Extrapulmonary small cell neuroendocrine carcinoma of the paranasal sinuses: a case report and review of the literature

Abstract

Primary small cell neuroendocrine carcinoma of the paranasal sinuses is an extremely rare and distinctive tumor with aggressive clinical behavior. We report on a patient who had a small cell neuroendocrine carcinoma of the ethmoid sinus with invasion of the upper aspect of the right maxillary sinus and medial aspect of the right orbit. After 2 cycles of chemotherapy that were unsuccessful, he underwent radiotherapy with complete tumor remission. Local recurrence was not identified after 11 months of follow-up; however, distant metastasis developed 2 months after radiotherapy. The natural history, pathologic features, and choice of therapy for this rare neoplasm are discussed, along with a review of the published data.