Horng Huann-Cheng, Wen Kuo-Chang, Wang Peng-Hui, Chen Yi-Jen, Yen Ming-Shyen, Ng Heung-Tat
Department of Obstetrics and Gynecology, Taipei Veterans General Hospital, Taipei, Taiwan; Department of Obstetrics and Gynecology, National Yang-Ming University School of Medicine, Taipei, Taiwan.
Department of Obstetrics and Gynecology, Taipei Veterans General Hospital, Taipei, Taiwan; Department of Obstetrics and Gynecology, National Yang-Ming University School of Medicine, Taipei, Taiwan; Department of Medical Research, China Medical University Hospital, Taichung, Taiwan.
Taiwan J Obstet Gynecol. 2016 Aug;55(4):472-9. doi: 10.1016/j.tjog.2016.04.034.
Endometrial stromal tumors are rare uterine tumors (<1%). Four main categories include endometrial stromal nodule, low-grade endometrial stromal sarcoma (LG-ESS), high-grade endometrial stromal sarcoma (HG-ESS), and uterine undifferentiated sarcoma (UUS). This review is a series of articles discussing the uterine sarcomas. LG-ESS, a hormone-dependent tumor harboring chromosomal rearrangement, is an indolent tumor with a favorable prognosis, but characterized by late recurrences even in patients with Stage I disease, suggesting the requirement of a long-term follow-up. Patients with HG-ESS, based on the identification of YWHAE-NUTM2A/B (YWHAE-FAM22A/B) gene fusion, typically present with advanced stage diseases and frequently have recurrences, usually within a few years after initial surgery. UUS is, a high-grade sarcoma, extremely rare, lacking a specific line of differentiation, which is a diagnosis of exclusion (the wastebasket category, which fails to fulfill the morphological and immunohistochemical criteria of translocation-positive ESS). Surgery is the main strategy in the management of uterine sarcoma. Due to rarity, complex biological characteristics, and unknown etiology and risk factors of uterine sarcomas, the role of adjuvant therapy is not clear. Only LG-ESS might respond to progestins or aromatase inhibitors.
子宫内膜间质肿瘤是罕见的子宫肿瘤（<1%）。主要包括四类：子宫内膜间质结节、低级别子宫内膜间质肉瘤（LG-ESS）、高级别子宫内膜间质肉瘤（HG-ESS）和子宫未分化肉瘤（UUS）。本综述是一系列讨论子宫肉瘤的文章。LG-ESS是一种依赖激素的肿瘤，伴有染色体重排，是一种惰性肿瘤，预后良好，但即使是I期疾病患者也有晚期复发的特点，提示需要长期随访。基于YWHAE-NUTM2A/B（YWHAE-FAM22A/B）基因融合的鉴定，HG-ESS患者通常表现为晚期疾病，且经常复发，通常在初次手术后几年内复发。UUS是一种高级别肉瘤，极为罕见，缺乏特定的分化谱系，是一种排除性诊断（即不符合易位阳性ESS的形态学和免疫组化标准的“垃圾桶类别”）。手术是子宫肉瘤治疗的主要策略。由于子宫肉瘤罕见、生物学特性复杂以及病因和危险因素不明，辅助治疗的作用尚不清楚。只有LG-ESS可能对孕激素或芳香化酶抑制剂有反应。
