PULMONARY LYMPHANGIOLEIOMYOMATOSIS IN TUB EROUS SCLEROSIS

Pulmonary lymphangioleiomyomatosis was diagnosed in a 26-year-old woma n with recurrent pneumothorax by histological evaluation of a lung bio psy obtained during video-thoracoscopy. A tumour of the right kidney h ad been removed 2 years previously; the histological picture was that of an angiomyolipoma. Immunohistochemical staining for anti-smooth muc le actin gave a strongly positive reaction in the tissue of the renal angiomyolipoma and in the pathologic lung tissue. Additional investiga tions showed an asymptomatic intracerebral tumour 5 cm in diameter in the left frontal lobe (brain scan). This multilocal renal, pulmonary a nd cerebral manifestation of benign mesenchymal proliferating tumours supports the classification of this case in the tuberous sclerosis com plex.