Intravenous leiomyomatosis with ventricular extension

A 38-year-old woman was presented with a 1-month history of recurrent syncope, predominantly occurring in the morning. A total hysteromyomectomy for uterine fibroids was performed 2 years ago. Echocardiography revealed cord-like structures filled in the inferior vena cava (IVC) and extended into the right atrium. The part protruding into the right atrium is ∼7.9 cm × 4.9 cm in size leading the blood flow to detour (Panel A), and it floated into the right ventricle during cardiac diastole (Panel B). Subsequent contrast-enhanced computed tomography demonstrated a 9.1 cm × 7.5 cm irregular, soft tissue mass with heterogeneous enhancement in the pelvis (Panel C), which invaded into and completely occluded the right vena iliac interna, common iliac vein and IVC, and extended into the right atrium and ventricle (Panel D). Volume-rendered image showed abundant tortuous and multiple vertical arteries in the pelvic and intracardiovascular lesions, respectively (Panel E), indicating rich blood supply. Considering the life-threatening complications such as pulmonary embolism caused by dropped mass, the lesions were completely removed at a one-stage surgery. Histopathological examination (see Supplementary data online, Figure S1) confirmed the recurrence of uterine fibroids with intravenous leiomyomatosis. At a 1-year post-operative follow-up, the patient was asymptomatic and felt well.