Abstract：
Uterine leiomyosarcomas represent the most common uterine sarcomas. The prognosis is poor with metastatic recurrence in more than half of the cases. The purpose of this review is to make French recommendations for the management of uterine leiomyosarcomas within the framework of the French Sarcoma Group - Bone Tumor Study Group (GSF-GETO)/NETSARC+ and Malignant Rare Gynecological Tumors (TMRG) networks in order to optimize their therapeutic management. The initial assessment includes a MRI with diffusion perfusion sequence. The diagnosis is histological with a review in an expert center (Reference Network in Sarcoma Pathology (RRePS)). Total hysterectomy with bilateral salpingectomy, en bloc without morcellation, is performed when complete resection is possible, whatever the stage. There is no indication of systematic lymph node dissection. Bilateral oophorectomy is indicated in peri-menopausal or menopausal women. Adjuvant external radiotherapy is not a standard. Adjuvant chemotherapy is not a standard. It can be an option and consists in doxorobucin based protocols. In the event of local recurrence, the therapeutic options are based on revision surgery and/or radiotherapy. Systemic treatment with chemotherapy is most often indicated. In case of metastatic disease, surgical treatment remains indicated when resecable. In cases of oligo-metastatic disease, focal treatment of metastases should be considered. In the case of stage IV, chemotherapy is indicated, and is based on first-line doxorubicin-based protocols. In the event of excessive deterioration in general condition, management by exclusive supportive care is recommended. External palliative radiotherapy can be proposed for symptomatic purposes.
摘要：
子宫平滑肌肉瘤是最常见的子宫肉瘤。在超过一半的病例中，预后差，转移性复发。这篇综述的目的是在法国肉瘤组-骨肿瘤研究组（GSF-GETO）/NETSARC和恶性罕见妇科肿瘤（TMRG）网络的框架内，为子宫平滑肌肉瘤的管理提出法国建议，以优化其治疗管理。初始评估包括具有扩散灌注序列的MRI。诊断为组织学，并在专家中心进行了审查（肉瘤病理学参考网络（RRePS））。全子宫切除术伴双侧输卵管切除术，没有分折的恩集团，当完全切除是可能的，无论舞台。没有系统性淋巴结清扫的指征。双侧卵巢切除术适用于围绝经期或绝经期妇女。辅助外放疗不是标准。辅助化疗不是标准。它可以是一个选项，由基于doxorobucin的协议组成。如果局部复发，治疗选择基于修正手术和/或放疗.最常使用化疗进行全身治疗。在转移性疾病的情况下，手术治疗仍然表明，当切除。在寡转移疾病的情况下，应考虑转移灶的局部治疗.在第四阶段，化疗表明，基于一线阿霉素的方案。如果一般情况过度恶化，建议通过独家支持性护理进行管理。可以出于症状目的提出外部姑息性放射治疗。
