Intravascular leiomyomatosis with cardiac extension: a case report

Intravascular leiomyomatosis (IVL) with intracardiac extension is a rare benign tumour seen exclusively in women, characterized by proliferation of uterine smooth muscle cells through the venous circulation into the inferior vena cava (IVC) and the right heart chambers.

A 47 years old women with history of previous hysterectomy due to myomatosis, presented with nausea, anorexia, and bilateral lower limb swelling over the preceding 2 months. An outpatient abdominal ultrasound discovered a mass in the IVC. Echocardiogram and computed tomography demonstrated a large intravascular mass extending from the pelvis to the right heart chambers. The tumour was completely removed in a concomitant open-heart surgery and laparotomy. Post-operative course was uncomplicated. A month later, the patient was feeling well and in good clinical condition. The histological analysis consisted with IVL.

Intracardiac leiomyomatosis is a rare clinical condition which requires high index of suspicion. Multimodality imaging is usually required to establish the preoperative diagnosis, although the final diagnosis is achieved with tissue investigation. Complete surgical resection of the tumour is curative and associated with good long-term prognosis.