Chang S E, Lee T J, Ro J Y, Choi J H, Sung K J, Moon K C, Koh J K
Department of Dermatology, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea.
J Dermatol. 1999 Jun;26(6):363-7. doi: 10.1111/j.1346-8138.1999.tb03489.x.
We report a case of cellular neurothekeoma showing unusual immunohistochemical findings and occurring on the left upper arm of a healthy 48-year-old woman. She presented with a 1 cm, red, asymptomatic, dermal nodule of 1 year duration. A biopsy showed dermal proliferation of plexiform fascicles of spindle-shaped or polygonal cells with plentiful eosinophilic cytoplasms. The immunohistochemical profile included negative stains for S-100, CD34, factor XIIIa, CD68, HMB45, cytokeratins, and EMA, strongly positive stains for neuron specific enolase (NSE), synaptophysin, and chromogranin A, and focally positive ones for NKI/C3 and alpha-smooth muscle actin. Ultrastructural analysis showed undifferentiated mesenchymal cells with cytoplasmic projections and abundant RER. Although we couldn't find any confirmative cell type in this cellular tumor, we believe that cellular neurothekeoma is predominantly composed of undifferentiated cells that can exhibit features of neuroendocrine cells in addition to fibroblastic or myofibroblastic ones, suggesting a divergent cell origin.
我们报告一例细胞性神经鞘黏液瘤，该病例具有不寻常的免疫组化表现，发生于一名48岁健康女性的左上臂。她出现一个1厘米大小、红色、无症状的皮肤结节，病程1年。活检显示真皮内梭形或多角形细胞呈丛状束状增生，细胞质丰富且嗜酸性。免疫组化结果包括S-100、CD34、因子XIIIa、CD68、HMB45、细胞角蛋白和EMA染色均为阴性，神经元特异性烯醇化酶（NSE）、突触素和嗜铬粒蛋白A染色呈强阳性，NKI/C3和α-平滑肌肌动蛋白染色呈局灶性阳性。超微结构分析显示未分化的间充质细胞，有细胞质突起和丰富的粗面内质网。尽管在这个细胞性肿瘤中我们未能找到任何确诊的细胞类型，但我们认为细胞性神经鞘黏液瘤主要由未分化细胞组成，这些细胞除了具有成纤维细胞或肌成纤维细胞特征外，还可表现出神经内分泌细胞的特征，提示其细胞起源具有异质性。
