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Case of the Week

History:The patient is a 61 year-old-female with postmenopausal bleeding. Ultrasonogram revealed an echogenic endometrium with a 2.4 cm cyst and multiple intramural fibroids. The images are provided from one of the fibroids. What is your diagnosis?

Answer: This is a uterine tumor resembling an ovarian sex cord tumor (UTROSCT). Comment: This is a rare uterine tumor of uncertain histogenesis that morphologically resembles ovarian sex cord tumors, affecting perimenopausal or menopausal women. Imaging studies are not diagnostic of this entity. Histopathology is the gold standard. The diagnosis is based on a predominant sex cord pattern (cord, nests, trabeculae, and tubules) and round to epithelioid-appearing cells with bland nuclei and scant to abundant eosinophilic to clear cytoplasm; nuclear atypia is rare and the tumor is mitotically quiescent. Immunophenotypically, UTROSCT co-expresses epithelial, smooth muscle, sex cord markers and steroid hormone receptors. Various postulated histogenetic theories include: derivation from the ovarian sex cord cells that have been displaced during embryogenesis; derivation from uncommitted mesenchymal stem cells; overgrowth of the sex cord elements within endometrial stromal neoplasms or adenosarcoma. Absence of JAZF1-SUZ12fusions and PFH1 gene rearrangements distinguish these neoplasms from endometrial stromal tumors. Despite the morphologic and immunohistochemical resemblance to sex cord-stromal tumors, the signature mutations (FOXL2 and DICER1) of ovarian sex cord-stromal tumors are not identified in UTROSCTs. These tumors are treated by surgical removal, based on age and parity of the patients (total hysterectomy with bilateral adnexectomy, simple hysterectomy, resection of the mass). UTROSCTs are considered neoplasms of uncertain malignant potential, but pursue a benign course in most cases. However, occasional recurrences and rare metastases (lymph nodes, appendix, omentum, small bowel, and subcutaneous tissue) have been reported. Patients with local recurrence or metastases may receive chemotherapy or pelvic radiation, although there is no established treatment protocol. A recent report highlighted that UTROSCTs with extensive rhabdoid differentiation and ESR1-NCOA2 fusions pursue an aggressive course. Thus, it is important to be aware of these specific features and recommend long-term follow-up due to the possibility of late recurrences.. PMID: 24283865 PMID: 24696722 PMID: 26097607 PMID: 26598979 PMID: 32675660 This case was provided by Dr. Sambit K Mohanty, a pathologist at CORE Diagnosis, New Delhi and Director of Surgical Pathology at the Advanced MedicalResearch Institute, India. He is also one of our authors at Innovative Science Press. Check out our titles at: 1-703-350-4308/703-340-3198

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