Multifocal micronodular pneumocyte hyperplasia, lymphangiomyomatosis and clear cell micronodules of the lung in a Chinese female patient with tuberous sclerosis

Abstract

Tuberous sclerosis is a rare genetic disease with protean clinical manifestations. The lesion most commonly described in the lung is lymphangiomyomatosis. There have been recent reports of multifocal micronodular pneumocyte hyperplasia, as well as a single case documentation of a clear cell tumor of the lung, in patients affected by the disease. We detail a case of a female Chinese patient with tuberous sclerosis who was incidentally discovered to have bilateral pneumothoraces. The open lung biopsy revealed combined histological features of multifocal micronodular pneumocyte hyperplasia, lymphangiomyomatosis and clear cell micronodules.