Esthesioneuroblastoma: A single institution's experience and general literature review

Olfactory neuroblastoma or esthesioneuroblastoma is a rare entity among head and neck neoplasms. In this paper, we report the experience of our institution and compare it with a comprehensive review of the literature. We retrospectively analysed clinical and treatment data of patients referred to the Lyon Sud University Hospital (France) for histologically proven olfactive esthesioneuroblastoma. Ten patients treated between 1993 and 2015 have been analysed. Disease stage at diagnosis, according to the Kadish staging system, was C in 90% of cases. Median follow-up was 136 months. Ten-year overall survival was 90%. Five- and ten-year progression-free survival were 70% and 50%. Nine patients (90%) underwent surgical resection first. Seven of the nine patients who underwent resection (77%) received adjuvant three-dimensional (3D)-conformal radiotherapy (n=7), intensity-modulated radiotherapy (n=1), or volumetric arctherapy (n=1). The mean dose to the tumour volume was 61Gy. None of the patients received elective nodal irradiation. Two patients received concurrent chemotherapy. Five patients (50%) presented with disease recurrence, which was local (n=1), nodal (n=2) and cerebral (n=2). Our results are consistent with the literature. Because of the lack of prospective study and the low number of cases in the literature, each institution's experience is of the utmost important to improve standardised management of these tumours.