Guérard M J, Arguelles M A, Ferenczy A
Gynecol Oncol. 1983 Jun;15(3):325-39. doi: 10.1016/0090-8258(83)90050-1.
Pure rhabdomyosarcomas are extremely rare and lethal primary neoplasms of the ovary. The available literature revealed 14 such cases. This report describes the light and electron microscopic findings of another case. At the electron microscopic level, rhabdomyogenesis recapitulates skeletal muscle differentiation during early fetal life. Rhabdomyoblasts in the ovary may arise from either the uncommitted stromal fibroblasts or from fibroblasts of endometriotic stroma. The age of patients at diagnosis ranged between 13 months and 86 years with 60% of women aged 40 years and older. About 50% of the cases were diagnosed with disease beyond the ovary, and survival ranged between 18 days and 15 months after diagnosis. Aggressive combination therapy including surgery, radiation, and chemotherapy appears to be the only hope for improved survival.
纯横纹肌肉瘤是极其罕见且致命的卵巢原发性肿瘤。现有文献报道了14例此类病例。本报告描述了另一例病例的光镜和电镜检查结果。在电镜水平上，横纹肌发生过程重现了胎儿早期骨骼肌的分化过程。卵巢中的成横纹肌细胞可能起源于未分化的基质成纤维细胞或子宫内膜异位基质中的成纤维细胞。诊断时患者年龄在13个月至86岁之间，60%的女性年龄在40岁及以上。约50%的病例在诊断时已出现卵巢外病变，诊断后生存期在18天至15个月之间。积极的综合治疗，包括手术、放疗和化疗，似乎是提高生存率的唯一希望。
