Chemotherapy for advanced esthesioneuroblastoma: The Mayo Clinic experience

OBJECTIVE: Esthesioneuroblastoma (olfactory neuroblastoma) is a rare neuroendocrine tumor that arises in the upper nasal cavity from the olfactory epithelium. Little information is available regarding the treatment of these tumors with chemotherapy in the advanced setting. A retrospective review of patients with recurrent esthesioneuroblastoma treated with chemotherapy between 1970 and 1995 at the Mayo Clinic was undertaken to gain more information regarding the efficacy of chemotherapy treatment for these patients. METHODS: Ten patients were identified using a computerized data base available at this institution. The clinical and pathological materials, when available, were reviewed, and each tumor reviewed was assigned a Hyams' grade. RESULTS: There were six men and four women, ranging in age from 22 to 74 years, all of whom had assessable Kadish Stage C disease at the time of chemotherapy treatment. The chemotherapy regimens and clinical follow-up varied during this 25-year time span. The only tumor regression resultant from chemotherapy was observed in patients with high-grade tumors. Two of four patients with high-grade tumors obtained regression from first-line, platinum-based chemotherapy, with a mean duration of regression of 9.3 months (range, 2-13 mo). Survival time from initial diagnosis was 139.5 months (range, 83-168 mo) in patients with low-grade tumors and 32.2 months (range, 5-84 mo) in patients with high-grade tumors. Survival from initial chemotherapy treatment was 44.5 months (range, 3-130 mo) in patients with low-grade tumors and 26.5 months (range, 2-67 mo) in patients with high- grade tumors. CONCLUSION: Hyams' grading of esthesioneuroblastoma tumors seems to be important in predicting response to chemotherapy. Despite sensitivity to platinum-based chemotherapy, patients with high-grade tumors in this series had a much more aggressive course than did those with lower- grade tumors. This series suggests that cisplatin-based chemotherapy is active in advanced, high-grade esthesioneuroblastoma and is a reasonable choice in the systemic treatment of these patients.