Intracardiac and intravascular leiomyomatosis associated with a pelvic arterio-venous fistula

An intravenous leiomyomatosis is a rare benign smooth muscle tumor originating from myometrial veins. The leiomyomatosis occasionally extends from the pelvic vein into the right cardiac chambers. A pelvic arterio-venous fistula (AVF) is also a rare pelvic vascular malformation. The massive arterio-venous shunting causes high output cardiac failure. We present a very rare case of intravascular and intracardiac leiomyomatosis associated with a pelvic AVF. The patient had congestive heart failure before surgery. We successfully performed one-stage surgery to remove the leiomyomatosis as well as the uterus and left ovary with the pelvic AVF.