The diffuse neuroendocrine system continues to be an enigmatic topic of study in pathology due to its controversial embryologic origins, biology and a variety of tumors engendered. Originally thought to be localized to the classic neuroendocrine organs (pituitary, thyroid, pancreas and adrenal medulla), the neuroendocrine cells are now known to be distributed in every organ system of the body. A number of human diseases have been linked to aberrations in the functioning of the neuroendocrine cells. Neoplasms of the neuroendocrine system can thus occur in myriad primary sites and range in behavior from benign to lethal. Small cell neuroendocrine carcinoma (SNEC) is a high-grade neuroendocrine tumor, rarely presenting in the sinonasal region. This article reports a case of a 68-year-old male patient with primary paranasal SNEC showing intraoral involvement. The diagnosis is based on a thorough clinical, histopathological and immunohistochemical workup to differentiate it from the other small round blue cell tumors.
Keywords: Neuroendocrine tumors; small cell neuroendocrine carcinoma; small round blue cell tumors.