Fibroblastic variant of low-grade endometrial stromal sarcoma mimicking an inflammatory pseudotumor: a case report

The World Health Organization currently divides endometrial stromal sarcomas into 4 different entities, based on their clinical and pathological features: endometrial stromal nodule, low-grade endometrial stromal sarcomas (LG-ESS), High-grade endometrial stromal sarcomas, and undifferentiated uterine sarcoma. The fibroblastic variant of LG-ESS is rare and is usually made of small tumoral cells of oval to fusiform shape, demonstrating low cytologic atypia and low mitotic activity, which can lead to confusion with a benign myofibroblastic proliferation. We hereby report a rare case of a fibroblastic variant of LG-ESS in a 37-year-old woman presenting abundant metrorrhagia, which was initially misdiagnosed as an inflammatory pseudotumor before proofreading in our laboratory, along with a review of a histological and immunohistochemical findings, aiming to help pathologists avoid this diagnosis pitfall.