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女性生殖道横纹肌肉瘤的局部治疗:来自儿童肿瘤学组、欧洲软组织肉瘤组和软组织肉瘤合作研究组的专家共识。

Local treatment of rhabdomyosarcoma of the female genital tract: Expert consensus from the Children's Oncology Group, the European Soft-Tissue Sarcoma Group, and the Cooperative Weichteilsarkom Studiengruppe.

机构信息

Department of Pediatric Surgery, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University, Chicago, Illinois.

Department of Pediatric Surgery, Bicetre Hospital, Assistance Publique-Hopitaux de Paris, Hopitaux Universitaires Paris-Sud, Le Kremlin Bicetre, France.

出版信息

Pediatr Blood Cancer. 2023 May;70(5):e28601. doi: 10.1002/pbc.28601. Epub 2020 Aug 6.

Abstract

The International Soft-Tissue Sarcoma Consortium (INSTRuCT) was founded as an international collaboration between different pediatric soft-tissue sarcoma cooperative groups (Children's Oncology Group, European Pediatric Soft-Tissue Sarcoma Group, and Cooperative Weichteilsarkom Studiengruppe). Besides other tasks, a major goal of INSTRuCT is to develop consensus expert opinions for best clinical treatment. This consensus paper for patients with rhabdomyosarcoma of the female genital tract (FGU-RMS) provides treatment recommendations for local treatment, long-term follow-up, and fertility preservation. Therefore, a review of the current literature was combined with recommendations of the treatment protocols of the appropriate clinical trials. Additionally, opinions of international FGU-RMS experts were incorporated into recommendations. Results were that the prognosis of FGU-RMS is favorable with an excellent response to chemotherapy. Initial complete surgical resection is not indicated, but diagnosis should be established properly. In patients with tumors localized at the vagina or cervix demonstrating incomplete response after induction chemotherapy, local radiotherapy (brachytherapy) should be carried out. In patients with persistent tumors at the corpus uteri, hysterectomy should be performed. Fertility preservation should be considered in all patients. In conclusion, for the first time, an international consensus for the treatment of FGU-RMS patients could be achieved, which will help to harmonize the treatment of these patients in different study groups.

摘要

国际软组织肉瘤联合会(INSTRuCT)是由不同儿科软组织肉瘤协作组(儿童肿瘤组、欧洲儿科软组织肉瘤组和软组织肉瘤协作组)之间的国际合作成立的。INSTRuCT 的主要目标之一除其他任务外,还为最佳临床治疗制定共识专家意见。本共识文件为女性生殖器官横纹肌肉瘤(FGU-RMS)患者提供了局部治疗、长期随访和生育力保存的治疗建议。因此,结合适当临床试验的治疗方案,对现有文献进行了回顾,并提出了建议。此外,还纳入了国际 FGU-RMS 专家的意见。结果表明,FGU-RMS 的预后良好,对化疗有极好的反应。不建议初始完全手术切除,但应正确诊断。对于阴道或宫颈局部肿瘤,在诱导化疗后无完全缓解的患者,应进行局部放疗(近距离放疗)。对于子宫体有持续性肿瘤的患者,应进行子宫切除术。应考虑所有患者的生育力保存。总之,首次为 FGU-RMS 患者达成了国际共识,这将有助于协调不同研究组对这些患者的治疗。

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