We report the clinical manifestation of a rare case of small cell neuroendocrine carcinoma (SNEC) originating in the right ethmoid sinus.
Among malignant tumors in the nasal cavity and sinuses, adenocarcinomas, adenoid cystic carcinomas and mucoepidermoid carcinomas are common, while small cell carcinomas, which can be classified into small cell neuroendocrine carcinomas (SNECs), and small cell ductal carcinomas, are rare.
A 31-year-old man consulted our hospital with a mass in the right cervical region. Anterior rhinoscopy showed a tumor in the right nasal cavity. MRI showed a mass in the right ethmoid sinus, maxillary sinus and nasal cavity, which extended to the skull base, and a mass 5cm in diameter in the right cervical lymph node. We biopsied the nasal tumor, and the diagnosis was SNEC. Fine needle aspiration of the tumor in the right cervical lymph node demonstrated class IV. The serum Neuron Specific Enolase (NSF) was 9.8ng/ml. Because the tumor extended to the skull base, we did not attempt radical surgery for the primary lesion, but we performed chemotherapy. Neither cisplatin nor irinotecan hydrochloride was effective. Radiotherapy with docetaxel effectively reduced the tumor size both in the primary lesion and metastatic lymph node.
Small cell carcinomas are associated with a poor prognosis. The 3-year and 5-year survival rates are 38% and 13%, respectively from the literature, and the locoregional group is particularly statistically poorer than the local group. Although no evidence of progress has been found after the treatment in the case herein reported, strict observation of the tumor's progress is required.
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