Pulmonary artery leiomyosarcoma

Pulmonary artery leiomyosarcoma is a rare but highly lethal disease, and ca n be mistaken for pulmonary thromboembolism. We report a case of pulmonary artery leiomyosarcoma managed with surgical resection, chemotherapy, and ra diotherapy. A 57-year-old woman was admitted with complaints of aggravated dyspnea. She was initially treated with oxygen therapy and heparinization f or a suspected pulmonary embolism. Echocardiography revealed a dilated righ t atrium and ventricle and severe tricuspid regurgitation, with an estimate d systolic right ventricular pressure of 95 mm Hg; a shadow of a mass in th e main pulmonary artery was also noted. Right ventriculography revealed a f illing defect, and to-and-fro motion of the mass in the main pulmonary arte ry. The left pulmonary artery was almost totally occluded by the mass. The patient's condition improved dramatically after palliative excision of the mass and patch reconstruction of the outflow tract of the right ventricle w ith a bicuspid xenograft. Pathologic examination of the mass revealed leiom yosarcoma. Chemotherapy and radiotherapy were subsequently administered and follow-up imaging studies 3 months postoperatively revealed no recurrence of the tumor. The patient remains well, more than 1 year after treatment. T his report emphasises that pulmonary artery sarcoma should be considered in the differential diagnosis in cases of suspected pulmonary thromboembolism .