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.