OBJECTIVETo investigate clinicopathological features, immunophenotype and differential diagnosis of intravenous leiomyomatosis with intracardiac extension.

METHODSClinical manifestations, morphologic features, and immunohistochemical staining were retrospectively analyzed in 19 cases of intravenous leiomyomatosis with intracardiac extension.

RESULTSThe patients' age ranged from 33 to 59 years (mean 44 years). Clinical presentation included chest tightness, palpitation, dyspnea, edema of low extremity, abdominal distention or hypermenorrhea. However, a few patients were asymptomatic. Grossly, intravenous leiomyomatosis in most cases demonstrated coiled or nodular growth within the myometrium with worm-like involvement of the uterine vein in broad ligament or other pelvic veins with continued extension into the vena cava and the heart. The intravenous tumor surface was generally smooth and rubbery, with a greyish-white color. Microscopically, the tumors were composed of spindle cells with rare mitotic figures and the presence of abundant thick-walled vessels. Marked fibrosis, hyalinization, myxoid and edematous changes were common. The tumor cells were positive for SMA, ER, PR and desmin but negative for HMB45 and S-100. CD10 and CD34 were positive in 4 patients and negative in the remaining cases.

CONCLUSIONIntravenous leiomyomatosis with intracardiac extension is a rare disease among women of child-bearing age, with specific morphologic manifestations and immunohistochemical profiles.