Sinonasal Carcinomas with Neuroendocrine Features: Histopathological Differentiation and Treatment Outcomes.

Abstract

Objectives Sinonasal cancers with neuroendocrine features share similar clinical, radiological, and histopathological features; however, these tumors often exhibit varying degrees of aggressive behavior presenting significant treatment challenges. The objective of this study was to report our experience with these rare malignancies and to present a review of current literature. Methods Following institutional review board approval, the records of all patients with biopsy-proven sinonasal malignancies over a 5-year period were reviewed. Results The study included 14 patients with olfactory neuroblastomas (ONBs), 7 patients with sinonasal undifferentiated carcinomas (SNUC), and 2 patients with sinonasal neuroendocrine carcinomas (SNEC). Histopathologic markers aided in final diagnosis, but showed variable specificity. In patients with sufficient follow-up, the 2-year disease-free survival rate was 81% (9/11) for ONB and 75% (3/4) for SNUC. Three patients developed a regional or distant recurrence (two with ONBs and one with SNUC). Two patients, one with SNEC and one with ONB, succumbed to brain radionecrosis related to proton radiation therapy. Conclusions Overlapping clinical and histopathological features in poorly differentiated sinonasal cancers with neuroendocrine features continue to present a diagnostic challenge. Individualized assessment and treatment strategies can improve the accuracy of the initial assessment and the treatment outcomes.