Management of nonesthesioneuroblastoma sinonasal malignancies with neuroendocrine differentiation.

Objectives/Hypothesis: Report the outcomes of patients with nonesthesioneuroblastoma (non-ENB) sinonasal malignancies with neuroendocrine differentiation. Study Design: Single Institution Retrospective Case Series. Methods: We conducted a retrospective chart review of 19 biopsy-proven non-ENB sinonasal neuroendocrine carcinomas diagnosed between 1997 and 2010 and treated with multimodality therapy. Kaplan-Meier estimates of local-regional control (LRC), distant metastasesfree survival (DMFS), and overall survival (OS) were calculated. Results: The 3-year LRC, FFDM, and OS rates were 63%, 50%, and 64%, respectively. All nine patients receiving neoadjuvant chemoradiotherapy (CRT) had at least a pathologic partial response: three of seven sinonasal undifferentiated carcinomas and two of two sinonasal neuroendocrine carcinomas had complete pathological responses. All five patients with pathologic complete responses were alive without disease at a median follow-up of 43 (2967) months. Conclusions: Having a complete response pathologically to CRT was a favorable prognostic indicator. Neoadjuvant CRT followed by surgery seems to be an efficacious sequence of multimodality therapy.