Sinonasal Small Cell Neuroendocrine Carcinoma: a Systematic Review of 80 Patients

Background: The objective of this study was to review the published literature related to sinonasal small cell neuroendocrine carcinomas (SCNECs). Clinical presentation, demographics, treatment, and outcomes of this uncommon disease are reported.

Methods: A systematic review of studies for sinonasal SCNECs in PubMed and Ovid databases from 1970 to 2014 was conducted. Bibliographies of selected articles were also examined. Articles were examined for patient data that reported disease outcome.

Results: Thirty-four articles were included in this analysis, comprising a total of 80 cases. The mean age of presentation was 53.0 years. Nasal obstruction (53.8%) and epistaxis (48.8%) were the most common presenting symptoms. The nasal cavity and septum (32.5%) was the most common site of involvement. Seventy percent of patients presented with American Joint Committee on Cancer (AJCC) stage IV disease. Combination chemotherapy and radiation therapy was the most common treatment modality, used in 21 cases (26.3%). The second and third most common treatment modalities was combination surgery and chemoradiation therapy (21.3%), and surgery alone (18.8%), respectively. A total of 37 patients (46.3%) were alive after a mean follow-up of 30.8 months (median 15.5 months), independent of treatment modality. Of the treatment modalities, multimodality therapy remained the most common therapy.

Conclusion: This review contains the largest pool of sinonasal SCNEC patients to date. Sinonasal SCNEC is a rare and aggressive neoplasm, and there is currently no standard of care for treatment. Various treatment modalities have been employed. Our systematic review suggests that multimodality therapy remains the most common treatment of sinonasal SCNEC.