Epithelioid leiomyosarcoma of the uterus and the diagnostic challenge in diagnosing it on small biopsy

Abstract

Epithelioid leiomyosarcoma of the uterus is a rare soft-tissue tumor of the pelvis with <1% cases. It can be asymptomatic or can reach an enormous size, resulting in chronic pelvic pain, postmenopausal bleeding, or may cause bowel and bladder dysfunction. The diagnostic dilemma arises especially when they have epithelioid morphology. This case is being presented because of its rare incidence along with the diagnostic dilemma it presented due to epithelioid morphology which is a very rare presentation, especially challenging on small biopsies. This case also highlights the usefulness of immunohistochemical markers, such as WT1, p53, and p16, which has important diagnostic as well as prognostic values in rare variants of usual tumors. Leiomyosarcoma per se is a highly aggressive tumor, therefore, timely diagnosis and management are advised.