Abstract
A rare case of classical tuberous sclerosis complex with pulmonary and lymph node involvement is presented. The lungs showed small cysts or honeycombing associated with smooth muscle proliferation in the walls of bronchioles, blood vessels, lymphatics, and alveolar septa. In the enlarged mediastinal and retroperitoneal lymph nodes, the normal structure was replaced by interlacing bundles of proliferated smooth muscle cells. These features were consistent with those of lymphangiomyomatosis. The close relationship between tuberous sclerosis and lymphangiomyomatosis is discussed. ACTA PATHOL. JPN. 33: 395–401, 1983.
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