Cavazzana A O, Schmidt D, Ninfo V, Harms D, Tollot M, Carli M, Treuner J, Betto R, Salviati G
Institute of Pathology, Padua University, Italy.
Am J Surg Pathol. 1992 Mar;16(3):229-35. doi: 10.1097/00000478-199203000-00002.
Twenty-one cases of embryonal rhabdomyosarcoma, composed mainly of elongated spindle cells arranged in a fasciculated or storiform pattern, were retrieved from the files of the German-Italian Cooperative Soft Tissue Sarcoma Study. The term spindle cell rhabdomyosarcoma is proposed to designate this histotype. Spindle cell rhabdomyosarcoma predilected male patients (18 males, three females) and involved mostly the paratesticular area (12 cases) and the head and neck region (six cases). Histologically, all cases were characterized by a uniform proliferation of elongated spindle cells with eosinophilic and fibrillar cytoplasm mimicking smooth muscle fibers; immunocytochemical studies disclosed high expression of the muscle markers titin, desmin, and myoglobin. Clinical information was available in 17 cases; according to the Intergroup Rhabdomyosarcoma Study (IRS) grouping system, 13 were classified in group I, two in group II, and two in group III. Sixteen patients were well and alive 24 to 100 months after diagnosis; one patient died from disease progression 24 months after diagnosis. Analysis of our results determined that spindle cell rhabdomyosarcoma constitutes a rare variant of the embryonal form, showing a high degree of skeletal muscle differentiation and a low malignant potential; it should therefore be distinguished from classical forms of embryonal rhabdomyosarcoma.
从德意合作软组织肉瘤研究档案中检索出21例胚胎性横纹肌肉瘤病例，其主要由呈束状或席纹状排列的细长梭形细胞组成。建议使用“梭形细胞横纹肌肉瘤”这一术语来命名这种组织学类型。梭形细胞横纹肌肉瘤好发于男性患者（18例男性，3例女性），主要累及睾丸旁区域（12例）和头颈部区域（6例）。组织学上，所有病例的特征均为细长梭形细胞呈一致增生，胞质嗜酸性且呈纤维状，类似平滑肌纤维；免疫细胞化学研究显示肌肉标志物肌联蛋白、结蛋白和肌红蛋白高表达。17例有临床资料；根据横纹肌肉瘤协作组研究（IRS）分组系统，13例为Ⅰ组，2例为Ⅱ组，2例为Ⅲ组。16例患者在诊断后24至100个月情况良好且存活；1例患者在诊断后24个月因疾病进展死亡。对我们的结果分析表明，梭形细胞横纹肌肉瘤是胚胎型的一种罕见变异型，具有高度的骨骼肌分化和低恶性潜能；因此应将其与经典的胚胎性横纹肌肉瘤区分开来。
