Lamovec J, Zidar A, Bracko M, Golouh R
Department of Pathology, Institute of Oncology, Ljubljana, Slovenia.
Pathol Res Pract. 1994 Jan;190(1):51-60. doi: 10.1016/s0344-0338(11)80496-6.
We present three cases of primary bone sarcoma with rhabdomyosarcomatous component which in one case appeared in a pure form, i.e., as rhabdomyosarcoma; in the other two cases it presented as one of multiple components of the tumor. The first patient was a 70-year-old man with a tumor of the left femur involving surrounding soft tissue of the thigh. Histologically, this was a case of so-called dedifferentiated chondrosarcoma with pleomorphic rhabdomyosarcoma representing high grade malignant component. The second patient, a 31-year-old man, developed a tumor in the left tibia which was a rare, fibrosarcoma-like type of rhabdomyosarcoma. The third patient was a 43-year-old woman with a tumor in the left tenth rib which was shown to be osteo- and chondrosarcoma with a minor rhabdomyosarcomatous component and was classified as malignant mesenchymoma. All patients were treated by surgery and chemotherapy. The first two of them died of metastatic disease 3 months, and 3 years and 4 months following surgery, respectively. Light microscopical diagnosis of rhabdomyosarcoma of hematoxylin-eosin sections was confirmed immunohistochemically in each case using reactions to desmin, muscle specific actin and myoglobin, and ultrastructurally in two cases. Rhabdomyosarcomatous component in primary bone tumors appears to be rarely present but more cases may be diagnosed in the future, if immunohistochemical and ultrastructural examinations are to be employed.
我们报告三例原发性骨肉瘤伴有横纹肌肉瘤成分，其中一例以纯形式出现，即横纹肌肉瘤；另外两例则作为肿瘤的多种成分之一出现。首例患者为一名70岁男性，左股骨肿瘤累及大腿周围软组织。组织学上，这是一例所谓的去分化软骨肉瘤，伴有多形性横纹肌肉瘤，代表高级别恶性成分。第二例患者为一名31岁男性，左胫骨出现肿瘤，为罕见的纤维肉瘤样横纹肌肉瘤。第三例患者是一名43岁女性，左第十肋骨有肿瘤，显示为骨肉瘤和软骨肉瘤，伴有少量横纹肌肉瘤成分，被归类为恶性间叶瘤。所有患者均接受了手术和化疗。前两名患者分别在手术后3个月、3年4个月死于转移性疾病。苏木精-伊红切片对横纹肌肉瘤的光镜诊断在每例中均通过免疫组化得以证实，免疫组化采用对结蛋白、肌肉特异性肌动蛋白和肌红蛋白的反应，两例还通过超微结构得以证实。原发性骨肿瘤中的横纹肌肉瘤成分似乎很少见，但如果采用免疫组化和超微结构检查，未来可能会诊断出更多病例。
