Uterine tumor resembling ovarian sex cord tumor: A series of six cases displaying varied histopathological patterns and clinical profiles

• Main Authors: Kavneet Kaur (Author), Madhu Rajeshwari (Author), Niteeka Gurung (Author), Hemanth Kumar (Author), Mehar C Sharma (Author), Rajni Yadav (Author), Sunesh Kumar (Author), Smita Manchanda (Author), Seema Singhal (Author), Sandeep R Mathur (Author)
• Format: Book
• Published: Wolters Kluwer Medknow Publications, 2020-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 0377-4929; 10.4103/IJPM.IJPM_340_19
• Keywords: mesenchymal neoplasm; ovarian; sex cord stromal; uterus; Pathology; RB1-214; Microbiology; QR1-502
• Source: Indian Journal of Pathology and Microbiology, Vol 63, Iss 5, Pp 81-86 (2020)

Introduction: 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. Materials and Methods: 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. Results: 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. Conclusion: 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.