Lymphangiomyomatosis with chylous effusions

We report two cases of lymphangiomyomatosis with pleural and peritoneal chylous effusions, one without pulmonary involvement. The first patient died after seven years. The second received external irradiation to control the chylous ascites and remains alive 12 years later. The disease appears to be estrogen-dependent, and bilateral oophorectomy has been recently reported for palliative treatment.