Olfactory neuroblastoma: long-term clinical outcome at a single institute between 1979 and 2003.

Abstract

Conclusions: The progression of olfactory neuroblastoma showed a biphasic pattern. As well as Hyams' histopathological grading and neck metastasis at presentation, early phase recurrence should be regarded as an important prognosticator. A high local failure rate suggests that craniofacial resection followed by postoperative radiotherapy should still be the standard treatment for olfactory neuroblastoma.

Objective: The aim of this study was to evaluate factors associated with survival and local control of olfactory neuroblastoma in the long run and to estimate treatment strategies.

Patients and methods: Twelve patients (seven men and five women) who had undergone initial curative treatment for olfactory neuroblastoma were retrospectively analyzed.

Results: Cause-specific 10-year survival was 64.8%, while disease-free 10-year survival remained 28.6%. Local failure was found in half of the patients. All of the three patients who did not receive radiotherapy developed local failure. A biphasic pattern of recurrence was observed. The early phase recurrence group showed a significantly poorer survival than the late phase recurrence group. Hyams' histopathological grading and neck metastasis at presentation were also correlated with survival.