Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis
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Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis | |
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Diagnosis in short | |
Micronodule of pneumocyte hyperplasia in multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis. H&E stain. | |
LM DDx | atypical adenomatous hyperplasia of the lung |
Site | lung |
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Associated Dx | lymphangioleiomyomatosis - also assoc. with tuberous sclerosis |
Syndromes | tuberous sclerosis |
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Prevalence | rare |
Prognosis | benign |
Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis, also multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis, is the presence of rare hamartomatous lesions in the lung seen in tuberous sclerosis.[1]
General
- Rare.
Gross
Features:[2]
- Multiple small lung nodules - apparently random distribution.
Microscopic
Features:
- Macrophages within the air spaces.
- Enlarged alveolar lining cells with:
- Hobnail morphology - free (luminal) surface area > attached/basal surface area.
- Round or oval nuclei.
DDx:
- Atypical adenomatous hyperplasia of the lung - usu. do not have macrophages within the air spaces.
Images
See also
References
- ↑ Nagar, AM.; Teh, HS.; Khoo, RN.; Morani, AC.; Vrishni, K.; Raghuram, J. (Feb 2008). "Multifocal pneumocyte hyperplasia in tuberous sclerosis.". Thorax 63 (2): 186. doi:10.1136/thx.2006.076604. PMID 18234663.
- ↑ Kobashi, Y.; Sugiu, T.; Mouri, K.; Irei, T.; Nakata, M.; Oka, M. (Jun 2008). "Multifocal micronodular pneumocyte hyperplasia associated with tuberous sclerosis: differentiation from multiple atypical adenomatous hyperplasia.". Jpn J Clin Oncol 38 (6): 451-4. doi:10.1093/jjco/hyn042. PMID 18535095.