Intravenous leiomyomatosis extending from the inferior caval vein to the pulmonary artery

Intravenous leiomyomatosis presenting as acute Budd-Chiari syndrome

“…[2][3][4][5] The neoplastic smooth muscles of intravenous leiomyomatosis are histologically and cytogenetically similar to benign leiomyomata but may display growth characteristics somewhere between those of benign and malignant smooth muscle tumors. 6,7 Previous case reports describe characteristic imaging features, including nodular, enhancing tumors that appear to originate in the uterus and extend into the venous system, causing expansion of the involved vascular structures.…”

“…6,7 Previous case reports describe characteristic imaging features, including nodular, enhancing tumors that appear to originate in the uterus and extend into the venous system, causing expansion of the involved vascular structures. 2,8 Although many reports describe involvement of local pelvic veins-including uterine, vaginal, and ovarian veins-a few reports describe extension into the common iliac veins, the IVC, renal veins, right atrium, right ventricle, and tumor emboli within the pulmonary arteries and their segmental branches. 2,3 The tumor thrombi are often closely associated with bland intravenous thrombi where clinical signs of venous obstruction are present.…”

“…Nearly all patients with intravenous leiomyomatosis have tumor estrogen-progesterone receptor positivity, and the treatment entails not only aggressive surgical resection but also adjunctive hormonal ablation with removal of ovaries and avoidance of future estrogen therapy. [2][3][4] BCS is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction that involves one or more draining hepatic veins. Typical clinical findings, which include abdominal pain, hepatomegaly, and ascites, are present in almost all patients with BCS.…”

“…[1][2][3][4] Intravenous leiomyomatosis is characterized by proliferation of neoplastic smooth muscle cells within the venous drainage system of the abdomen and pelvis, often secondary to direct intravascular extension of the uterine leiomyomata. 2,3 Several reports describe significant morbidity, often related to deep venous thrombosis or pulmonary thromboembolism, or both, when leiomyomatosis extends into the inferior vena cava (IVC). 2,3 Tricuspid valve dysfunction has been described with further proximal extension of the tumor emboli into the right atrium.…”

“…2,3 Several reports describe significant morbidity, often related to deep venous thrombosis or pulmonary thromboembolism, or both, when leiomyomatosis extends into the inferior vena cava (IVC). 2,3 Tricuspid valve dysfunction has been described with further proximal extension of the tumor emboli into the right atrium. To our knowledge, only one patient with Budd-Chiari syndrome (BCS) as a complication of intravenous leiomyomatosis has been described.…”