Uchida H, Yokoyama S, Nakayama I, Zeze K
First Department of Pathology, Medical College of Oita, Japan.
Acta Pathol Jpn. 1988 Aug;38(8):1087-96. doi: 10.1111/j.1440-1827.1988.tb02381.x.
An autopsy case of malignant rhabdoid tumor arising from soft parts in the left inguinal region of a 31-year-old Japanese male is reported. The tumor grew rapidly and appeared as a large mass measuring 21.5 X 16.5 cm located in the subcutaneous tissues of the lower abdominal wall. Histologically the tumor was composed of small round cells resembling rhabdomyoblasts, which showed a diffuse or alveolar arrangement. The cytoplasm of the tumor cells was eosinophilic and frequently contained round inclusion bodies, each consisting of an aggregation of globular filaments by electron microscopy. Immunohistochemistry showed the tumor cells to be positive for vimentin and keratin in the cytoplasm, but negative for actin, myoglobin and myosin. At autopsy, extensive metastatic or invasive tumors were observed mainly in the retroperitoneum and pelvic organs. The patient showed hypercalcemia which disappeared for only short periods just after surgery.
报告了一例31岁日本男性左腹股沟区软组织来源的恶性横纹肌样瘤尸检病例。肿瘤生长迅速,表现为位于下腹壁皮下组织的一个大小为21.5×16.5 cm的巨大肿块。组织学上,肿瘤由类似成横纹肌细胞的小圆形细胞组成,呈弥漫性或肺泡状排列。肿瘤细胞的细胞质嗜酸性,经常含有圆形包涵体,通过电子显微镜观察,每个包涵体由球状细丝聚集而成。免疫组织化学显示肿瘤细胞细胞质中的波形蛋白和角蛋白呈阳性,但肌动蛋白、肌红蛋白和肌球蛋白呈阴性。尸检时,主要在腹膜后和盆腔器官观察到广泛的转移性或浸润性肿瘤。患者出现高钙血症,仅在手术后短时间内消失。