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Pleuropulmonary blastoma MeSH Supplementary Concept Data 2025


MeSH Supplementary
Pleuropulmonary blastoma
Unique ID
C537516
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/C537516
Registry Numbers
0
Heading Mapped to
*Pulmonary Blastoma
Frequency
147
Note
A rare pediatric pulmonary blastoma that arises during fetal lung development and is often part of an inherited cancer syndrome. Tumors contain both EPITHELIAL CELLS and mesenchymal cells and arise from cysts in the lung airspace epithelium. In some patients, overgrowth of the mesenchymal cells produces a SARCOMA, which is associated with a poorer prognosis. Germline mutations in the DICER1 gene have been identified. OMIM: 601200
Date of Entry
2010/08/25
Revision Date
2015/08/18
Pleuropulmonary blastoma Preferred
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