Small cell neuroendocrine carcinoma of nose and paranasal sinuses: The Shaukat Khanum Memorial Cancer Hospital experience and review of literature.

Small cell neuroendocrine carcinoma (SCNEC) is a rare disease of nose and paranasal sinuses. In contrast to other regions SCNEC of this region has been reported to be recurrent and locally aggressive. No definite treatment has been established till date because of rarity of this disease. The purpose of this descriptive study is to present the series of 8 cases with SNEC of nose and paranasal sinuses. Retrospective review of 8 patients presenting with Small cell neuroendocrine carcinoma of nose and paranasal sinuses, from January, 2005 to December, 2014 treated at Shaukat Khanum Memorial Cancer Hospital & Research Centre, Lahore was performed to determine the clinical characteristics and outcome of this disease. The subjects were 7 males and 1 female with a mean age of 45±15 years (range 24 to 59 years). Tumours were present in nasal cavity (n=4), ethmoid sinus (n=3) and maxillary sinus (n=1). Almost 50% of patients presented with AJCC stage IV-A. All patients had immunohistochemistry proven diagnosis. All patients (08) received radiotherapy with mean doses 58±Gy (Range 54-66Gy). Surgery was performed in 2 while chemotherapy was administered in 4 patients. Recurrence occurred in 3 patients, one each with loco-regional, distant and both. At a median follow up of 38 months, 5 patients were alive with no evidence of disease. SCNEC is a rare but aggressive neoplasm. Current standard of care varies but multi-modality approach should be the cornerstone in management of SCNEC. Early diagnosis and intervention improve the final outcome.