PDF(Pubmed)
Abstract：
Uterine Tumors Resembling Ovarian Sex Cord Tumors (UTROSCTs) are rare uterine mesenchymal neoplasms with uncertain biological potential. These tumors, which affect both premenopausal and postmenopausal women, usually have a benign clinical course. Nevertheless, local recurrences and distant metastases have been described. By analyzing 511 cases retrieved from individual reports and cases series, we provide here the most comprehensive overview of UTROSCT cases available in the literature, supplemented by two new cases of UTROSCTs. Case 1 was an asymptomatic 31-year-old woman who underwent a laparoscopic resection of a presumed leiomyoma. Case 2 was a 58-year-old postmenopausal woman with abnormal vaginal bleeding who underwent an outpatient hysteroscopic biopsy of a suspicious endometrial area. In both cases, immunohistochemical positivity for Calretinin and Inhibin was noted, typical for a sex cord differentiation. In both cases, total laparoscopic hysterectomy with bilateral salpingo-oophorectomy was performed. In light of the available literature, no pathognomonic clinical or imaging finding can be attributed to UTROSCT. Patients usually present with abnormal uterine bleeding or pelvic discomfort, but 20% of them are asymptomatic. In most cases, a simple hysterectomy appears to be the appropriate treatment, but for women who wish to become pregnant, uterus-preserving approaches should be discussed after excluding risk factors. Age, tumor size, lymphovascular space invasion, nuclear atypia, and cervical involvement are not reliable prognostic factors in UTROSCT. The current research suggests that aggressive cases (with extrauterine spread or recurrence) can be identified based on a distinct genetic and immunohistochemical phenotype. For instance, UTROSCTs characterized by GREB1::NCOA1-3 fusions and PD-L1 molecule expression appear to be predisposed to more aggressive behaviors and recurrence, with GREB1::NCOA2 being the most common gene fusion in recurrent tumors. Hence, redefining the criteria for UTROSCTs may allow a better selection of women suitable for fertility-sparing treatments or requiring more aggressive treatments in the future.
摘要：
类似于卵巢性索肿瘤（UTROSCTs）的子宫肿瘤是罕见的子宫间充质肿瘤，具有不确定的生物学潜力。这些肿瘤，影响绝经前和绝经后的妇女，通常有一个良性的临床过程。然而，已经描述了局部复发和远处转移。通过分析从个别报告和案例系列中检索到的511个案例，我们在这里提供文献中提供的UTROSCT病例的最全面概述，辅以两例新的UTROSCT。病例1是一名无症状的31岁妇女，她接受了假定的平滑肌瘤的腹腔镜切除术。病例2是一名58岁的绝经后妇女，患有异常阴道出血，她接受了可疑子宫内膜区域的门诊宫腔镜活检。在这两种情况下，注意到Calretinin和抑制素的免疫组织化学阳性，典型的性索分化。在这两种情况下，行腹腔镜全子宫切除术和双侧输卵管卵巢切除术。根据现有文献，没有病理的临床或影像学发现可归因于UTROSCT。患者通常表现为异常子宫出血或盆腔不适，但其中20%无症状。在大多数情况下,简单的子宫切除术似乎是适当的治疗方法，但对于想要怀孕的女性来说,在排除危险因素后，应讨论保留子宫的方法。年龄,肿瘤大小,淋巴管间隙侵入，核非典型性,宫颈受累不是UTROSCT的可靠预后因素。目前的研究表明，可以根据不同的遗传和免疫组织化学表型来识别侵袭性病例（宫外扩散或复发）。例如,以GREB1::NCOA1-3融合和PD-L1分子表达为特征的UTROSCT似乎倾向于更具攻击性的行为和复发，GREB1::NCOA2是复发性肿瘤中最常见的基因融合。因此，重新定义UTROSCT的标准可能会让更多的女性选择适合保留生育力的治疗或将来需要更积极的治疗.
