Pulmonary lymphangiomyomatosis: treatment with castration.

Pulmonary lymphangiomyomatosis (PLM) is a rare disease of smooth muscle proliferation that generally leads to an early death due to respiratory insufficiency. The disease only affects women. Exacerbations with pregnancy and after hormonal manipulation have been documented. There is presently no successful treatment of the disease. Castration as a means of early therapy has been associated with a stable respiratory picture in our patient. Peritoneal-jugular shunt can benefit women with significant ascities.