Olfactory Neuroblastomas: What Actually Happens in the Long-Term?

Objective: The aim of this study was to investigate the long-term oncologic outcome and review the state of the art in the management of olfactory neuroblastomas. Material and Methods: The records of all patients treated for olfactory neuroblastomas in two academic departments between 1975 and 2012 were evaluated retrospectively. Data on epidemiological parameters were collected (age, gender), along with staging (Kadish, Morita), histologic grading (Hyams), time and form of treatment, locoregional control, and disease-specific and overall survival. Patients with other malignant diseases, distant metastases of olfactory neuroblastomas at the time of initial diagnosis, a follow-up time of less than 5 years, or insufficient clinical-pathological data were excluded from further analysis. Results: In total, 53 cases made up our final study sample (26 men, 27 women; male-female ratio 0.96:1). Their mean age was 48.6 years (range: 10-84 years). The mean follow-up time was 137.5 months (4-336 months, SD: 85.0). A total of 5 out of 53 study cases (9.4%) showed metastatic involvement of the neck at the time of initial presentation. Local recurrence was detected in 8/53 (15.1%) and regional recurrence in 7/53 of our study cases (13.2%). Three patients (42.8%) from the group of cases with surgery as the sole form of management (7/53, 13.2%) died due to the disease. The cumulative disease-specific survival and overall survivalfor the whole group of patients were 88.6% and 63.6%, respectively. The cumulative disease-specific survival stratified by Kadish A/B vs. Kadish C/D as well as Hyams I/II vs. Hyams III/IV showed superior results for limited tumors, albeit without significance, and low-grade tumors (highly significant difference). Conclusion: Craniofacial or sometimes solely endoscopically controlled resection can warrant resection of the olfactory neuroblastoma with wide margins. However, locoregional failures and distant metastases can occur after a long period of time. The non-negligible incidence of regional recurrences, partly in unusual localizations, leads us to consider the need to identify the "recurrence-friendly" cases and to perform individualized elective irradiation of the neck in cases with high-risk features.