Intravenous leiomyomatosis

Abstract

Introduction. Intravenous leiomyomatosis is a rare uterine neoplasm, about 300 cases have been reported in the literature so far. This condition is characterized by the invasion of the venous uterine vasculature and extrauterine vasculature by smooth muscle tissue. Early diagnosis and determining the degree of extension, increase the probability of successful treatment. In cases with advanced vascular and cardiac compromise, the outcome is uncertain. Case presentation. We report the case of a 35 year old woman who presented at our institution with lower limb edema, dyspnea and pelvic pain. She had a history of uterine miomatosis and intracardiac myxoma resection. Physical examination identified signs of heart failure and an abdominal mass. Radiographic studies and biopsy of the lesion confirm diagnosis of intravenous leiomyomatosis with inferior vena cava and right heart chambers extension. It was proposed to initiate neoadjuvant therapy with GnRH analogues to reduce tumor size and then perform surgical resection. Unfortunately on the third day of diagnosis the patient died from heart failure. Conclusions. Intravenous leiomyomatosis with invasion of inferior vena cava and right heart chambers is uncommon, although this entity is histologically benign, it can be potentially fatal because the cardiovascular structural and functional damage. This disease should be considered in the differential diagnosis of intracardiac tumors involving the vena cava, in women with a history of uterine myomatosis. The treatment of choice is complete extrauterine tumor resection and total hysterectomy with salpingo-oophorectomy.