p63 Expression in olfactory neuroblastoma and other small cell tumors of the sinonasal tract.

Olfactory neuroblastoma (ONB) is a rare neoplasm of the head and neck region that is included in the differential diagnosis of other sinonasal tract malignancies. We studied the usefulness of using p63 as an aid in the diagnosis of ONB and other tumors of the sinonasal region. The specimens were 14 ONBs; 4 nasopharyngeal carcinomas (NPCs), nonkeratinizing subtype; 2 NPCs, undifferentiated subtype; 10 sinonasal undifferentiated carcinomas (SNUCs); 7 malignant melanomas; and 2 extranodal natural killer (NK)/T-cell lymphomas. We observed p63 expression in 5 ONBs (36%), 4 nonkeratinizing NPCs (100%), 1 undifferentiated NPC (50%), 2 SNUCs (20%); 0 malignant melanomas (0%); and 1 extranodal NK/T-cell lymphoma (50%). While all cases of NPC with positive staining for p63 showed strong and diffuse immunoreactivity, the ONB, SNUC, and lymphoma cases with positive immunoreactivity showed only focal staining for p63. No p63 expression was observed in malignant melanoma. We think p63 is a useful marker to help distinguish nonkeratinizing or undifferentiated NPC subtypes from various sinonasal tract malignancies. In particular, p63 helps distinguish nonkeratinizing and undifferentiated NPC subtypes from SNUC.

Esthesioneuroblastoma: continued follow-up of a single institution's experience.

OBJECTIVES: To analyze outcomes and to provide follow-up for our increasing patient cohort with esthesioneuroblastoma. DESIGN: Retrospective cohort analysis. SETTING: Patients were examined from September 1, 1976, to May 30, 2004, in a tertiary care academic hospital. PATIENTS: Fifty consecutive patients diagnosed as having esthesioneuroblastoma were treated with a standardized protocol during a 28-year period. Patients with tumors staged Kadish A or B received preoperative radiotherapy followed by craniofacial resection, while patients with Kadish stage C disease were treated with preoperative sequential chemotherapy and radiotherapy followed by a craniofacial resection. The mean follow-up is 93 months (range, 1-330 months). RESULTS: The disease-free survival was 86.5% and 82.6% at 5 and 15 years, respectively. There were 17 patients (34%) who developed recurrent disease, most of which was locoregional (12 patients [71%]). There was a long interval to relapse (mean, 6 years), with the longest time to regional recurrence being 10 years. Distant relapses occurred sooner, with poorer outcomes. Of these 17 patients, 7 (41%) underwent successful salvage surgery, while 3 remain alive with disease. CONCLUSIONS: Excellent outcomes for esthesioneuroblastoma are achievable. Long-term follow-up is necessary because of the extended interval for recurrent disease; unlike most sinonasal malignancies, surgical salvage is possible.