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Abstract
We report a case of sporadic pulmonary lymphangioleiomyomatosis presenting with chylothorax and with parietal pleural involvement by the disease. The implications for the anatomic distribution of lesions in pulmonary lymphangioleiomyomatosis is discussed. The pathogenesis of pulmonary lymphangioleiomyomatosis is reviewed using pulmonary lymphangitic carcinomatosis as a model.
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