Human Pathology Reports · Published 2025-09-11 · DOI 10.1016/j.hpr.2025.300793
Leiomyosarcoma (LMS) is a malignant tumor of smooth muscle cells. When the tumor originates from a blood vessel, it arises from the smooth muscle cells in the tunica media of the vessel wall. Approximately 15 % of soft tissue sarcomas originate in the retroperitoneum (Kotilingam et al., 2006) [1]. However, LMS of the gonadal vein is extremely rare, with fewer than 15 documented cases. This emphasizes not only its rarity but also the narrow insight into its clinical behaviour and management. Here, we discuss a 42-year-old female patient who presented to our institution with a palpable abdominal mass that had been noticeable for two months. A computed tomography (CT) scan of her abdomen and pelvis revealed a large, irregular right adnexal mass measuring approximately 13 cm, with areas of necrosis and cystic degeneration, resulting in a heterogenous appearance. During surgical resection, the mass appeared to originate from the gonadal vein. Histopathological examination confirmed a diagnosis of leiomyosarcoma. In this article, we review the clinical, radiologic, and histopathologic features of gonadal vein leiomyosarcoma, along with a review of similar case reports.
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