[Pulmonary lymphangioleiomyomatosis].

The second and third cases of this rare entity reported on in Czechoslovak literature were observed in two females, one 59, the other 24 years old. In both, there was typical diffuse pulmonary involvement with honeycomb transformation, recurrent spontaneous pneumothorax, and cor pulmonale decompensation. In the older patient, there was, at the same time, pronounced paraoesophageal node leiomyomatosis. In the younger one, there were associated renal and hepatic angiomyolipomas such as are found in tuberous sclerosis.