Abstract
The aim of this meta-analysis was to provide treatment guidelines for sinonasal neuroendocrine carcinoma (SNC) by combining all available data in the literature.A literature search for all studies concerning SNC was performed against the MEDLINE and EMBASE databases. Available clinical data was normalized, pooled, and statistically analyzed.A total of 701 cases of SNC were available for analysis, comprising 127 well or moderately differentiated sinonasal neuroendocrine carcinomas (SNEC), 459 sinonasal undifferentiated carcinoma (SNUC) and 115 sinonasal small cell carcinoma (SmCC). Tumor type was the most important predictor of survival, with a 5-year disease-specific survival (DSS) of 70.2% for SNEC, 35.9% for SNUC and 46.1% for SmCC. Tumor stage on presentation was of limited value in predicting survival or response to treatment. Overall, the application of surgery yielded significantly better results (5-year DSS 52.2% versus 30.1%, p<0.001). In SNUC, radiotherapy was a beneficial supplement to surgery (5-year DSS 54.7% versus 15.7%, p=0.027), while radiotherapy as monotherapy performed poorly (5-year DSS 17.9%). Chemotherapy did not appear to contribute to survival.Based on these findings, we can conclude that the most important predictors of survival in SNC are differentiation grade and the associated choice of treatment modality. In contrast to other head and neck cancers, tumor staging appears of limited value in predicting survival or deciding on a treatment strategy. Surgery should be the cornerstone of treatment, supplemented by radiotherapy in poorly differentiated subtypes (SNUC, SmCC). Chemotherapy does not appear to contribute to survival.
Highlights
Sinonasal tumors with neuroendocrine differentiation are a rare group of neoplasms that account for only 5% of all sinonasal malignancies [1]
The tumor stage on presentation was stage IV in 75.0% of cases. This distribution significantly differed amongst tumor types, with well or moderately differentiated sinonasal neuroendocrine carcinoma (SNEC) presenting with stage IV in 57.1% of cases, small cell carcinoma (SmCC) in 70.4% and sinonasal undifferentiated carcinoma (SNUC) in 80.6% (p < 0.001)
It is clear that a proper histological diagnosis with emphasis on differentiation grade is of paramount importance in predicting prognosis and treatment response in sinonasal neuroendocrine carcinoma (SNC)
Summary
Sinonasal tumors with neuroendocrine differentiation are a rare group of neoplasms that account for only 5% of all sinonasal malignancies [1]. A broad distinction is made between tumors of neuroectodermal origin - esthesioneuroblastoma - and those of epithelial origin - sinonasal neuroendocrine carcinoma (SNC). Latter can be subdivided based on differentiation grade into well, moderately and poorly differentiated SNC. Differentiated SNC are further subdivided into a small and large cell variants. In contrast to well and moderately differentiated SNC, large cell poorly differentiated SNC are denoted by sinonasal undifferentiated carcinoma (SNUC) and small cell poorly differentiated SNC by sinonasal small cell carcinoma (SmCC), discounting their neuroendocrine nature. In order to prevent further ambiguity, well and moderately differentiated SNC are referred to by their common abbreviation, SNEC, in this article
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
