Preferred Label : Pleuropulmonary Blastoma;
NCIt synonyms : Pulmonary Blastoma of Childhood; PPB;
NCIt definition : 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 lung or kidney cysts, and ovarian
or testicular neoplasms. Heterozygous germline mutations in DICER1 gene have been
identified in families harboring pleuropulmonary blastomas.;
Alternative definition : NICHD: 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 there is a predisposition for other tumors. Heterozygous germline
mutations in DICER1 gene have been identified in families harboring pleuropulmonary
blastomas.; NCI-GLOSS: A rare and very aggressive (fast-growing) cancer that forms in tissues
of the lung and pleura (a thin layer of tissue that covers the lungs and lines the
interior wall of the chest cavity). Pleuropulmonary blastoma is most common in children.;
Neoplastic status : Malignant;
ICD-O code : 8973/3;
Codes from synonyms : CDR0000446557;
Origin ID : C5669;
UMLS CUI : C1266144;
- Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- DO Cross reference
- Disease may have findings
- Excludes anatomical site(s)
- Has associated anatomic sites
- Semantic type(s)
- UMLS correspondences (same concept)
- concept_is_in_subset
- disease_excludes_finding
- disease_excludes_normal_tissue_origin
- disease_has_abnormal_cell
- disease_has_associated_disease
- disease_has_finding
- disease_has_molecular_abnormality
- disease_has_normal_cell_origin
- disease_has_normal_tissue_origin
- disease_has_primary_anatomic_site
- disease_mapped_to_gene
- disease_may_have_associated_disease
- disease_may_have_cytogenetic_abnormality
- disease_may_have_molecular_abnormality
- neoplasm_has_special_category