Olfactory neuroblastoma [esthesioneuroblastoma]: a retrospective review of 21 cases

Introduction

Despite the presence of non-neuroendocrine tumors with neuroendocrine characteristics within the sinonasal region, neuroendocrine tumors [NET] may be roughly separated into those derived from neuroectodermal origin, olfactory neuroblastomas [ONB], and those of epithelial origin, neuroendocrine carcinomas. A comprehensive review of 21 cases of ONB is presented.

Materials and Methods

Retrospective search of OHSU, Dept. of Pathology records [1990-2020] ICD codes for ONB/esthesioneuroblastoma, focusing on established inclusion criteria. Statistical analysis was performed using Kaplan–Meier method and the log-rank test. Statistical significance was considered at P < .05.

Results

38.10% presented with Hyams grade I, 33.33% grade II, 9.52% grade III, and 19.05% grade IV. Most patients presented with modified Kadish stages III and IV [42.86%], followed by stages I [4.76%] and II [9.52%]. The majority of patients were managed by surgery + RT/IMRT, followed by surgery + RT/IMRT + chemo and surgery only. For patients with Hyams grade I, the 5- and 10-year recurrence rates were compared across Kadish staging: stage I [0%, 25%], stage II [14.3%, 48.6%], stage III and IV [50%, 100%]; and the 100% survival rate dropped to 83.1% at 10 years.

Conclusion

The current study highlights significant prognostic differences between different Hyams grades [P = .0384] but did not show the same for modified Kadish tumor stages [P = .3432], suggesting that Hyams grading was more predictive of patients’ outcomes when compared to Kadish staging.