Inferior vena cava leiomyosarcoma in a young woman with autosomal dominant polycystic kidney disease

Leiomyosarcomas of the inferior vena cava pose a significant clinical challenge due to their rarity and complex vascular involvement. This report discusses a case of a 29-year-old woman with autosomal dominant polycystic kidney disease who experienced recurrent abdominal pain. Imaging revealed a retroperitoneal mass, which surgical exploration identified as originating from the inferior vena cava. Surgical resection and subsequent histopathological analysis confirmed the mass to be a leiomyosarcoma. This case highlights the importance of early detection, multidisciplinary management, and continuous monitoring in treating rare vascular leiomyosarcomas. While it is possible that genetic factors could play a role in the development of both conditions, the presence of both in one patient may simply be coincidental. To establish a genetic association, a larger study involving multiple cases would be required and to develop standardized guidelines for managing these tumors.