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 metastases-free 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 (29-67) 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.