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DiseaseTop 23% connected

pleuropulmonary blastoma

A malignant neoplasm affecting the lungs and/or the pleura. Pleuropulmonary blastoma is seen in children. Microscopically, the tumor may show features of chondrosarcoma, leiomyosarcoma, rhabdomyosarcoma, liposarcoma, or undifferentiated sarcoma. In approximately 25% of patients with pleuropulmonary blastoma, there are other lesions or neoplasms that may affect patients or their families, including...

Rare disease
MONDO0011014OMIM601200DOID4769EFO0009052UMLS
Also known as:childhood pulmonary blastoma, paediatric pulmonary blastoma, pediatric pulmonary blastoma, pleuropulmonary blastoma, pleuropulmonary blastoma (morphologic abnormality) +4 more

Ontology

4 subtypes
pleuropulmonary blastoma
fetal lung interstitial tumor
pleuropulmonary blastoma type 1
pleuropulmonary blastoma type 2
pleuropulmonary blastoma type 3

Key Facts

Causal genes
1
GenCC classification
Strong (1 genes)
OMIM status
molecular basis known
OMIM genes
DICER1
Key phenotypes
Medulloblastoma, Pleuropulmonary blastoma, Rhabdomyosarcoma

Relationships

Scores are summed across disease subtypes (may exceed 1.0)
No data available for this relationship type.
C1266144
MeSHC537516