论文部分内容阅读
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 investigate the clinicopathological features and differential diagnosis of skin nerve sheath myxoma. Methods The pathological features and immunohistochemical features of 5 cases of cutaneous nerve sheath myxoma were observed and reviewed. Results In 5 children with cutaneous nerve sheath myxoma, the age of onset was from 2 months to 13 years with a mean age of 4.4 years. Macroscopic observation of tumor non-enveloped, section gray brown, gray-black, medium quality, some mucus-like. Microscopic tumor cells were significantly divided into lobular arrangement, there are fibrous interlobular septa, lobular mass mucus, tumor cells was star-shaped or fusiform, rare cell abnormalities and mitotic. Immunohistochemistry: PGP9.5, NSE, CD34 and vimentin both (+), calponin, HMB45 and Melan-A fraction (+), while S-100 (-). Histochemistry: Sulphated mucus in the interstitial of the tumor was observed by staining with Alcian blue (pH 1.0), acid mucopolysaccharide was found in the interstitial of the tumor with Alcian blue (pH2.5) staining, Masson trichrome staining Collagen basement membrane-like substance. Postoperative follow-up prompts good prognosis, no recurrence and metastasis signs. Conclusion Pathological morphological features combined with immunohistochemistry, special staining helps to clarify the diagnosis of skin sheath sclerosis.