皮肤原发性外周原始神经外胚叶肿瘤  

Primary cutaneous peripheral primitive neuroectodermal tumor

作　　者：熊晓刚[1] 刘业强[1] 陈文彬[2] 徐晓[3] 雷晴[1] 张英[1] 胡仕宏[1] 张萍[1] 宋秋荷[1] 童务华[1] 汪莹[1] 卢军[1] 余泽莹[1] 陈方元[1] 

机构地区：[1]九江学院附属医院皮肤科,江西九江332000 [2]九江学院附属医院肿瘤科,江西九江332000 [3]九江学院附属医院病理科,江西九江332000

出　　处：《临床皮肤科杂志》2011年第9期521-524,共4页Journal of Clinical Dermatology

基　　金：江西省卫生厅重大项目基金(20084012)资助项目

摘　　要：报告1例发生于皮肤的原发性外周原始神经外胚叶肿瘤。患者女,65岁。躯干、大腿内侧多发性丘疹、结节3个月。皮损组织病理检查:表皮大致正常,表皮与瘤团间有境界带,肿瘤深达皮下,片状生长,由深染的小至中等大的圆细胞组成,瘤细胞胞核深染,核仁明显,胞质淡染,细胞排列拥挤,个别细胞有异常核分裂象。免疫组化检查:CD99、波形蛋白(vimentin)、神经特异性烯醇化酶(NSE)均阳性。电子显微镜检查:瘤细胞内有神经内分泌颗粒。根据患者临床表现和组织病理、免疫组化及电镜检查结果,诊断为皮肤原发性外周原始神经外胚叶肿瘤。Objective： To study the clinicopathological features, differential diagnosis, therapy and prognosis of primary cutaneous peripheral primitive neuroectodermal tumor （pPNET）. Methods： the clinical data, histopathology examination, immunohistochemical stain, transmission electron microscopic feature and relevant reference of pPNET were analyzed. Results： A 65- year-old woman had a 3-month history of multiple papules and nodules on the trunk and inner thighs. Physical examination revealed there were 2-12 mm, tender, firm, dark red, round, dome-shaped and smooth papules and nodules, concentrated on the trunk and inner thighs. Histopathology revealed epidermis was normal, there was a boundary band between epidermis and tumor. The tumor was composed of cohesive sheets of small to medium sized round ceils containing hyperchromatic nuclei with small nucleoli and scanty pale or clear cytoplasm with indistinct cytoplasmic borders. Some cells had atypical mitotic figures. Immunohistochemical stain showed that CD99,Vimentin and NSE were positive. Transmission electron microscopy showed that neuroendocrine granules detected in the cells of the tumor. The clinical, pathological, immunohistochemical and transmission electron microscopic feature of the case support the diagnosis of pPNET. Conclusion： pPNET is a malignant, small round cell sarcoma exhibiting primitive neuronal differentiation which rarely involves the skin.

关 键 词：外周神经外胚叶肿瘤 原发性 皮肤 

分 类 号：R739.5[医药卫生—肿瘤]