Abstract
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.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 902-906 |
| Number of pages | 5 |
| Journal | Modern Pathology |
| Volume | 8 |
| Issue number | 9 |
| State | Published - Dec 1 1995 |
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Keywords
- atypical adenomatoid proliferation
- lymphangioleiomyomatosis
- multifocal micronodular hyperplasia of pneumocytes
- tuberous sclerosis
ASJC Scopus subject areas
- Pathology and Forensic Medicine
Cite this
Multifocal micronodular pneumocyte hyperplasia : A distinctive pulmonary manifestation of tuberous sclerosis. / Guinee, D.; Singh, R.; Azumi, N.; Singh, Gurmukh; Przygodzki, R. M.; Travis, W.; Koss, M.
In: Modern Pathology, Vol. 8, No. 9, 01.12.1995, p. 902-906.Research output: Contribution to journal › Article
}
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, Gurmukh
AU - Przygodzki, R. M.
AU - Travis, W.
AU - Koss, M.
PY - 1995/12/1
Y1 - 1995/12/1
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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UR - http://www.scopus.com/inward/citedby.url?scp=0029618374&partnerID=8YFLogxK
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 -