Kaur Kavneet, Rajeshwari Madhu, Gurung Niteeka, Kumar Hemanth, Sharma Mehar C, Yadav Rajni, Kumar Sunesh, Manchanda Smita, Singhal Seema, Mathur Sandeep R
Department of Pathology, All India Institute of Medical Sciences, New Delhi, India.
Department of Obstetrics and Gynaecology, All India Institute of Medical Sciences, New Delhi, India.
Indian J Pathol Microbiol. 2020 Feb;63(Supplement):S81-S86. doi: 10.4103/IJPM.IJPM_340_19.
Uterine tumors resembling ovarian sex cord tumor (UTROSCT) are a unique group of neoplasms with diverse morphology and immunophenotypic characteristics, coexpressing sex cord, epithelial, and smooth-muscle markers. To date, less than 100 cases have been reported and there is paucity of data concerning their clinical behavior.
All cases of uterine body tumors diagnosed over a period of two and a half years (2016-2018) were retrieved. Histopathological features were reviewed and extended panel of immunohistochemistry was performed to identify cases of UTROSCTs.
Six cases of UTROSCTs were identified with a median age of 46.5 years. Four of them presented with menorrhagia, while two with postmenopausal bleeding including one with a history of carcinoma breast. Three of these cases were initially misdiagnosed as endometrial stromal sarcoma and adenocarcinomas. They all underwent hysterectomy with bilateral salpingo-oophorectomy.
It is considered a tumor with low malignant potential; however, one out of six cases (16.7%) in our study showed metastasis, within 1 year of diagnosis. It is important to recognize this entity as it mimics a wide range of both benign and malignant tumors. Molecular pathogenesis and exact management protocols remain elusive due to rarity,hence, multi-institutional studies are warranted.
子宫肿瘤样卵巢性索肿瘤（UTROSCT）是一组独特的肿瘤，具有多样的形态学和免疫表型特征，同时表达性索、上皮和平滑肌标志物。迄今为止，报道的病例不足100例，关于其临床行为的数据也很匮乏。
检索了在两年半时间（2016 - 2018年）内诊断的所有子宫体肿瘤病例。回顾了组织病理学特征，并进行了扩展的免疫组化检查以识别UTROSCT病例。
共识别出6例UTROSCT，中位年龄为46.5岁。其中4例表现为月经过多，2例表现为绝经后出血，其中1例有乳腺癌病史。这些病例中有3例最初被误诊为子宫内膜间质肉瘤和腺癌。她们均接受了子宫切除术及双侧输卵管卵巢切除术。
它被认为是一种恶性潜能较低的肿瘤；然而，在我们的研究中，6例中有1例（16.7%）在诊断后1年内出现转移。认识到这一实体很重要，因为它可模仿多种良性和恶性肿瘤。由于其罕见性，分子发病机制和确切的治疗方案仍不明确，因此，有必要开展多机构研究。
