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