Indian Journal of Obstetrics and Gynecology Research 2023;10(2):217–220 Content available at: https://www.ipinnovative.com/open-access-journals Indian Journal of Obstetrics and Gynecology Research Journal homepage: www.ijogr.org Case Report Low-grade endometrial stromal sarcoma: differential with uterine leiomyoma: A rare case report Kafil Akhtar 1, *, Saba Shakil 1 , Mazhar Fahim 1 , Mohammad Adil 1 1 Dept. of Pathology, Jawaharlal Nehru Medical College, Faculty of Medicine, Aligarh Muslim University, Aligarh, Uttar Pradesh, India ARTICLE INFO Article history: Received 07-07-2022 Accepted 21-12-2022 Available online 18-05-2023 Keywords: Endometrial stromal tumor Sarcoma Uterine leiomyoma Immunohistochemistry ABSTRACT Endometrial stromal tumors are extremely rare uterine malignancy, mainly among young females. Herein, we report a case of low-grade endometrial stromal tumor in a 19-year-old women who presented with the non-specific symptoms of lower abdominal pain, swelling and vaginal bleeding similar to the uterine leiomyoma. Ultrasound finding showed bulky uterus with multiple fibroid and degenerative changes. Contrast enhanced computed tomography (CECT) displayed diffusely enlarged uterus with heterogenous enhancement of the solid cystic mass with normal bilateral tubes and ovary. Considering the patient in the reproductive age group and her fertility status, she was operated with surgical subtotal hysterectomy. Histopathological examination and immunohistochemistry confirmed the diagnosis of Low-Grade Endometrial Stromal Sarcoma (LG-ESS). Patient was closely followed up for recurrence. Endometrial Stromal Sarcoma (ESS) should be included in the differential diagnosis of uterine leiomyoma to avoid recurrence of the lesion. This is an Open Access (OA) journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. For reprints contact: reprint@ipinnovative.com 1. Introduction Endometrial stromal tumors (ESTs) are rare uterine mesenchymal tumors, accounting around 20% of all uterine sarcomas. 1 Mostly they are composed of cells that resemble proliferative-type endometrial stromal cells. 2 According to the latest WHO 2020 classification, ESTs have been divided into 4 categories: Endometrial Stromal Nodule (ESN), Low-Grade Endometrial Stromal Sarcoma (LG- ESS), High Grade Endometrial Stromal Sarcoma (HG-ESS) and Undifferentiated Uterine Sarcoma (USS). 3 ESTs usually affects younger female as compared with other uterine tumors. 3 They typically occur in the reproductive or postmenopausal age groups and the mean age is 42-58 years. 3 They have a non-specific presentation (vaginal bleeding, enlarged uterus, and pelvic pain/pressure) * Corresponding author. E-mail address: drkafilakhtar@gmail.com (K. Akhtar). or are an incidental finding. 4 2. Case Report A 19-year old unmarried female patient presented to the Obstetrics & Gynecology OPD with the history of lower abdominal pain, swelling and excessive vaginal bleeding. On physical examination, she was found to have pallor and lower abdominal mass. Uterus was enlarged to 26 week size and soft in consistency. Vitals were stable and her past history was unremarkable. Laboratory investigations showed hemoglobin 7 gm/dl. Other test findings such as total leukocyte count, differential leukocyte count and platelet count were within normal limit. Tumor markers and hormone levels were also within normal limit. Patient was referred to the radiology department with a probable diagnosis of Leiomyoma. https://doi.org/10.18231/j.ijogr.2023.045 2394-2746/© 2023 Innovative Publication, All rights reserved. 217