Montag T W, D'ablaing G, Schlaerth J B, Gaddis O, Morrow C P
Gynecol Oncol. 1986 Oct;25(2):171-94. doi: 10.1016/0090-8258(86)90098-3.
Embryonal rhabdomyosarcomas of the uterus and cervix are rare tumors with a very poor prognosis historically. Adjuvant multidrug chemotherapy combined with local radiation therapy and/or surgery has resulted in markedly improved survival rates. Six patients with embryonal rhabdomyosarcoma of the uterus/cervix are reported, with a compilation of 28 additional patients from the literature. Nineteen of 24 patients (79%) treated with combination therapy were alive and well 4-147+ months (median 61 months) after diagnosis. The six cases from USC Medical Center and affiliated hospitals are all well 17-144 months (mean 95 months) after diagnosis. Analysis of the collected series indicates that extent of disease is an important prognostic factor. Adjuvant chemotherapy with vincristine, actinomycin D, and cyclophosphamide (VAC) improves survival. Initial therapy for embryonal rhabdomyosarcoma confined to the uterus/cervix should include radical hysterectomy, pelvic lymphadenectomy, and adjuvant chemotherapy with VAC. Pelvic radiotherapy should be employed for involved surgical margins or positive nodes. Advanced disease managed initially with chemotherapy and radiotherapy may allow subsequent curative extirpative surgery.
子宫和宫颈胚胎性横纹肌肉瘤是罕见肿瘤，历史上预后极差。辅助多药化疗联合局部放疗和/或手术已使生存率显著提高。本文报告了6例子宫/宫颈胚胎性横纹肌肉瘤患者，并汇总了文献中另外28例患者的情况。接受联合治疗的24例患者中有19例（79%）在诊断后4 - 147 +个月（中位时间61个月）存活且情况良好。南加州大学医学中心及附属医院的6例患者在诊断后17 - 144个月（平均95个月）均情况良好。对所收集病例系列的分析表明，疾病范围是一个重要的预后因素。采用长春新碱、放线菌素D和环磷酰胺（VAC）进行辅助化疗可提高生存率。局限于子宫/宫颈的胚胎性横纹肌肉瘤的初始治疗应包括根治性子宫切除术、盆腔淋巴结切除术以及VAC辅助化疗。对于手术切缘受累或淋巴结阳性的情况应采用盆腔放疗。最初采用化疗和放疗治疗的晚期疾病可能允许后续进行根治性切除手术。
