Lebreton Coriolan, Meeus Pierre, Genestie Catherine, Croce Sabrina, Guyon Frédéric, Moscardo Carmen Llacer, Taieb Sophie, Blay Jean-Yves, Bonvalot Sylvie, Bompas Emmanuelle, Chevreau Christine, Lécuru Fabrice, Rossi Léa, Joly Florence, Rios Maria, Chaigneau Loïc, Duffaud Florence, Pautier Patricia, Ray-Coquard Isabelle
Institut Bergonié, département d'oncologie médicale, 33000 Bordeaux, France; Centre Léon-Bérard, département d'oncologie médicale, 69008 Lyon, France.
Centre Léon-Bérard, département de chirurgie, 69008 Lyon, France.
Bull Cancer. 2023 Jul-Aug;110(7-8):844-854. doi: 10.1016/j.bulcan.2023.03.003. Epub 2023 Mar 27.
Low-grade endometrial stromal sarcoma (LG-ESS) accounts for approximately 15% of all uterine sarcomas. Median age of patients is around 50 years and half of the patients are premenopausal. In all, 60% of cases present with FIGO stage I disease. Preoperatively radiologic findings of ESS are not specific. Pathological diagnosis remains essential. This review aimed to present the French guidelines for low grade ESS treatment within the Groupe sarcome français - Groupe d'étude des tumeurs osseuse (GSF-GETO)/NETSARC+ and tumeur maligne rare gynécologique (TMRG) networks. Treatments should be validated in multidisciplinary team involved in sarcomas or rare gynecologic tumors. Hysterectomy is the cornerstone of treatment for localized ESS, and morcellation should be avoided. Systematic lymphadenectomy in ESS does not improve the outcome and is not recommended. Leaving the ovaries in situ in stage I tumors could be discussed for young women. Adjuvant hormonal treatment could be considered, for two years for stage I with morcellation or stage II and livelong for stages III or IV. Nevertheless, several questions remain, such as optimal doses, regimens (progestins or aromatase inhibitors) and duration of therapy. Tamoxifen is contraindicated. Secondary cytoreductive surgery if feasible for recurrent disease, appears to be an acceptable approach. Systemic treatment for recurrent or metastatic disease is mainly hormonal, with or without surgery.
低级别子宫内膜间质肉瘤（LG-ESS）约占所有子宫肉瘤的15%。患者的中位年龄约为50岁，其中一半患者处于绝经前。总体而言，60%的病例为国际妇产科联盟（FIGO）I期疾病。ESS术前的影像学表现不具有特异性。病理诊断仍然至关重要。本综述旨在介绍法国肉瘤研究小组-骨肿瘤研究小组（GSF-GETO）/NETSARC+以及罕见妇科恶性肿瘤研究小组（TMRG）网络中关于低级别ESS治疗的指南。治疗方案应在参与肉瘤或罕见妇科肿瘤治疗的多学科团队中得到验证。子宫切除术是局限性ESS治疗的基石，应避免粉碎术。ESS的系统性淋巴结清扫术并不能改善预后，不建议进行。对于年轻女性，I期肿瘤保留卵巢原位的问题可以讨论。对于I期伴粉碎术或II期患者，可考虑进行两年的辅助激素治疗；对于III期或IV期患者，则需终身治疗。然而，仍存在一些问题，如最佳剂量、治疗方案（孕激素或芳香化酶抑制剂）以及治疗持续时间。他莫昔芬禁用。对于复发性疾病，如有可能，二次减瘤手术似乎是一种可接受的方法。复发性或转移性疾病的全身治疗主要是激素治疗，可联合或不联合手术。
