Micronodular hyperplasia of type II pneumocytes--a new lung lesion associated with tuberous sclerosis.
Open lung biopsy in a 38-year-old female with Pringle-Bourneville syndrome and recurrent pneumothorax revealed a micronodular pneumocyte II hyperplasia, a new entity probably associated with the tuberous sclerosis syndrome. The lesion caused an obstruction of the alveolar lymphatic vessels and alveolar ducts, resulting in an emphysema-like picture. This cystic dilation of alveoli and draining lymphatics followed by rupture caused the recurrent pneumothorax. The epithelial pneumocytic nature of the lesion was confirmed by immunohistochemistry and electronmicroscopy.
Popper
H H
HH
Institute of Pathology, University of Graz, Austria.
Juettner-Smolle
F M
FM
Pongratz
M G
MG
D000328:Adult; D015415:Biomarkers; D001706:Biopsy; D005260:Female; D006801:Humans; D006965:Hyperplasia / Q000209:etiology / Q000473:pathology; D007150:Immunohistochemistry; D008173:Lung Diseases, Obstructive / Q000209:etiology / Q000473:pathology*; D011030:Pneumothorax / Q000175:diagnosis* / Q000209:etiology; D014402:Tuberous Sclerosis / Q000150:complications*
D015415:Biomarkers