Perivascular epithelioid cell tumor of the falciform ligament with recruitment of the paraumbilical vein

We report a case of a perivascular epithelioid cell tumor occurring in the falciform ligament of an otherwise healthy 32-year-old woman. The mass was hypervascular with central necrosis on computed tomography (CT) and contrast-enhanced ultrasound. Our case is unique as CT and ultrasound revealed a paraumbilical vein arising from the expected location in the fissure for the falciform ligament and terminating in the superior aspect of the mass. The vascular anatomy of a recanalized paraumbilical vein, typically associated with portal hypertension, was complete with drainage of blood from the inferior aspect of the mass through the inferior epigastric veins to the femoral veins. The patient had no evidence of liver disease or portal hypertension.