Esthesioneuroblastoma: The northwestern university experience

Objective. To review our experience with esthe-sioneuroblastoma, a rare malignancy of the head and neck. Study Design: Retrospective review of Tumor Registry data. Methods. We performed a computerized search of the Northwestern Memorial Hospital Tumor Registry database from 1981 to 2000. Results: Sixteen patients with esthesioneuroblastoma were identified and analyzed. Their mean age was 42 years. Eleven of 16 patients (69%) had Kadish stage C; 8 patients (50%) had brain involvement at presentation. Craniofacial resection was performed in 13 patients (81%). Fourteen patients received either preoperative or postoperative therapy; radiation therapy was employed in 11 cases and chemotherapy in 4. The actuarial 5-year survival was 60%, and the actuarial 5-year disease-free survival was 33%, with a median follow-up of 4.3 years. Recurrences occurred at a median time of 11 months after diagnosis (2.5 mo-18 y). The first site of failure was locoregional alone in 10 of 12 patients who progressed, and in 6 patients involved the brain or the meninges. Two patients were successfully salvaged. Patients with high-grade tumors had a trend toward work survival. Conclusions. Esthesioneuroblastoma is a rare tumor that is potentially curable by surgical resection and radiation therapy. However, the rate of local failure is high, and late recurrences are not uncommon. The role of chemotherapy warrants further investigation.