Summary

Although intravenous leiomyomatosis is widely documented, intravenous extension of leiomyosarcoma into the inferior vena cava (IVC) and subsequently into the right atrium is extremely rare. Less than five such cases have been reported in the literature worldwide. Uterine leiomyosarcoma is an aggressive smooth muscle tumour occurring with an incidence of 1% in all female genital tract cancers and comprises about 3–7% of uterine cancers. It carries a generally poor prognosis with 5-year survival rates ranging from 18.8% to 65% across all stages. We report a case of primary uterine leiomyosarcoma with intravascular tumour propagation extending to the renal vein, IVC and right atrium of the heart, which was successfully resected in a one stage operation by a multidisciplinary team. This case demonstrates the importance of preoperative radiological staging and multidisciplinary planning.