Treatment of Disseminated Intravenous Leiomyomatosis With ALK Targeting Crizotinib: A Successful Case Report

摘要

Introduction: Intravenous leiomyomatosis (IVL) is a rare and benign smooth muscle tumor known to be a variant of uterine leiomyomas, with the same histologic appearance but with a different growth pattern that extends through great vessels. Despite the benign histology, with no tissue invasion, IVL has a very aggressive pattern and can extend through the lumen of the iliac vein and upward to varying locations, including the inferior vena cava (IVC) and the right heart, resulting in intracardiac leiomyomatosis. Because of its insidious growth pattern and involvement of great vessels, IVL can lead to heart failure, pulmonary embolism, and sudden cardiac death because of obstruction of the venous return. Surgical treatment for this benign tumor results in low recurrence rates when complete and early resection is done. Given the presence of estrogen and progesterone receptors in leiomyoma cells, alternative approaches include an oophorectomy or hormonal therapies in combination with surgery. For patients with life-threatening unresectable tumors, few options remain after the failure of hormonal therapy. Here, we present a case of a patient with IVL who developed a rapid and aggressive recurrence despite early treatment with surgery and hormonal therapy. Fortunately, the use of next-generation sequencing (NGS) of the tumor allowed for a targeted therapeutic approach, leading to a drastic response.