Anti-estrogen therapy for hereditary hemorrhagic telangiectasia - a long-term clinical trial.

STATEMENT OF PROBLEM: Hereditary hemorrhagic telangiectasia (HHT) is associated with recurrent epistaxis in 90% of the cases. Good response to hormone treatment has been documented, although its use remains controversial. The aim of this study was to examine the efficacy of an anti-estrogenic agent, tamoxifen, in the treatment of HHT-associated epistaxis. METHOD OF STUDY: Out of 46 patients with diagnosis of epistaxis due to HHT who started treatment with tamoxifen 20 mg/d, 38 patients completed a mean of 23.4 months of treatment. All patients filled out a self-assessment questionnaire of rhinologic Quality of Life and epistaxis grading scale. MAIN RESULTS: The bleeding score and the Quality of Life score improved. Hemoglobin concentration also improved. None of the patients needed blood transfusions during the treatment period. Only one patient had minor side effects of the drug. PRINCIPAL CONCLUSIONS: Tamoxifen appears to be an effective agent for the treatment of epistaxis due to HHT.

Squamous cell carcinoma of the oral tongue in patients younger than 30 years: clinicopathologic features and outcome.

OBJECTIVE: To assess the possible effect of young age on clinical behaviour and survival outcome of squamous cell carcinoma of the oral tongue. DESIGN: Retrospective, case control study. SETTING: A major tertiary referral centre. PARTICIPANTS: Eighty-five patients with oral tongue squamous cell carcinoma with at least 2 years of follow-up. MAIN OUTCOME MEASUREMENTS: Clinical and histopathological staging, disease-free survival, disease-specific survival and overall survival. RESULTS: Eleven patients (13%) were younger than 30 years. Compared to the older patients, they had a significantly worse N stage (P = 0.041), more perineural invasion (P = 0.012), and higher rates, though not significant, of treatment failure (46%, including 60% with distant metastases, versus 35%, nearly all locoregional) and mortality (100% of treatment failures versus 73%). There were no significant between-group differences in 5-year disease-free, disease-specific, and overall survival. CONCLUSION: In this study, patients younger than 30 years of age presented with advanced tumour stages and with a different failure pattern compared to the older age group. This may be attributable to age-related biologic behaviour or delayed cancer diagnosis. Differences in disease free survival and overall survival could not be established.

The impact of positive resection margins in partial laryngectomy for advanced laryngeal carcinomas and radiation failures.

OBJECTIVES: To investigate the prognostic significance of positive margin and disease course in partial laryngectomy for advanced laryngeal carcinoma and radiation failure. DESIGN: Retrospective case control study. SETTING: A major tertiary referral centre. PARTICIPANTS: Patients who underwent partial laryngectomy for advanced laryngeal carcinoma or for radiation failure with at least 2 years of follow-up. MAIN OUTCOME MEASURES: Margin status versus primary treatment and salvage treatment versus disease-free survival and overall survival. RESULTS: Twenty nine patients with sufficient follow-up data were found. Twelve patients had histological positive margin. Five of the 12 patients with a histological positive margin failed surgery as did four patients with clear margins. A positive margin had no effect on disease-free survival (P = 0.287) but was associated with poorer overall survival (P = 0.051). Of 11 patients treated primarily with surgery, recurrence was documented in one of eight with a positive margin and none of three with clear margins. Of 18 patients who underwent surgery secondary to radiation failure, recurrence was documented in all four with a positive margin and 4 of 14 with negative margins. Extended frontolateral resection, performed only in radiation failures, was associated with worse disease-free survival. CONCLUSIONS: Non-irradiated patients with involvement of a single margin after partial laryngectomy may be spared total laryngectomy if adjuvant radiation is administered. Patients who fail radiation should undergo radical partial laryngectomy, with conversion to total laryngectomy in those with a positive margin.

Histological changes in the cervical lymph nodes after radiotherapy.

Radiotherapy to the neck is frequently employed in the treatment of malignancies, sometimes alone and sometimes before or after surgery or with or without chemotherapy. We checked the histopathological changes in the neck after radiotherapy, dividing the changes into two groups. One with short-term changes between six months to one year, and the second group with long-term changes after more than six years. We compared these two groups with a control group without radiotherapy. The changes in the long-term group were more pronounced than in the short-term group, with more stromal fibrosis, vascular changes, and specially lymph nodes smaller than one centimeter. Thus, bigger lymph nodes (more then one centimeter) are more radiosensitive and become smaller after six years.

Squamous cell carcinoma of the oral tongue.

Between 1958 and 1983 at Beilinson Medical Center, Petah Tiqva, Israel, 75 patients were diagnosed as having squamous cell carcinoma of the body of the tongue. Of these, 42 were male and 33 female and the average age at the time of diagnosis was 61.6 years. 29.33% were in clinical stage I, 36% in stage II, 26.67% in stage III and 8% in stage IV. Fifteen patients underwent only surgery, nine received only irradiation and 51 received combined treatment. The 5-year survival rate was 68.3% with a significant difference between that for stage I (90%) and stage II (83.7%) and that for stages III and IV (43.07% and 0%, respectively) (P less than 0.001). Among the 24 with recurrence of the malignancy, 20 died despite treatment and four patients died of causes unrelated to the malignancy. From this experience it was concluded that local excision of the tumor should be performed only in stage I patients and that in stage II patients prophylactic treatment of the neck (neck dissection and irradiation) should be undertaken because of the high incidence of occult metastases.

Anaplastic carcinoma of the thyroid.

A review of 48 cases of anaplastic cell carcinoma of the thyroid gland treated in the Department of E.N.T. of Beilinson Medical Center revealed more than 90% of the patients to be over 50 years of age. Twenty-five percent of the patients showed, histologically, areas of transition from differentiated carcinoma (either papillary or follicular) into anaplastic carcinoma. The patients were treated by surgery and/or irradiation and/or chemotherapy. Twenty-seven patients underwent subtotal or total thyroidectomy and 21 patients underwent biopsy or partial thyroidectomy. The survival rate was very poor, two-year survival rate was 28%. Twenty-eight of the patients (58%) died within one year of diagnosis.

Supraglottic carcinoma: a retrospective study of 114 patients.

We retrospectively evaluated the management of supraglottic carcinoma at our centre during the last 35 years to determine the preferred mode of treatment. A review of the medical records yielded 114 patients with supraglottic T1 and T2 carcinoma who were diagnosed and treated in the Departments of Otolaryngology, Head and Neck Surgery, and Oncology between 1959 and 1993. Of these, 47 (41.2%) had T1 carcinoma (stage I) and 67 (58.8%) T2 (stage II). Treatment varied among radiotherapy, surgery, or combined radiotherapy and surgery. Twelve patients underwent elective neck dissection, one of whom (8.3%) was found to have occult metastases. Local failures were noted in 22 patients (11 T1: 11 T2), three of whom also had neck metastases. All except one T2 patient received radiotherapy. Radiotherapy yielded the best survival rates for T1 disease as combined therapy did for T2. Five-year recurrence rates for T1 patients were 35% for those treated by radiotherapy and 42% for those treated with combined therapy; corresponding figures for T2 patients were 39% and 28%. We suggest that patients with T1 supraglottic carcinoma be managed with radiotherapy and patients with T2 with combined therapy. We believe there is no need for elective neck dissection, especially in T1.

T1 glottic carcinoma involving the anterior commissure.

This study reviews the records of 56 patients with early glottic carcinoma involving the anterior commissure (T1N0M0) who were treated between 1958 and 1988 at Beilinson Medical Center. Five- and 10-year survival rates were 82% and 60%, respectively. Most failures were local (15 patients). In addition, three patients who were treatment failures had neck lesions (regional failure) and one had lung lesions (distant metastases). All 56 patients received irradiation as the only mode of initial treatment. The effectiveness of irradiation for anterior commissure lesions is therefore evaluated. The study supports previous reports suggesting that glottic carcinoma involving the anterior commissure is associated with a high rate of treatment failure.

Stapedotomy technique and results: ten years' experience and comparative study with stapedectomy.

Fifty of the 810 patients who underwent stapedotomy for otosclerosis from 1969 through 1988 were randomly chosen for follow-up of at least 5 years. Most of the patients (65%) had follow-up of 10 years after stapedotomy, but another 50 patients who underwent stapedectomy had follow-ups of longer than 10 years. In 50 patients, stapedectomy under local anesthesia was performed by removal of the footplate of the stapes, using an endaural incision, and covering the oval window with Gelfoam. In the other 50 patients, stapedotomy was performed under general anesthesia, using an endopreauricular incision, making a small hole in the footplate, and covering the hole only with blood from the surgical area. Although both groups showed improvement in hearing after the operation, the air-bone gap in the stapedotomy group was significantly better than that in the stapedectomy group. The use of the endopreauricular incision under general anesthesia was preferable to endaural incision under local anesthesia because the operative field was wider, more convenient for the surgeon, and conducive to patient safety. There were no significant complications in either group.

Endoscopic transnasal transsphenoidal microsurgery versus the sublabial approach for the treatment of pituitary tumors: endonasal complications.

OBJECTIVE: To evaluate the nasal complications after transnasal transsphenoidal operations for pituitary tumors, comparing two surgical techniques: traditional sublabial transseptal and endoscopic transseptal techniques. STUDY DESIGN: We retrospectively evaluated by self-reported questionnaire and endoscopic examination the nasal condition of 40 consecutive patients with pituitary tumors: 20 patients had endoscopic surgery and 20 had surgery with the traditional sublabial technique. RESULTS: Compared with the traditional technique, the endoscopic approach was associated with a shorter operative time (about 40 min), shorter hospitalization time (about half), absence of recurrent epistaxis snoring and denture problems, and lower incidence of septal perforation, synechia, and crust formation. Furthermore, loss of nasal tip projection was found only in the group that had surgery with the sublabial technique. CONCLUSIONS: Endoscopically guided transseptal transsphenoidal surgery is simple to perform and time-saving, and it results in fewer nasal and denture complications than the sublabial technique. At the same time, it allows the surgeon all the benefits of the binocular microscopic surgical field that are associated with the traditional approach.

Clinicopathologic study and classification of vocal cord cysts.

Cysts of the true vocal cords are less common than other laryngeal cysts. They are usually easily recognized and managed. Patients present with complaints of hoarseness and/or dyspnea. We report our experience with 41 cases of cysts located in the true vocal cords. Clinical and histological aspects are reviewed and discussed. A new histological classification is proposed: A: cysts lined by columnar epithelium with mucous content; B: lined by columnar epithelium with cilia; C: lined b squamous epithelium without keratinization; D: lined by squamous epithelium with keratinization.

Detection of specific IgA antibodies to varicella zoster virus in serum of patients with Ramsay Hunt syndrome.

Varicella zoster virus (VZV)-specific IgG and IgA antibody titers were determined in serial serum samples of 23 patients with Ramsay Hunt syndrome by the immunoperoxidase assay. Varicella zoster virus-specific IgG antibodies were found in the first serum samples of all the patients. In 80% of 20 patients in whom a serum sample was available within 5 days after the onset of the disease. VZV-specific IgA antibodies were detected. The second serum sample was VZV-specific IgA-positive in all of the patients. While all the healthy age- and sex-matched control subjects had VZV-specific IgG antibodies, VZV-specific IgA antibodies were detected in a low titer (dilution = 2) in only three of the subjects. By using VZV-specific IgA antibody titers greater than or equal to 2 and greater than or equal to 4 by the immunoperoxidase assay as a "cutoff" for younger and older patients with Ramsay Hunt syndrome, respectively, an early diagnosis of the disease can be obtained in 89% of the younger and in 64% of the older patients by a single serum sample.

Respiratory distress due to diffuse cervical hyperostosis.

Cervical spondylosis and ankylosing hyperostosis of the cervical vertebrae are common findings. Although these hypertrophic changes can be completely asymptomatic, it is known that dysphagia may occur occasionally in the presence of massive cervical hyperostosis. Laryngotracheal symptoms due to cervical hyperostosis are less frequent and may be managed initially as tumors of the esophagus, trachea, or thyroid gland. The management of two severe cases of dyspnea due to cervical ankylosing hyperostosis are discussed.

Fistulae in the inner canthus associated with ethmoiditis.

A case of fistulae in the inner canthus arising from the ethmoidal air cell sinuses is presented. This type of complication is very rare, and to the best of our knowledge no similar case has been reported in the literature. Clinical findings, imaging, pathology and treatment are presented and discussed.

Isolated sphenoid sinus changes--history, CT and endoscopic finding.

This study reviews the records of 21 patients with isolated sphenoid sinus disease who were treated by rigid endoscopic sphenoidotomy at the Nose and Sinus Unit, Department of Otolaryngology of Beilinson Medical Center, Israel. Diagnosis was made on the basis of history, rigid nasal endoscopy and computed tomography (CT) scan. The most frequent symptom was headache; no instances of 'pathognomonic' headache were found. Sphenoidotomy was performed through the area of the natural ostium. The pathological finding was infection in 11 patients, cyst in four patients, polyps in three patients, mucocoele in two, and inverted papilloma in one patient. Surgical results were very good. Endoscopic sphenoidotomy proved to be safe, with minimal blood loss, reduced operating time, decreased morbidity, and short post-operative hospitalization.

Anaplastic thyroid carcinoma metastatic to the tonsil.

We describe a 65-year-old woman who presented with a left tonsillar tumour. Biopsy revealed metastatic anaplastic cell carcinoma of the thyroid. Metastatic tumours are rare in the tonsils. This case, to the best of our knowledge, is the first in which the thyroid was the primary site.

Heterotopic salivary tissue and branchial sinuses.

Heterotopic salivary tissue and branchial sinuses occur not infrequently (Goodman et al., 1981; Stingle and Priebe, 1974), caused probably by heteroplasia within remnants of the second cleft (Stingle, 1974). Bilateral lesions which presented clinically as branchial cleft sinuses have been removed from a patient's neck; both of them proved to be branchial cleft sinuses with elements of salivary tissue. The paper describes salivary tissue and branchial sinuses along the anterior border of the sternocleidomastoid muscle on both sides of the neck and bilateral preauricular fistulae occurring in a boy and probably in his sister.

Laser posterior ventriculocordectomy with partial arytenoidectomy for the treatment of bilateral vocal fold immobility.

Bilateral vocal fold immobility in adduction usually creates severe dyspnoea. Many surgical procedures have been established to improve the airway insufficiency in affected patients. Over the last six years 22 patients with bilateral vocal fold immobility in our department have undergone CO(2) laser posterior ventriculocordectomy with partial arytenoidectomy (PVCPA). None had dyspnoea or a disturbance in the immediate post-operative period or during follow up, which ranged from five months to six years. Only one patient required a second procedure. Laser PVCPA appears to be an effective and reliable method for the treatment of bilateral vocal fold immobility in selected patients.

Mucus retention cyst of the maxillary sinus: the endoscopic approach.

OBJECTIVE: To present our experience of endoscopic surgery for symptomatic mucus retention cyst of the maxillary sinus. DESIGN: Retrospective study. SETTING: Teaching hospital, Israel. PATIENTS: 60 patients with 65 symptomatic cysts of the maxillary sinus who were operated on endoscopically. Only patients with large cysts that filled at least 50% of the sinus space were included. INTERVENTION: A rigid nasal endoscope was used in all cases; most of the cysts were removed through the natural sinus ostium. RESULTS: Cysts recurred in only two patients during the first postoperative year. There were no complications from the procedure. CONCLUSION: The endoscopic approach to the treatment of maxillary sinus cyst is associated with a low rate of recurrence (3% in this study) and no complications, and we recommend it as the surgical procedure of choice.

Multiple branchiogenic anomalies.

A family with three generations of male-to-male transmission of a rare syndrome is presented. The syndrome includes bilateral cervical branchial sinuses, bilateral preauricular sinuses, bilateral malformed auricles and bilateral hearing impairment. Two important aspects of this syndrome are discussed. First, male-to-male transmission rules out a sex-linked mode of inheritance. Second, the finding of both conductive and sensorineural hearing loss is puzzling since the middle and inner ear differ in embryogenesis as to both origin and timing. Few explanatory mechanisms are discussed.