Faculty Bibliography

BACKGROUND: Fanconi anemia (FA) is a rare autosomal recessive disorder characterized by a high degree of genomic instability and predisposition to cancer development. Recent evidence suggests that the incidence of head and neck squamous cell carcinoma (HNSCC) may be increased in patients with FA. OBJECTIVE: To determine the cumulative incidence, tumor distribution, and outcome of HNSCC in patients with FA. DESIGN AND SETTING: We analyzed data from 754 subjects from the International Fanconi Anemia Registry, a prospectively collected database of patients with FA. MAIN OUTCOME MEASURES: Cumulative incidence of HNSCC and 2-year overall, relapse-free and disease-specific survival. RESULTS: Of the 754 patients in the International Fanconi Anemia Registry, 19 (3%) had HNSCC. This is a significantly higher incidence of HNSCC compared with that observed in the general population (standardized incidence ratio, 500; 95% confidence interval, 300-781) (P<.001). The patients' age ranged from 15 to 49 years (median, 31 years), and there was a 2:1 female predominance. Surgical treatment was well tolerated (n = 17); however, radiation therapy and chemotherapy were associated with significant morbidity and mortality. Of the 19 patients, 10 (53%) developed locoregional recurrences within a median of 16 months from diagnosis. The median follow-up was 29 months. The 2-year disease-specific, overall, and relapse-free survival rates were 49%, 49%, and 42%, respectively. The cumulative incidence of relapse by the age of 40 years was 50%. CONCLUSIONS: In patients with FA, there is a high incidence of aggressive HNSCC at a young age. Surgery remains the mainstay of treatment because patients with FA tolerate radiation therapy and chemotherapy poorly, with significant morbidity. An increased understanding of FA-associated malignancies is not only important in the clinical management of patients with FA but can also elucidate the role of chromosomal instability in the development of HNSCC in general

OBJECTIVE: To evaluate the benefits of cochlear implantation in infancy and compare them to those obtained in children implanted at a slightly older age. MATERIAL AND METHODS: Using standard language measurement tools, including the Grammatical Analysis of Elicited Language--Presentence Level (GAEL-P) and the Reynell Developmental Language Scales, progress was documented in a child who received a cochlear implant in infancy and compared to that achieved in children implanted at older ages. A new measurement tool, the Visual Habituation Procedure, was used to document early skills and the results were compared to those obtained in normal-hearing infants. RESULTS: By the age of 2 years the subject implanted in infancy achieved scores on the GAEL-P which were nearly equivalent to those achieved at the age of 5 1/2 years by children implanted at later ages. Age-equivalent scores on the Reynell Developmental Language Scales were achieved by the subject implanted in infancy and the ability to discriminate speech patterns was demonstrated using the Visual Habituation Procedure. CONCLUSION: This report demonstrates enhanced language development in an infant who received a cochlear implant at the age of 6 months

Ameloblastic carcinoma is a rare primary tumor of the maxillofacial skeleton with a distinct predilection for the mandible. These lesions may initially show histologic features of ameloblastoma that dedifferentiate over time. Other ameloblastic carcinomas initially present with morphologic features suggestive of ameloblastoma with areas of epithelial dedifferentiation. We herein report a rare case of aggressive ameloblastic carcinoma in a 22-year-old white man who developed widespread bony metastases and expired 4 years after initial diagnosis. (Am J Otolaryngol 2003;24:64-69

BACKGROUND AND OBJECTIVES: Marginal mandibulectomy (MM) has been shown to provide an oncologically sound technique with preservation of function and cosmesis. We reviewed our experience with MM and analyzed oncologic and nononcologic complications. PATIENTS AND METHODS: Retrospective review of patients, with clinical and/or radiological evidence of tumor attached to the mandible without cortical erosion, who underwent vertical, horizontal, or oblique marginal mandibulectomy. Data collection included demographics; tumor characteristics; clinical and radiologic relation to the mandible; surgical technique, with attention to neck dissection and facial artery ligation; radiation therapy; osteoradionecrosis (ORN); fractures; and recurrence. RESULTS: Twenty-five patients underwent MM. Primary tumors included oral cavity (22), oropharynx (1). and metastatic neck tumor abutting the inferior/lateral border of the mandible (2). Two patients had local recurrence. Both had close soft tissue margins. Local control rate was 92%. Of 11 patients who had postoperative radiation to the primary site, 4 (36.4%) developed ORN, resulting in fractures in the mandibulectomy site in 2 of them. Two other patients developed early postoperative fractures: 1 patient had had previous radiation, and 1 patient had neck dissections with bilateral facial artery ligation and periosteal stripping. This poor technique led to necrosis of the remaining anterior mandible even before starting radiotherapy. Salvage segmental mandibulectomy was required in both patients. CONCLUSIONS: MM provides an oncologically sound approach to tumors abutting the mandible. Careful attention to preservation of the remaining periosteum and facial arteries will prevent immediate postoperative complications. However, ORN is an important long-term complication that should be taken into account

BACKGROUND: Treatment options for unilateral glottic carcinoma include radiation therapy, partial laryngectomy, and endoscopic cordectomy. We used partial laryngectomy with imbrication laryngoplasty (PLIL) for definitive treatment with curative intent in a select group of patients. STUDY DESIGN: Retrospective multicenter review of 24 patients treated with PLIL. Data collection included demographics, tumor characteristics, time to decannulation, time to oral food intake, local control, survival, voice result as judged by the physician, voice result as judged by the patient, and patient satisfaction. SETTING: Five academic medical centers. METHODS: PLIL includes a composite resection of the entire vocal fold, with its ligament, muscle, adjacent paraglottic tissues, and the adjacent block of thyroid cartilage. A neocord is reconstructed by imbricating the remaining thyroid cartilage strips and covering them with a false vocal fold flap. RESULTS: A total of 24 patients (T1, n = 13; T2, n = 10; and T3, n = 1) underwent PLIL. Median time to decannulation was 4 days, and median time to oral food intake was 5 days. Clear margins were achieved in 23 patients (96%). Follow-up ranged from 1 to 11 years (median duration of follow-up, 5.5 years). In the patients who had clear margins at the initial surgery, the rate of overall disease control was 100%. Voice quality was judged by the physician as good or excellent in 100% of the patients who underwent PLIL, and as better than typical hemilaryngectomy in 23 patients (96%). Twenty-three patients (96%) were satisfied with their voice quality. CONCLUSIONS: PLIL provides us with a single modality curative approach to unilateral glottic carcinoma. It also provides rapid recovery of oral and/or nasal airway and swallowing, excellent voice quality, and a disease-control rate similar to or better than other treatment modalities

Recurrent aphthous stomatitis (RAS) is the most common oral mucosal disorder found in men and women of all ages, races, and geographic regions. There are three forms of the lesions (minor, major, and herpetiform), with major aphthous ulcers causing significant pain and potential for scarring. In HIV-infected individuals, these ulcers occur more frequently, last longer, and produce more painful symptoms than in immunocompetent persons. In addition, they may be associated with similar ulcerations involving the esophagus, rectum, anus, and genitals. The diagnosis of HIV-induced RAS requires a careful history of the condition, and a thorough extra- and intra-oral examination. Oral mucosal biopsies are required for non-healing ulcers in order to exclude the possibility of deep fungal infections, viral infections, and neoplasms. The cause of the ulcers in HIV-positive persons has not been elucidated--local diseases, genetic, immunologic, and infectious factors all probably play a role. The goals of current treatments are to promote ulcer healing, to reduce ulcer duration and pain while maintaining nutritional intake, and to prevent or diminish the frequency of recurrence. Initial therapy for infrequent RAS recurrences includes over-the-counter topical protective and analgesic products. Initial therapy for frequent RAS outbreaks requires topical anesthetics, binding agents, and corticosteroids. Major RAS and non-healing minor or herpetiform RAS may require intralesional corticosteroids and systemic prednisone. Second-line immunomodulators for frequent and non-healing ulcers includes thalidomide and other immunomodulators.