Advances in the Research on Small Cell Neuroendocrine Carcinoma of Paranasal Sinuses

Small cell neuroendocrine carcinoma (SNEC) of paranasal sinuses is uncommon. The histogenesis is not fully defined. Presence of neurosecretory granules and tumor cell affinity for silver stains are features suggestive of neuroendocrine differentiation. It's also reported to be related to the existence of accessory salivary gland. Some researches support the hypothesis that the extrapulmonary SNEC might be derived from pluripotent stem cells. The clinical manifestations are non-specific, mainly are rhinological syndrome and ophthalmic signs. Histology, immunocytochemistry and electron microscopy are necessary in the diagnosis of SNEC of paranasal sinuses. It should be differentiated from poorly differentiated squamous carcinoma, melanoma, olfactory nerve blastoma and neurospongioma. There's no standard treatment plan, and 2003 French protocol was a successful one. Reccurrence is frequent and the prognosis is poor. Combined therapy can help improve the patient survival.