Edel G, Wuisman P, Erlemann R
Gerhard-Domagk-Institute of Pathology, University of Münster, FRG.
Pathol Res Pract. 1993 Feb;189(1):102-7; discussion 107-10. doi: 10.1016/S0344-0338(11)80127-5.
A rare case of the leiomyomatous variant of embryonal rhabdomyosarcoma is reported. A 13-year-old boy presented with a recurrent painless mass on the ventral side of the right forefoot. Microscopically, the tumor consisted of spindle-shaped and round tumor cells in a fascicular or storiform, focally hemangiopericytoma-like growth pattern. The cytoplasm of the spindle-shaped tumor cells was eosinophilic and fibrillary, in some areas resembling smooth muscle cells. Immunohistologically, all tumor cells were vimentin-positive, most of them also stained with antibodies to desmin and muscle specific actin (MSA). In addition, many tumor cells showed a co-expression of alpha-sarcomeric actin and myoglobin. All tumor cells were negative with alpha-smooth muscle actin. Two years after surgical treatment and chemotherapy the patient is well with no evidence of distant metastases. The clinicopathological features and differential diagnostic problems are discussed.
报告了一例罕见的胚胎性横纹肌肉瘤平滑肌瘤样变体病例。一名13岁男孩右前足腹侧出现复发性无痛肿块。显微镜下，肿瘤由梭形和圆形肿瘤细胞组成，呈束状或车辐状生长，局部呈血管外皮细胞瘤样生长模式。梭形肿瘤细胞的细胞质呈嗜酸性且呈纤维状，在某些区域类似于平滑肌细胞。免疫组织化学检查显示，所有肿瘤细胞波形蛋白阳性，大多数肿瘤细胞也被结蛋白和肌肉特异性肌动蛋白（MSA）抗体染色。此外，许多肿瘤细胞显示α-肌动蛋白和肌红蛋白共表达。所有肿瘤细胞α-平滑肌肌动蛋白均为阴性。手术治疗和化疗两年后，患者情况良好，无远处转移迹象。讨论了临床病理特征和鉴别诊断问题。
