儿童皮肤神经鞘黏液瘤5例临床病理分析  

Clinicopathologic features of neurothekeoma in children:a study of five cases

作　　者：曹娟[1] 文飞球[2] 徐金永[1] 李红[1] 王玲[1] 

机构地区：[1]重庆医科大学附属深圳市儿童医院病理科,广东深圳518026 [2]重庆医科大学附属深圳市儿童医院血液肿瘤科,广东深圳518026

出　　处：《诊断病理学杂志》2013年第7期390-393,共4页Chinese Journal of Diagnostic Pathology

基　　金：深圳市科技计划项目(201102071);广东省自然科学基金(S2011010004349)

摘　　要：目的探讨皮肤神经鞘黏液瘤的临床病理学特征及鉴别诊断要点。方法对5例皮肤神经鞘黏液瘤进行病理形态和免疫组化特点观察,并进行文献复习和讨论。结果 5例皮肤神经鞘黏液瘤患儿,发病年龄2个月～13岁,平均年龄4.4岁。肉眼观察肿瘤无包膜,切面灰褐、灰黑色,质中等,部分呈黏液状。镜下瘤细胞呈明显分叶状排列,小叶间有纤维性间隔,小叶内有大量黏液,瘤细胞呈星状或梭形,罕见细胞异型及核分裂象。免疫组化:PGP9.5、NSE、CD34和vimentin均(+),calponin、HMB45和Melan-A部分(+),而S-100(-)。组织化学:阿尔辛蓝(pH1.0)染色显示肿瘤间质内含有硫酸黏液,阿尔辛蓝(pH2.5)染色显示肿瘤间质内含有酸性黏多糖;Masson三色染色显示肿瘤内含Ⅳ型胶原基底膜样物质。术后随访提示预后较好,无复发和转移征象。结论以病理学形态特征结合免疫组化、特殊染色有助于明确皮肤神经鞘黏液瘤的诊断。Objective To explore the pathological and clincal characteristics of neurothekeoma.Methods The histopathological changes and immunohistochemical expression of five cases were investigated and the relevant literature was reviewed.Results Five patients ranged in age from 2 month to 13 years and mean ages was 4.4 years old.Generally,it had a unclear edge with grey or black cut surface and moderate texture.A portion of the tumor showed mucoid.Microscopically,the tumor cells showed lobulated arrangement,interlobular fibrous septum,a large number of mucus within the loule.The tumor cells were stellate or spindle-shaped.Cell atypia and mitotic figures were rare.Immunohistochemical study showed that neurothekeoma was positive for PGP9.5,NSE,CD34 and Vimentin,partially positive for Calponin,HMB45 and Melan-A,and negtive for S-100.Allie New Zealand（pH1.0） staining indicated that the tumor stroma contained sulfuric acid mucus.The mucin was connective tissue type acidic mucopolysac charide and stained strongly with Allie New Zealand（pH2.5）.Masson trichrome staining indicated that the tumor presented with type Ⅳ collagen in basement membrane-like material.Long term follow-up showed the neurothekeoma cases had a favourate prognosis after tumor excision.Conclusion It is helpful for diagnosis to master its histopatholigical characteristics combined with immunohistochemistry and special staining.

关 键 词：皮肤神经鞘黏液瘤 皮肤肿瘤 临床病理 

分 类 号：R739.5[医药卫生—肿瘤]