pulmonary disorders
Lymphangioleiomyomatosis
Etiology of Lymphangioleiomyomatosis
The cause of lymphangioleiomyomatosis is unknown. The tempting hypothesis that female sex hormones play a role in pathogenesis remains unproved. The disease usually arises spontaneously, but LAM bears many similarities to the pulmonary findings of Tuberous Sclerosis Complex (TSC) (TS); LAM occurs in some patients with TS and is thought by some to be a forme fruste of TS. Mutations in the tuberous sclerosis complex-2 gene ( Somatic mosaicism forTSC-2mutations within the lungs and kidneys results in foci of disease superimposed against a background of normal cells within these tissues (although multiple discrete sites of disease might be expected). LAM represents a low-grade, destructive, metastasizing neoplasm, perhaps of uterine origin, that spreads through the lymphatic system. 