副鼻窦小细胞神经内分泌癌的研究进展

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

副鼻窦小细胞神经内分泌癌极为罕见,发病机理不明,神经内分泌颗粒的存在提示其神经内分泌分化特征,也有报道与副涎腺的存在有关,部分基础研究显示其可能来源于多能干细胞。临床症状无特异性,以鼻腔和眼科症状为主,诊断主要取决于组织病理学表现、免疫组化结果,以及电镜下超微结构特征。病理学方面需与低分化鳞癌、黑色素瘤、嗅神经母细胞瘤和神经胶质瘤等相鉴别。治疗并无统一方案,目前综合治疗具有明显疗效。肿瘤易复发,预后较差,综合治疗方案的成熟有利于改善预后。

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