Pulmonary lymphangitic sarcomatosis and a review of the literature.

Intrapulmonary spread of a sarcoma via lymphatics is a rare cause of death in a young adult. A 31-year old man was admitted to our hospital complaining of dyspnea and malaise of 2 months' duration. A chest radiography revealed bilateral hilar enlargement, and reticulonodular infiltrations. Thoracic CT-scans demonstrated mediastinal lymphadenopathy, thickening of interlobular septa, polygonal lines, and thickening of bronchovascular bundles. The diagnosis was made by open-lung biopsy. The patient died within 3 months after diagnosis. Pulmonary lymphangitic sarcomatosis is a rare but important manifestation of an angiosarcoma. Optimal treatment of these patients is not well defined, but a trial of chemotherapy may be warranted.