Sinonasal Neuroendocrine Carcinoma: 15 Years of Experience at a Single Institution

Objectives: Sinonasal neuroendocrine carcinomas (SNEC) are among the rarest paranasal sinus cancers. Consensus guidelines for therapy are difficult to develop due to limited data regarding the natural history and successful treatment of these tumors. This study presents 15 years of experience treating SNEC at a single institution and a review of the literature.

Design: Present study is a retrospective review.

Setting: The study was conducted at an academic medical center in the United States.

Participants: Patients diagnosed with primary sinonasal neuroendocrine carcinoma.

Main Outcome Measures: Primary outcome measure was overall survival.

Results: Thirteen patients were identified and included. Overall estimated survival was 72.7% at 5 years. Ten of 13 (76.9%) patients were diagnosed with high-grade neuroendocrine carcinoma and three (23.1%) with intermediate or low grade. All three patients with low- or intermediate-grade cancer survived more than 10 years from their initial diagnosis (median survival: 10.8 years) and are currently alive. The four patients who died had high-grade carcinoma, and estimated overall 5-year survival for all patients with high-grade carcinomas was 62.5%. Four of six patients who completed primary chemoradiation had residual tumor. All had high-grade carcinoma and underwent salvage resection with negative margins. Two of these four (50%) are alive without disease, now 3.7 and 5.9 years after diagnosis.

Conclusion: With an aggressive, multimodal treatment strategy, outcomes in our institution have been better than many recent case series and reviews. As with most of the existing case series, our cohort is small, and further studies will be required to determine an optimal treatment methodology for these patients; however, our approach of surgical resection as primary therapy for low-grade tumors, and primary CRT for high-grade SNEC has been successful, and could indicate hope for improved survival among these patients.

Fig. 1 Estimated overall survival.

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
12 February 2021