TY - JOUR
T1 - Multifocal micronodular pneumocyte hyperplasia
T2 - A distinctive pulmonary manifestation of tuberous sclerosis
AU - Guinee, D.
AU - Singh, R.
AU - Azumi, N.
AU - Singh, G.
AU - Przygodzki, R. M.
AU - Travis, W.
AU - Koss, M.
N1 - Copyright:
Copyright 2007 Elsevier B.V., All rights reserved.
PY - 1995
Y1 - 1995
N2 - We report a peculiar multifocal micronodular proliferation of pneumocytes occurring in a 24-yr-old woman with tuberous sclerosis and lymphangioleiomyomatosis. A computed tomographic scan of the chest demonstrated multiple minute nodules present throughout both lung fields. Histologically, the nodules were well demarcated, measured up to 1.6 mm in diameter, and were composed of thickened, fibrotic, alveolar septa lined by pleomorphic, type II pneumocytes. Positive immunohistochemical stains for keratin, BER-EP4, and surfactant, and negative immunohistochemical staining with an antibody recognizing Clara cells support an epithelial origin from type II pneumocytes. The absence of immunohistochemical staining for HMB45 suggests a histogenesis separate than the lesions of lymphangioleiomyomatosis. We failed to detect estrogen or progesterone receptors in either the lesions of lymphangioleiomyomatosis or the micronodular proliferations. Recognition of these unique lesions facilitates their distinction from other epithelial proliferations, particularly atypical bronchioloalveolar cell hyperplasia. This lesion appears to be a distinctive manifestation of tuberous sclerosis. It is probably hamartomatous.
AB - We report a peculiar multifocal micronodular proliferation of pneumocytes occurring in a 24-yr-old woman with tuberous sclerosis and lymphangioleiomyomatosis. A computed tomographic scan of the chest demonstrated multiple minute nodules present throughout both lung fields. Histologically, the nodules were well demarcated, measured up to 1.6 mm in diameter, and were composed of thickened, fibrotic, alveolar septa lined by pleomorphic, type II pneumocytes. Positive immunohistochemical stains for keratin, BER-EP4, and surfactant, and negative immunohistochemical staining with an antibody recognizing Clara cells support an epithelial origin from type II pneumocytes. The absence of immunohistochemical staining for HMB45 suggests a histogenesis separate than the lesions of lymphangioleiomyomatosis. We failed to detect estrogen or progesterone receptors in either the lesions of lymphangioleiomyomatosis or the micronodular proliferations. Recognition of these unique lesions facilitates their distinction from other epithelial proliferations, particularly atypical bronchioloalveolar cell hyperplasia. This lesion appears to be a distinctive manifestation of tuberous sclerosis. It is probably hamartomatous.
KW - atypical adenomatoid proliferation
KW - lymphangioleiomyomatosis
KW - multifocal micronodular hyperplasia of pneumocytes
KW - tuberous sclerosis
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M3 - Article
C2 - 8751329
AN - SCOPUS:0029618374
VL - 8
SP - 902
EP - 906
JO - Modern Pathology
JF - Modern Pathology
SN - 0893-3952
IS - 9
ER -