Case Report: Leiomyosarcoma Originating from the Gonadal Vein

Leiomyosarcoma of the gonadal vein is an exceedingly rare entity, representing a small subset of smooth muscle tumors that more commonly arise in the retroperitoneum, uterus, and blood vessels. To date, fewer than 10 cases of gonadal vein leiomyosarcoma have been reported in the literature, highlighting its rarity and the limited understanding of its clinical behavior. These tumors are often diagnosed incidentally or present with nonspecific symptoms, such as abdominal pain or a palpable mass, which can complicate early detection. The proximity of gonadal vein leiomyosarcomas to critical structures, such as the ureter, renal vessels, and surrounding organs, introduces unique diagnostic and surgical challenges. Previous reports have underscored the importance of advanced imaging techniques, including CT and MRI, in delineating the tumor’s anatomical relationships and guiding surgical planning. This case, involving a leiomyosarcoma closely associated with the patient’s left ureter, provides an opportunity to build on existing knowledge by addressing the clinical presentation, diagnostic approach, treatment pathway, and long-term follow-up strategies required for optimal management. By presenting this detailed review, we aim to contribute valuable insights into the diagnosis and management of this rare malignancy.