Chang Y, Dehner L P, Egbert B
Department of Pathology, University of California, San Francisco Medical Center 94143.
Am J Surg Pathol. 1990 Oct;14(10):977-82. doi: 10.1097/00000478-199010000-00012.
We report an unusual case of primary cutaneous embryonal rhabdomyosarcoma presenting as a solitary skin lesion on the anterior chest of a 20-month-old child. The tumor was characterized by small, round to oval, poorly differentiated cells. Immunohistochemically, the tumor was negative for NSE, S-100 protein, LCA, and keratin but positive for muscle-specific actin, myoglobin, desmin, and vimentin, thus indicating the presence of myogenous differentiation. Ultrastructural analysis demonstrated thick and thin filaments. Special studies showed no evidence of a primary rhabdomyosarcoma in the patient at a more typical location, nor was there any evidence of metastases.
我们报告了一例罕见的原发性皮肤胚胎性横纹肌肉瘤病例,该肿瘤表现为一名20个月大儿童前胸的孤立性皮肤病变。肿瘤的特征是细胞小,呈圆形至椭圆形,分化差。免疫组化显示,肿瘤对神经元特异性烯醇化酶(NSE)、S-100蛋白、淋巴细胞共同抗原(LCA)和角蛋白呈阴性,但对肌肉特异性肌动蛋白、肌红蛋白、结蛋白和波形蛋白呈阳性,从而表明存在肌源性分化。超微结构分析显示有粗肌丝和细肌丝。特殊检查未发现患者在更典型部位存在原发性横纹肌肉瘤的证据,也没有转移的证据。