Angiomyolipomas in a case of lymphangiomyomatosis syndrome: Relationships to tuberous sclerosis

In 17 of the 31 reported cases of lymphangiomyoma, cystic pulmonary changes have been noted which are similar to those seen in some patients with tuberous sclerosis. The contention that lymphangiomyoma represents a forme fruste of tuberous sclerosis is based on this observation, and is strengthened by reports of lymphangiomyoma in patients with tuberous sclerosis and cystic pulmonary changes. Further support is presented here where bilateral renal angiomyolipomas, virtually confined to patients with tuberous sclerosis, were discovered in association with extensive lymphangiomyoma and cystic pulmonary changes.