[Mediastinal-pulmonary lymphangioleiomyomatosis. The anatomicoclinical aspects]

The lymphangioleiomyomatosis is a rare disease which has a severe course and prognosis and occurs exclusively in females. The symptomatology is not characteristic and the radiological picture is that of a reticular and micronodular diffuse interstitial process. The morphological examination is the only which elucidates the diagnosis. Two cases are reported, the diagnosis of which was established postmortem. In the first case, the site was predominantly pulmonary; in the second case, in addition to the lung, the mediastinal lymph system was also strongly damaged. The morphological changes are characterized by an abnormal proliferation of muscular tissue at the level of the pulmonary interstitium and of the pulmonary and mediastinal lymphatics. The process develops towards destructive lesions of the bronchiolo-alveolar and vascular structures. The pathogenesis is unknown, but genetic and endocrine factors seem to be involved.