Pulmonary tuberous sclerosis treated with tamoxifen and progesterone.

Tuberous sclerosis is a rare disease and pulmonarymanifestations are extremely rare. Like pulmonary lymph-angioleiomyomatosis, tuberous sclerosis ofthe lung affectsonly women of childbearing age and carries a very poorprognosis. Theradiological andpathological features ofthetwo conditions in the lung are indistinguishable and someauthorsthereforeconsiderlymphangioleiomyomatosistobeaformefruste oftuberoussclerosis,1 althoughothersbelievethat the relationship is unclear.2In pulmonary lymphangioleiomyomatosis bilateraloophorectomy and high dose progesterone have sometimesbeen