Pulmonary Vein Myxoid Leiomyosarcoma

A case of myxoid leiomyosarcoma located in the right pulmonary veins is presented. The patient complained of progressive dyspnea, orthopnea, sputum cruentum and right chest pain. Angiography revealed an obliteration of right pulmonary veins by a tumor mass that expanded into the left atrium. Histologically, the lesion contained densely packed fusiform cell areas that alternated with other much less cellular and richer in interstitial myxoid matrix. The tumor cells showed specific immunoreactivity to desmin antibodies and contained abundant thin filaments with focal densities and micropinocytic vesicles.