mature lipocytes invade adjacent tissue.
The phenotypic features include soft-tissue and skeletal hypertrophy, premature dental eruption, and regional macrodontia.
There is a high risk for regrowth after resection that is, perforce, subtotal.
The etiology, natural history, optimal management, and relationship to other disorders of fatty overgrowth are unclear.
In this study, the clinical features, radiographic findings, histopathology, and postoperative results were analyzed in 13 patients with facial infiltrating lipomatosis.

The condition was diagnosed in infancy (eight male subjects, five female subjects) and characterized by
enlargement of the cheek (n = 12) or chin (n = 1).
Other findings included
cutaneous capillary blush (n = 9),
ipsilateral macroglossia (n = 8), and
mucosal neuromas (n = 6).
Most patients had
early eruption of ipsilateral deciduous and permanent teeth (n = 12).

Computed tomography and magnetic resonance imaging showed an infiltrated soft-tissue mass of fatty density (n = 13) and skeletal overgrowth (n = 9).

Multiple resection was performed on six patients (mean number of operations per patient, 2.5; range, one to six operations);
regrowth and/or worsening of the capillary stain occurred in all six patients.

Because surgical removal of the mass is usually unsuccessful, specific management of this condition will require insight into its etiopathogenesis.
Given the presence of mucosal neuromas and lipomatosis, this study included testing for the known mutations in three entities that are associated with these soft-tissue findings (Cowden syndrome, Bannayan-Riley-Ruvalcava syndrome, and multiple endocrine neoplasia type 2B). Results of DNA analyses for these germline mutations were negative. It is more likely that this disorder is caused by a somatic mutation involving a local increase in growth factor(s).

Multiple endocrine neoplasia type 3 (also known as "Mucosal neuromata with endocrine tumors," "Multiple endocrine neoplasia type 2B," "Multiple mucosal neuroma syndrome," and "Wagenmann–Froboese syndrome") is a genetic disease that causes multiple tumors on the mouth, eyes, and endocrine glands. It is the most severe type of multiple endocrine neoplasia,differentiated by the presence of oral and submucosal tumors in addition to endocrine tumors. It was first described by Wagenmann in 1922.

MEN 2B typically manifests before a child is 10 years old. Affected individuals tend to be tall and lanky, with a elongated face and protruding, blubbery lips. Benign tumors (neoplasms) develop in the mouth, eyes, and submucosa of almost all organs in the first decade of life,^[4]followed by adrenal and thyroid tumors after puberty. Medullary thyroid cancer almost always occurs, and cancer of the adrenal glands (pheochromocytoma) occurs in 50% of cases.

A variety of eponyms have been proposed for MEN 2B, such as Williams-Pollock syndrome, Gorlin-Vickers syndrome, and Wagenmann-Froboese syndrome. However, none ever gained sufficient traction to merit continued use, and are no longer used in the medical literature.

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a rare hamartomatous disorder with occurrence of multiple subcutaneous lipomas,macrocephaly and hemangiomas.^[1]^[2] The disease is inherited in an autosomal dominant form, but sporadic cases have been reported. The disease belongs to a family of hamartomatous polyposis syndromes, which also includes Peutz-Jeghers syndrome, juvenile polyposis andCowden syndrome.

Presentation

Most lesions are slowly growing and easily resectable. Visceral as well as intracranial involvement may occur in rare cases, and can cause bleeding and symptomatic mechanical compression, especially of the spinal cord or spinal nerve roots. This may require surgical resection.

The macroencephaly is symmetrical, and does not cause widening of the ventricles or raised ICP (intracerebral pressure).

Etymology

The syndrome combines Bannayan-Zonana syndrome, Riley-Smith syndrome, and Ruvalcaba-Myrhe-Smith syndrome.^[3] Bannayan-Zonana syndrome is named for George A. Bannayanand Jonathan Zonana.^[4]^[5]^[6] Riley-Smith syndrome was named for Harris D. Riley, Jr. and William R. Smith.^[7] Ruvalcaba-Myrhe-Smith syndrome is named for S. Myhre, Rogelio H. A. Ruvalcaba and David Weyhe Smith.^[8]

Cowden syndrome (also known as "Cowden's disease," and "Multiple hamartoma syndrome"^[1]^:673) is a rare autosomal dominant inherited disorder characterized by multiple tumor-like growths called hamartomas and an increased risk of certain forms of cancer.^[2]

Signs and Symptoms

Multiple white, smooth papules spread over the lower lip

The hamartomas are small, noncancerous growths that are most commonly found on the skin and mucous membranes (such as the lining of the mouth and nose), but can also occur in the intestinal tract and other parts of the body. They are largely benign. However, people with Cowden syndrome have an increased risk of developing several types of cancer, including cancers of the breast, thyroid, and uterus. Women with Cowden syndrome have as much as a 25-50% lifetime risk of developing breast cancer^[3] and up to 75% have benign breast conditions such as ductal hyperplasia, intraductal papillomatosis, adenosis, lobular atrophy, fibroadenomas, and fibrocystic changes.^[4] Nonmedullary thyroid cancer develops in up to 10 percent of affected individuals.^[2] In addition, over one-half of those affected have follicular adenomas or multinodular goiter of the thyroid. Other malignancies that appear to be associated with Cowden and Cowden-like syndrome include endometrial and renal cancers.^[5] Other signs and symptoms of Cowden syndrome can include an enlarged head, a rare noncancerous brain tumor calledLhermitte-Duclos disease, and glycogenic acanthosis of the oesophagus.^[6] The majority of affected individuals develop the characteristic skin lesions by age 20.

History

Cowden syndrome was first described in 1963 by Lloyd & Dennis. They named the condition after the surname of the patient.^[7]

^Epidemiology

Because Cowden syndrome can be difficult to diagnose, the exact prevalence is unknown; however, it probably occurs in at least 1 in 200,000 people.

Genetics

Mutations in the PTEN gene cause Cowden syndrome. PTEN is a tumor suppressor gene, which means it helps control the growth and division of cells. Inherited mutations in the PTEN gene have been found in about 80 percent of people with Cowden syndrome. These mutations prevent the PTEN protein from effectively regulating cell survival and division, which can lead to the formation of tumors. Cowden syndrome is one of several inherited diseases caused by mutations in the PTEN gene.

In the other 20 percent of Cowden syndrome cases, the cause is not yet known. Some of these cases may be caused by mutations in a region of DNA that regulates the activity of the PTEN gene. Others may have mutations in certain subunits of succinate dehydrogenase,^[8] a mitochondrial enzyme.

This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In some cases, an affected person inherits the mutation from one affected parent. Other cases may result from new ("de novo") mutations in the gene. These cases occur in people with no history of the disorder in their family. It is characterized by numerous hamartomas, among other symptoms.

Treatment

Patients are usually managed by a multidisciplinary team including surgeons, gynecologists, and dermatologists because of the complex nature of this disorder. Follow-up for the increased risk of breast cancer risk includes monthly breast self-examination, annual breast examination, and mammography at age 30 or five years earlier than the youngest age of breast cancer in the family.^[2] The magnitude of the risk of breast cancer justifies routine screening with breast MRI as per published guidelines.^[9]

^Gallery

Additional lesions were found on the tongue

Lesions on the maxillary gingiva (palate)

Papilliferous oral mucosa with mild epithelial hyperplasia, acanthosis, hyperkeratosis and inflammatory infiltrate (H&E, x20)

Narrowband Imaging for Early Detection of Malignant Tumors and Radiation Effect After Treatment of Head and Neck Cancer

2010-03-16T01:14:00.000-07:00

NBI-assisted endoscopy is highlyuseful for the detection of precancerous lesions in the oropharyngealand hypopharyngeal mucosa and is not affected by a history ofradiotherapy in patients with HNSCC.

Head and neck squamous cell carcinoma of the upper aerodigestive tract is well known for its frequently late presentation and diagnosis at an advanced stage. In addition, it is well recognized that it may arise in multiple sites, either synchronously or metachronously. Thus it should be imperative to endoscopically screen the upper aerodigestive tract of patients at risk for head and neck squamous cell carcinoma with a new diagnostic tool, especially due to the fact that early lesions are very difficult to detect even by multiple passes with a standard endoscopy, if they are ≤ 1 cm in diameter. Lugol chromoendoscopy, which is mainly used in the oesophagus, is not suitable for the head and neck region due to severe mucosal irritation. Herein, narrow-band imaging is described, a diagnostic tool already proved as a useful screening method in other endoscopic fields, and its application in the early detection of head and neck squamous cell carcinoma is reviewed, as reported by previous studies in the otolaryngologic literature. Narrow-band imaging relies on the principle of depth of penetration of light, with the narrow-band blue light having a short wavelength (415 nm) penetrating into the mucosa and highlighting the superficial vasculature. Furthermore, the blue filter is designed to correspond to the peak absorption spectrum of haemoglobin to enhance the image of capillary vessels on surface mucosa. Thus, superficial mucosal lesions that would be missed by regular white light endoscopy, are identified, in view of their neoangiogenetic pattern of vasculature, using the blue light of the narrow-band imaging. Narrow-band imaging has been used extensively in the lower aerodigestive system, yet there are only 2 reports of applications in the region of the head and neck, specifically the oropharynx and the hypopharynx. However, these are not the only sites that can benefit from narrow-band imaging. Herewith, the uses and importance are highlighted of narrow-band imaging as a future diagnostic tool in otolaryngology, in the pre-, intra- and post-operative settings.

Keywords: Upper aero-digestive tract, Malignant tumours, Diagnosis, Endoscopy, Narrow-band imaging

Introduction

Head and neck squamous cell carcinoma (HNSCC) of the upper aerodigestive tract (UADT) is well known for its frequently late presentation and diagnosis at an advanced stage ¹. This is mainly due to the complex head and neck anatomy and the presence of relatively silent areas. In addition, it is well recognized that HNSCCs may arise in multiple sites, either synchronously or metachronously ². This worsens the prognosis and makes it more difficult to treat the patient without risking detrimental effects on speech and swallowing. Thus it should be imperative to endoscopically screen the UADT of patients at risk for HNSCC (i.e., those to be treated for an already recognized primary tumour and those previously managed and undergoing follow-up), in order to detect either synchronous or second malignancies at an early stage. Moreover, endoscopic screening of cancer-free patients, at high risk due to excessive alcohol consumption and smoking, would be of great value to detect any HNSCC as early as possible.

The best case scenario for early detection of SCC of the UADT would be at the stage of squamous dysplasia or even carcinoma in situ (CIS). These early lesions are very difficult to detect even with multiple passes with the endoscope, if they are ≤ 1 cm in diameter ². A well established technique, already in use for the oesophagus, is Lugol chromoendoscopy, where squamous dysplasia and CIS appear as Lugol voiding lesions (LVL) ³ ⁴. These precancerous lesions can be easily resected via endoscopic mucosal resection (EMR), which is a minimally invasive procedure with short hospital stay. However, this technique cannot be applied in the head and neck region due to the severe mucosal irritation that may lead to severe dysphagia and even aspiration. Watanabe et al. ³ even reported that they needed to put their patients, for whom they used Lugol chromoendoscopy on the UADT, under general anaesthesia, which defeats the purpose of a screening method. It would be of great value if a new technique could be used to somehow enhance the endoscopic view of the UADT to reveal these small mucosal lesions that would otherwise escape diagnosis even by the experienced endoscopist. In the same vein, this new technique should be non-invasive, and preferably not relying on the experience of the endoscopist, in order to be as objective as possible. Achieving these goals would set a milestone in the screening and early detection of HNSCC.

We herein describe narrow-band imaging (NBI) (Olympus Medical Systems Corporation, Tokyo, Japan), a diagnostic tool already proven as a useful screening method in other endoscopic fields, and demonstrate its usefulness in the early detection of HNSCC, as reported by previous studies in the otolaryngologic literature².

Technology

The perception of the endoscopist of abnormal conditions of the UADT with regard to the morphology of pre-malignant and malignant lesions is changing ⁵. These lesions often present with non-elevated morphology, with slight discolouration, as the superficial mucosal vascular structures are constantly changing during the process of tumour neoangiogenesis. Digital reconstruction of images, captured by the videoendoscope, makes image processing possible ⁶. The haemoglobin index (i.e., haemoglobin content in the mucosa) can be estimated by adjusting the colour of reflected light ⁷. The technique of NBI combines the potentials of both technologies (magnification of videoendoscopy and image processing) ⁸, based on the principle of modifying the spectral characteristics of the illuminating light by narrowing the bandwidth of the optical filter in the light source ⁹.

From a technical point of view, the NBI system contains the same components of a conventional red, green, and blue (RGB) sequential videoendoscope system. In NBI mode, an optical filter allows a narrow-band light to pass at a short wave length of 400-430 nm (centered at 415 nm), that only penetrates the mucosa superficially enhancing the mucosal vasculature. Another narrow-band light of longer wavelength 525-555 nm (centered at 540 nm) penetrates deeper into the tissues revealing the submucosal vessels ⁹.

Basically, NBI relies on the principle of depth of light penetration. In contrast to red light, blue light has less penetration and less scattering thus enhancing image resolution ¹⁰. Furthermore, the blue filter is designed to correspond to the peak absorption spectrum of haemoglobin to enhance the image of capillary vessels on surface mucosa. The reflection is increased by a monochromatic charge coupled device (CCD), and an image processor creates a composite pseudocolour image, which is displayed on a high definition video screen ⁹, enabling NBI to enhance mucosal contrast without the use of dyes. Thus, superficial mucosal lesions that would previously have been missed by regular white light during endoscopy would be identified by the blue light of NBI, based on the increased vascularity and neoangiogenesis of the tumour.

NBI is not cumbersome and is easy to set up and operate with just the switch of a button on the videoendoscope, videocamera or monitor console between conventional white light and filtered narrow-band light. Moreover, the diagnostic value of this optical technology is enhanced by combining it with magnifying endoscopy (NBI-ME) that allows the endoscopist to zoom on the mucosa, simply switching a dedicated button.

The best image definition, both for conventional rigid endoscopy and NBI, is achieved by the use of a HDTV (High Definition Television) camera incorporated on the endoscope, thus no degradation occurs in the quality of images during magnification. HDTV offers up to 1080 lines of resolution, allowing a signal definition 4.26 times superior to that of regular standard definition television (SDTV). The result is a very sharp, detailed and bright picture that looks almost three dimensional ¹¹. Unfortunately, for technical reasons, at the time of preparing this report, the HDTV camera can only be incorporated on the rigid endoscopes, with the flexible type only taking advantage of a standard definition camera.

Non-ENT NBI applications

There are many reports in the literature on the use of NBI in various anatomic regions of the aerodigestive tract, namely the tracheobronchial tree, oesophagus, stomach, duodenal ampullary, and colorectal regions. Hamamoto et al. ¹² evaluated the usefulness of combining NBI-ME in the diagnosis of Barrett’s oesophagus (BE). Images and videos, both by conventional ME and by NBI-ME, were obtained in a group of patients by an experienced endoscopist, and the differences were evaluated by another. They found that visualization of the oesophago-gastric junction, net-like blood vessels, and columnar-lined oesophagus were all visualized far better by NBI-ME.

The value of NBI in the detection of high grade dysplasia and early cancer (HGD/EC) in BE was evaluated by Kara et al. ¹³ in a prospective randomized crossover study. Patients with BE underwent high resolution endoscopy (HRE) followed by indigo carmine chromoendoscopy (ICC), or HRE followed by NBI in a randomized sequence. They found that a good quality HRE, in experienced hands, is capable of detecting most lesions with HGD/EC in patients with BE, and that ICC and NBI, in combination with HRE, are of limited additional value for primary detection of lesions. They found similar results between ICC and NBI in the detection of HGD/EC in patients with BE, concluding that these techniques are most suitable for targeted detailed inspection of suspected areas. Moreover, Kara et al. ¹⁴ introduced a classification system of mucosal and vascular patterns in BE with NBI. They found that NBI was useful in revealing mucosal morphology characteristics of non-dysplastic BE and high-grade intraepithelial neoplasia without the need of staining, concluding that such a technique had a relatively high diagnostic value for these lesions when used for targeted detailed examination of areas of interest.

Sharma et al. ¹⁵ in a prospective blinded study on patients with known or suspected BE, found that NBI was a diagnostic tool with a high degree of accuracy for the detection of metaplastic and dysplastic tissue within the BE segment, permitting what they called “virtual in vivo histology” and potentially eliminating the need for random biopsies.

In the stomach, Uedo et al. ⁹ investigated the use of NBI-ME for the detection of intestinal metaplasia in the gastric mucosa by the appearance of what they called a light blue crest (LBC). LBC was defined as a fine blue-white line on the crests of the epithelial surface or gyri. They devised a grading system for LBCs and found that their appearance correlated with histological evidence of intestinal metaplasia with a sensitivity of 89%, a specificity of 93%, a positive predictive value of 91%, a negative predictive value of 92%, and an accuracy of 91%. They concluded that detection of LBC with NBI is a highly accurate sign of the presence of histological intestinal metaplasia.

In another study, Uchiyama et al. ¹⁶ investigated the reliability of NBI-ME to diagnose and differentiate between benign and malignant duodenal ampullary tumours. In their study, they investigated patients whose ampullas were noted, with conventional endoscopy, to be significantly enlarged or protruding. The correlation between NBI-ME images and histo-pathological findings were investigated. Once again, they found that the NBI system was able to predict the histological characteristics of ampullary lesions and concluded that there is a great potential for foci of adenocarcinoma to be accurately diagnosed by NBI, which escaped detection with forceps biopsy.

Machida et al. ¹⁷ studied the use of NBI for evaluating colo-rectal lesions. They estimated the accuracy of differentiation between neoplastic and non-neoplastic lesions using the NBI system in comparison with conventional colonoscopy, and with chromoendoscopy. They found that in the examination of colonic lesions the NBI system provides additional imaging features to those obtained both with conventional endoscopy and chromoendoscopy. For distinguishing neoplasms from non-neoplastic lesions, NBI was equivalent to chromoendoscopy. A further investigation was reported by Su et al. ¹⁸ comparing conventional colonoscopy, chromoendoscopy, and NBI in the differential diagnosis of neoplastic and non-neoplastic colonic polyps. They also found NBI to be far more specific, sensitive, and accurate than conventional colonoscopy, and equal to chromoendoscopy. However, NBI is a far less demanding endoscopic technique when compared to chromoendoscopy.

On the other hand, in the lower airway, Shibuya et al. ¹⁹ investigated the use of high magnification bronchovideoscopy combined with NBI for detailed examination of angiogenic squamous dysplasia (ASD) in the bronchial tree of heavy smokers at high risk for lung cancer. The study was carried out on sputum cytology specimens suspicious or positive for malignancy. They found that the microvessels, vascular networks of various grades, and dotted vessels in ASD tissues were clearly visible on NBI. Diameters of the dotted vessels identified by NBI images were in keeping with the diameters of ASD capillary blood vessels diagnosed by pathological examination, confirming the importance of NBI in detecting ASD and its discrimination from other pre-invasive bronchial lesions.

ENT NBI applications

When it comes to the field of otolaryngology and head and neck surgery, the literature on the use of NBI is very sparse. Indeed, we found only two studies in the literature where NBI was used in the assessment of UADT SCC.

Muto et al. ¹ were the first to use a prototype NBI system for screening the oropharynx and hypopharynx for SCC in patients with oesophageal cancer. The clinical examination was performed during routine endoscopic screening or surveillance procedures, and carried out by a single endoscopist. At first, a non-magnifying observation with NBI was performed to identify abnormal mucosal areas. If identified as well demarcated brownish lesions, photographs of the non-magnified view were taken. Subsequently, they observed the lesion and surrounding normal mucosa under magnification. If scattered brownish dots were observed, within the lesion, under the magnifying NBI, they diagnosed the lesion as malignant, according to the criteria reported for oesophageal lesions ²⁰. Magnified NBI clearly revealed scattered brown dots within all malignant lesions. These dots were histologically confirmed to be dilated capillaries on immunohistochemical staining with antihuman monoclonal CD-31 antibody. This microvascular proliferation (MVP) pattern was observed in all lesions by NBI, whereas it was rarely detected by conventional observation, even with magnification. Over a period of 17 months, 34 consecutive superficial lesions were found in 18 patients. Multifocal carcinoma was found in 5 (28%) of them. All lesions exhibited a MVP pattern on magnified NBI as well demarcated brownish areas. Overall, 13 patients with a combined total of 29 lesions underwent EMR under general anaesthesia. Of these, the piriform sinus was the most frequent primary site (66%, or 19 out of 29 lesions). The median tumour diameter was 20 mm (range, 1.3-40 mm). Twenty-one (72%) lesions were histologically confirmed to be carcinoma in situ, and the remaining showed evidence of microinvasion (0.05-1 mm) beneath the epithelium. Vascular invasion was observed in only one lesion. After a median follow-up period of 8 months (range 1-16 months), there were no cases of local recurrence. The Authors concluded that the NBI technique could significantly improve the efficacy of screening for, and surveillance of, lesions of the head and neck region, especially those at oropharyngeal and hypopharyngeal sites, during routine endoscopic examination.

Watanabe et al. ² further investigated the efficacy of the NBI system in endoscopic screening of the oropharynx and hypopharynx in patients with oesophageal cancer. The study was carried out on 217 consecutive patients by the same otolaryngologist. Among these patients, 6 superficial lesions were detected with the NBI system, one in the oropharynx and 5 in the hypopharynx. Of these lesions, only 4 could hardly be recognized by conventional endoscopy because of their small diameter (5 mm or less). The other two lesions had a diameter of 1 cm or more and could be recognized by both conventional endoscopy and NBI. However, the Authors stated that NBI was more beneficial in recognizing the superficial lesions than the conventional endoscopic view. On NBI observation, all lesions were also recognized as well demarcated brownish areas, exhibiting scattered brown dots within these areas on close view. EMR was performed for all the 6 patients and the histological examination revealed CIS. Even though detecting 6 lesions of the UADT in 217 patients with oesophageal cancer may seem a small number, Watanabe et al. concluded that, over 10 years, they had previously performed 9940 conventional endoscopic screenings for 1118 patients with oesophageal cancer. Among those, 142 early HNSCCs were detected in 127 patients, resulting in an average of 70 endoscopic screenings needed to detect one lesion with their conventional endoscopy screening programme. If the same ratio were to be applied in their more recent study, 217 conventional endoscopic examinations should reveal about 3 superficial lesions only. Thus they felt that the NBI system might improve the sensitivity by about two-fold over the conventional method. Furthermore, they emphasized the major differences between the NBI system they used and that being used for the digestive tract, reported in previous papers ²¹. The diameter of the endoscope is much smaller, only 3.2 mm in diameter, allowing a thorough examination of the UADT to be performed transnasally, and the time needed for the examination about 3-5 minutes, much shorter than the time required in the digestive tract examination setting. These differences allow the use of NBI as an outpatient procedure with the patient in a seated position, and totally avoiding the endoscopy laboratory.

NBI future trends in ENT

Even though the experience of the Department of Otorhinolaryngology – Head and Neck Surgery of Brescia, in the use of NBI, is still in its infancy, the easy application of this technical device and the optimistic impressions already developed in the otolaryngologic as well as in other endoscopic disciplines, prompted us to prospectively recruit patients for clinical trials to precisely evaluate its diagnostic accuracy. As already demonstrated, the NBI system plays an important role in the screening and diagnosis of superficial lesions in various anatomic sites of the aerodigestive tract. The paucity of studies in the otolaryngologic literature should stimulate future multi-institutional investigations for the early detection of HNSCCs in high-risk patients (including those enrolled in a surveillance protocol after treatment of a previous head and neck cancer). The oropharynx and hypopharynx are not the only sites that can benefit from this kind of technology, but even the oral cavity (Figs. (Figs.1,1, ,2,2,,3),3), nasal fossa, nasopharynx, and larynx should be accordingly investigated with NBI. Future refinements in the HDTV system and flexible videoendoscopic instrumentation will allow a more accurate evaluation of the UADT in head and neck cancer patients even in the pre-operative setting. Moreover, another interesting field of research is represented by the intra-operative examination of tumour boundaries by rigid endoscopes coupled to the already existing HDTV monitors with the NBI system in order to assess the validity of such a tool in ensuring complete tumour resection and assessing the surgical margins. Far from being the panacea of early HNSCC detection, we foresee NBI as a useful tool in the future pre-, intra-, and post-operative endoscopic work-up of neoplastic lesions of the UADT.http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2644976/

Ophthalmologic Disorders in Children With Syndromic and Nonsyndromic Hearing Loss

2010-03-16T00:58:00.001-07:00

Comprehensive ophthalmologic examination revealeda rate of ophthalmologic disorders in children with SNHL inthe lower end of the previously reported rates of 31% to 61%.

Children with nonsyndromic SNHL have an approximately 2- to3-fold increase in ocular abnormalities compared with the generalpediatric population. Ophthalmologic and genetic consultationsare warranted in patients with congenital SNHL.

Bifocal distraction osteogenesis

2010-03-16T00:55:00.000-07:00

http://archfaci.ama-assn.org/cgi/content/full/4/1/8Use of bifocaldistraction osteogenesis (BDO) with internal devices to treatpatients having bony defects of the maxillofacial skeleton followingtumor ablation and to focus on outcomes of dental implant placementin patients having maxillomandibular segmental defects.

Inclusion criteria:
a bony defect in the maxillofacial skeleton,
moderate soft-tissue defect,
local or general conditions thatpreclude more aggressive surgery,
and adequate patient compliance.

Types of BDO included horizontal mandibular or maxillar alveolar,
bilateral alveolar,
vertical mandibular or maxillar,
ramus andbody,
mandibular angle,
symphysis,
the 2-step procedure,
temporalismuscle flap reconstruction,
vascularized free–fibularflap reconstruction,
radial forearm free-flap reconstruction,
and pectoralis muscle flap reconstruction.

Bifocal distraction osteogenesis potentiallybenefits patients with bony defects following tumor ablationat various locations in the maxillofacial skeleton.
Sufficientbone is gained to allow dental implant placement, an importantfunctional outcome.

Three types of distraction osteogenesis have been described:
monofocal,
bifocal, and
trifocal.

A, Monofocal distraction is used to lengthen abnormally shortened bones and involves separation of 2 bone segments across a single osteotomy.

B, Bifocal distraction is used to repair a segmental defect and requires creation of a transport disk, which is then distracted across the defect until it docks with the opposing bony segment.

C, Trifocal distraction is similar to bifocal distraction attempts to halve the distraction time by transporting 2 disks from opposite ends of a defect to dock in the middle.

Relationship between hOGG1 loss of heterozygosity (LOH), Hashimoto thyroiditis (HT), and papillary thyroid cancer (PTC)

2010-03-16T00:48:00.001-07:00

hOGG1 LOH is strongly associated with PTCand HT but not with benign thyroid.
We hypothesize that thyroidfollicular epithelia accumulate aberrant genetic changes inlong-standing HT, which may represent a precursor lesion ofPTC.

Laryngeal sarcoidosis

2010-03-16T00:46:00.001-07:00

Minimally invasive endoscopic surgery withintralesional corticosteroid injection and laser reduction isan effective method of controlling laryngeal sarcoid.
It improvessymptoms immediately with minimal morbidity and, most importantly,reduces the need for systemic steroid administration in mostpatients.
This study supports early recognition and endoscopicintervention in the management of laryngeal sarcoidosis.

Tuberculous otitis media

2010-03-15T02:37:00.000-07:00

Worldwide, TB is the single most important bacterial infection, with more than 7 million active cases. Fortunately the ear is rarely infected by mycobacterium tuberculosis; current incidence is estimated to be between 0.9% and 0.04%. The tubercle bacillus can spread to the middle ear by several routes. The most common route is hematogenous. Other routes of infection include regurgitation of the tubercle bacillus through the eustachian tube, through previously existing tympanic membrane perforations; and by direct extension from a nasopharyngeal site of infection. TB can also be transmitted congenitally and is associated with a high incidence of ear involvement. However, congenital TB is extremely rare and hardly ever presents with isolated ear involvement.

Classically, tuberculous otitis media is described as having
an insidious onset with painless otorrhea,
multiple tympanic membrane perforations,
abundant pale granulation tissue in the middle ear,
early severe hearing loss out of proportion to clinical findings, and
bone necrosis.

However, the current literature indicates most patients actually present with a single perforation. Indications of tuberculous otitis media include hearing loss out of proportion to clinical findings, failure to respond to the usual medical therapy, post-mastoidectomy recurrence of granulation tissue, slow wound healing, persistent otorrhea, and the formation of bony sequestra.

The evaluation of these patients should include a history of contact to active tuberculosis, placement of a PPD skin test with control, culture and stain of ear drainage for AFB, biopsy of granulation tissue for histology and culture, and evaluation of the immune system if indicated. All patients should be evaluated with a chest x-ray, urinalysis, sputum for AFB culture and smear, gastric aspirates in children, and lumbar puncture if CNS involvement is suspected or if the patient has miliary TB. On examination of the ear, the tympanic membrane will appear dull and thickened with dilated vessels on the surface early and will later develop perforations from liquification of caseous tubercles. There will also be abundant granulation tissue in the middle ear. Late complications include facial paralysis, labyrinthitis, postauricular fistulae, subperiosteal abscess, petrous apicitis, and intracranial extension of infection. Radiologic evaluation of the temporal bone cannot differentiate TB from non-specific infections. A well-pneumatized mastoid with chronic otitis media is suggestive of tuberculous otitis media but not diagnostic, as these cases can also have sclerotic and destructive mastoid lesions.

The differential diagnosis of tuberculous otitis media includes
fungal infections,
Wegener's granulomatosis,
midline granuloma,
sarcoidosis,
syphilis,
necrotizing otitis externa,
atypical mycobacterial infections, and
histiocytosis X.

The treatment of tuberculous otitis media is primarily medical and should include a 6- to 9-month course of Isoniazid, Rifampin, and Pyrazinamide. Indications for surgical intervention include the late complications of tuberculous otitis media; cases unresponsive to medical therapy; extensive disease with bone sequestra or necrotic bone; and reconstruction of the tympanic membrane and ossicular chain after the middle ear disease has been eradicated.

Ethmoid Mucocele

2010-03-15T00:04:00.000-07:00

http://waent.org/picture_of_the_month/20100312-ethmoid-mucocele/ethmoid-mucocele.htm

Presbylarynges

2010-03-14T23:58:00.001-07:00

http://www.entusa.com/presbylarynges_2009.htm

Thyroid hemangioma

2010-03-14T04:11:00.001-07:00

Thyroid hemangioma is very rare, and only a few cases have previously been reported. We encountered a patient with thyroid hemangioma diagnosed after surgery.

Case Report: A 71‑year‑old woman visited our hospital with a mass in the left thyroid region. A 5‑cm, elastic mass of the thyroid was palpable in the left anterior neck. On cytology by fine‑needle aspiration (FNA), the specimen mainly contained blood components without apparent atypical cells. A tumor with abundant blood flow was suspected based on Doppler ultrasonography. Adenomatous goiter was suspected, and subtotal thyroidectomy was performed. A blood clot was present in the tumor, and hemangioma was diagnosed on postoperative histopathological examination.

Discussion: Preoperative diagnosis of thyroid hemangioma is difficult. However, Doppler ultrasonography and FNA are useful for diagnosis. A differential diagnosis of hemangioma should be considered when blood flow is abundant and only blood components are collected.

Autonomic failure - Multiple system atrophy - Central sleep apnea - Vocal cord abductor palsy - Urinary incontinence - Serotonin

2010-03-14T04:05:00.000-07:00

Survival of multiple system atrophy (MSA) depends on whether a variety of sleep-related breathing problems as well as autonomic failure (AF) occur.

Since the brainstem lesions that cause respiratory and autonomic dysfunction overlap with each other, these critical manifestations might get worse in parallel.

If so, the detection of AF, which is comparatively easy, might be predictive of a latent life-threatening breathing disorder.

In 15 patients with MSA, we performed autonomic function tests composed of postural challenges and administered a questionnaire on bladder condition, as well as polysomnography and laryngoscopy during wakefulness and under anesthesia.

Polysomnographic variables such as the apnea-hypopnea index (AHI) and oxygen saturation (SpO₂) and the findings of laryngoscopy were compared with the degree of cardiac and urinary autonomic dysfunction. AHI, mean SpO₂ and the lowest SpO₂showed significant correlations with urine storage dysfunction.

In addition, patients with vocal cord abductor paralysis (VCAP) or central sleep apnea (CSA) contributing to nocturnal sudden death had more severe storage disorders than those without.

On the other hand, no significant relationship between polysomnographic variables and orthostatic hypotension was observed except in the case of mean SpO₂.

These results indicate that life-threatening breathing disorders have a close relationship with AF, and especially urine storage dysfunction.

Therefore, longitudinal assessment of deterioration of the storage function might be useful for predicting the latent progress of VCAP and CSA.

Subfrontal schwannomas, Olfactory groove schwannomas, Olfactory ensheathing cells

2010-03-14T04:01:00.000-07:00

Subfrontal schwannomas, sometimes referred to as olfactory groove schwannomas, are rare tumors (34 cases reported to date).

Despite the name and several theories proposed in the literature, there is no officially recognized description of the tumor's cell origin. Yasuda proposed the concept of an olfactory ensheathing cell (OEC) tumor in 2006.

Olfactory ensheathing cells are glial cells that ensheath the axons of the first cranial nerve.

Microscopically, both olfactory ensheathing cells and Schwann cells have similar morphological and immunohistochemical features. However, immunohistochemically olfactory ensheathing cells are negative for Leu7 and Schwann cells positive. A 30-year-old woman presented with a subfrontal, extraaxial, enhancing tumor, and underwent gross total resection.

Immunohistochemical reactivity data suggested a schwannoma (positive for S-100 and negative for epithelial membrane antigen).
However, the tumor was negative for Leu7.
Accordingly, our final diagnosis was that of an OEC tumor.
Subfrontal schwannoma immunohistochemical staining, if negative for Leu7, is indicative of an OEC tumor. It is possible that schwannoma-like extraaxial tumors at the anterior skull base are OEC tumors, which negative Leu7 staining can confirm.