Faculty Bibliography

Objectives: To report changes in survival probabilities as patients survive one or more years with head and neck melanoma, and to characterize prognostic factors for this conditional survival statistic. Study Design: Retrospective population-based cohort study. Methods: All subjects from the Surveillance, Epidemiology and End Results (SEER 17) database of the National Cancer Institute with head and neck cutaneous melanoma were analyzed. Using the life table actuarial method, conditional 5 year disease specific survival (DSS) and relative survival were determined for patients surviving one to ten years after diagnosis. Grouped comparisons were performed for anatomic subsites within the head and neck and the rest of the body. Probabilities were also stratified by histologic subtype, thickness of invasion and lymph node status. Results: Five year DSS for scalp and neck melanomas increases from 83.1% to 93.9% for patients surviving five years, compared to an increase from 89.2% to 96.2% for other anatomic sites. Nodular melanoma displayed the worst initial survival among histologic subtypes (73.4%) but improves to 91% at five years. Conditional DSS for node positive patients improves from 47.9% to 83.1%. Survival at diagnosis is stratified by tumor thickness from 96.7% (T1) to 62.3% (T4), but tends to converge between five to ten years of survivorship. Conclusions: For patients with head and neck melanoma who have survived several years, conditional survival provides accurate and useful prognostic information. In general, expected survival increases with time survived since diagnosis, and for low and high T-stage patients, conditional survival statistics converge over time

BACKGROUND:This study reports the combined experience of the University of Miami and University of Pittsburgh with endoscopic endonasal resection of esthesioneuroblastoma (ENB). A retrospective case series review was performed in a tertiary care university hospital. METHODS:Twenty-three patients, 16 men and 7 women, were reviewed. Mean age was 56.6 years (15-79 years). Nineteen patients received primary endoscopic endonasal anterior skull base resection. Of these, the modified Kadish stage at presentation was A in 2 patients, B in 11 patients, C in 5 patients, and D in 1 patient. Three patients had revision surgeries for recurrent tumors. The main outcome measures were complete resection and margin assessment, short-term and long-term complications, and recurrence rate. RESULTS:Complete resection and negative intraoperative resection margins were achieved endoscopically in 17 of the primarily treated cases. The two other cases had one patient that required an additional craniotomy approach to complete the resection of a positive lateral dual margin, another patient had positive margins at the orbital apex. All patients tolerated the endoscopic procedure very well with no meningitis. There were four cerebral spinal fluid leaks. Mean follow-up period for the primarily treated cases was 45.2 months (11-152 months), all were disease free at the most recent available follow-up. CONCLUSION/CONCLUSIONS:In experienced hands and carefully selected patients, endoscopic resection of ENB respects the principles of oncologic surgery, providing an adequate exposure for margin assessment as well as reliable reconstruction of the anterior skull defect with a relatively low morbidity.

Autoimmune Inner Ear Disease (AIED) is poorly characterized clinically, with no definitive laboratory test. All patients suspected of having AIED are given glucocorticoids during periods of acute hearing loss, however, only half initially respond, and still fewer respond over time.We hypothesized that AIED is a systemic autoimmune disease characterized by dysfunctional peripheral blood mononuclear cells (PBMC) responses to a unique cochlear antigen(s). To test this hypothesis, we examined end-stage AIED patients undergoing cochlear implant surgery and compared autologous perilymph stimulated PBMC from AIED patients to controls. We determined that autologous perilymph from AIED patients was unable to induce expression of a long membrane-bound Interleukin-1 Receptor Type II (mIL1R2) transcript in PBMC as compared with controls, despite similar expression of the short soluble IL1R2 (sIL1R2) transcript (p<0.05). IL1R2 is a molecular decoy that traps interleukin-1beta (IL-1beta) and does not initiate subsequent signaling events, thereby suppressing an inflammatory response. IL1R2 transcript length is regulated by alternate splicing, and the major inhibitory function is attributed to the full-length mIL1R2. In addition, IL1R2 expression is induced by dexamethasone.Separately, we prospectively examined patients with newer onset glucocorticoid-responsive AIED. Immediately prior to clinical treatment for acute deterioration of hearing thresholds, their PBMC demonstrated a robust induction of mIL1R2 in PBMC in response to dexamethasone in vitro that correlated with a clinical response to prednisone in vivo (p<0.0001) as measured by hearing restoration. In contrast, clinically steroid unresponsive patients demonstrated high basal levels of mIL1R2 in their PBMC and only minimally augmented expression in response to dexamethasone. Thus, induced expression of mIL1R2 appears to be a protective mechanism in hearing homeostasis and warrants further investigation in a large prospective clinical trial to determine if IL1R2 can be used as a specific biomarker for AIED.

Vocal cords stiffness can be associated with a variety of etiologic agents, but it is rarely seen with atypical mycobacteria, for example, Mycobacterium avium complex. We report a case of a 35-year-old white woman who is and was maintained on inhaled steroids. She presented with hoarseness and low-grade fever, but her medical history was otherwise unremarkable. Laryngoscopy revealed bilateral scarring of the vocal cords. Vocal cord biopsies were performed. Histologic examination revealed unremarkable laryngeal mucosa, except for abundant subepithelial non-necrotizing granulomata. The differential diagnosis included sarcoid, Klebsiella scleroma, and tuberculosis. Special stains reviewed abundant acid-fast bacilli, later confirmed by a DNA assay on induced deep sputum, consistent with M avium complex. Subsequently, steroids were withdrawn, and the patient was treated with a multiple-drug antituberculous regimen and had a full recovery. This unusual clinical presentation of the atypical mycobacteriosis may be encountered by otolaryngologists and pathologists, and it is critical to recognize it in patients immunocompromised because of steroids

BACKGROUND: The term "collision tumor" refers to the coexistence of two histologically distinct malignant tumors within the same mass. Collision tumors involving the thyroid gland and/or neck region are especially uncommon, with most reported cases involving papillary thyroid carcinoma in coexistence with medullary thyroid carcinoma, follicular carcinoma, and metastatic disease, the latter including a rare occurrence of metastatic liposarcoma and thyroid papillary carcinoma. A collision tumor of papillary thyroid carcinoma and squamous cell carcinoma primary to the thyroid has also been reported. SUMMARY: We now report a collision tumor comprised of well-differentiated papillary thyroid carcinoma and squamous cell carcinoma that originated as a laryngeal primary tumor. Histologically, the thyroid mass is consisted of well-differentiated papillary thyroid carcinoma. The laryngeal mass consisted of in situ and invasive squamous cell carcinoma. Both cancers were extensively infiltrative into the soft tissues of the neck and paraglottic space where the histologically distinct tumor types approximated one another but were not admixed with each other. Additionally, cervical lymph nodes showed metastatic carcinoma, including independent cervical lymph nodes with metastatic squamous cell carcinoma only, cervical lymph nodes with metastatic papillary thyroid carcinoma only, and cervical lymph nodes with foci of both papillary thyroid carcinoma and squamous cell carcinoma. CONCLUSION: As best we can determine this is the first publication of a collision tumor comprised of a well-differentiated papillary thyroid carcinoma and squamous cell carcinoma that originated as a laryngeal primary tumor. For any patient with a thyroid mass that appears to be in continuity with a laryngeal mass, more commonly one would find invasive thyroid cancer, but one must consider a collision tumor in the differential diagnosis.

OBJECTIVE: Demonstrate the benefits of a multidisciplinary pediatric airway team prepared to evaluate and treat otolaryngology patients with flexible bronchoscopy. DESIGN: Case series. SETTING: Tertiary, academic children's hospital. PATIENTS: 10 children (5 male, 5 female age range 2 months-16 years) presenting with complex symptoms potentially referable to large airways. INTERVENTION: Flexible bronchoscopy for diagnostic (bronchoalveolar lavage, ciliary biopsy, assess ongoing surgical intervention, and rule in or rule out foreign body; N=6) or therapeutic (evacuate bronchial mucus plug, laser subglottis when patient has fused cervical spine, and distal instillation [fibrin glue for bronchopleural fistula and dornase alpha for plastic bronchitis]; N=4). MAIN OUTCOME MEASURE: Retrospectively ask if flexible bronchoscopy and interdisciplinary management improved patient care in these select otolaryngology cases. RESULTS: 10/10 patients benefited from interdisciplinary management including flexible bronchoscopy. CONCLUSION: Our experience illustrates many uses for flexible bronchoscopy in otolaryngology patients, and suggests that an airway team prepared to use flexible bronchoscopy will create opportunities for improved patient care.