Small Cell Neuroendocrine Carcinoma of Ethmoid Sinus: Case Report and Literature Review

Abstract

Malignant tumors arising in the paranasal sinuses account for 0.2 to 0.8% of all cancers. Squamous cell carcinoma is by far the most common malignant tumor. Primary small cell neuroendocrine carcinoma of ethmoid sinus is an uncommon and distinctive tumor with an aggressive clinical behavior. Besides, its morphological and immuno-histo-chemical features are similar to those of small cell carcinoma of lung. The need for a multi-disciplinary treatment approach combining surgery or radiotherapy and chemotherapy is indicated in treating these aggressive tumors. We report a case of 40-year-old man who has small cell neuroendocrine carcinoma of ethmoid sinus with invasion of upper aspect of right maxillary sinus and medial aspect of right orbit. The patient was treated with surgery and adjuvant radiotherapy followed by systemic chemotherapy. Neither local recurrence nor distant metastasis was noted during follow-up of 6 months. The optimal treatment and the immuno-histo-chemical characteristics are discussed.