Pulmonary lymphangioleiomyomatosis: a case report in postmenopausal woman treated with pleurodesis and progesterone (medroxyprogesterone acetate).

The main problem in the treatment of pulmonary lymphangioleiomyomatosis, which frequently occurs in the reproductive age, is the control of chylothorax and disease progression. We herein report a case of a 62-year-old woman who underwent surgery for recurrent chylothorax. Histologic examination of lung and lymph node biopsies demonstrated lymphangioleiomyomatosis. Thirty-six months after tetracycline pleurodesis and high-dose medroxyprogesterone acetate therapy, the disease was stable and cyclothorax effectively controlled. It would therefore appear that hormonal treatment with medroxyprogesterone acetate may be beneficial in postmenopausal women.