Intravenous leiomyomatosis presenting as acute Budd-Chiari syndrome

Intravenous leiomyomatosis is a rare condition that has been described as being associated with venous occlusion by direct intravascular tumor extension, typically from a pelvic organ. We report an exceedingly rare case of intravenous leiomyomatosis extending into the inferior vena cava, leading to pulmonary embolism, hepatic venous outflow obstruction, and an acute Budd-Chiari syndrome. This is the second reported patient with intravenous leiomyomatosis with Budd-Chiari syndrome and, to our knowledge, the first reported patient who survived with surgery. Correlative images, illustrating computed tomography and magnetic resonance imaging findings characteristic of intravenous leiomyomatosis with secondary Budd-Chiari syndrome, are presented and discussed.