Pleuropulmonary Blastoma

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;

Details

Origin ID

C5669;

UMLS CUI

C1266144;

Automatic exact mappings (from CISMeF team)
- Pleuro-pulmonary blastoma [ICD-11 More detail]
- Pleuropulmonary blastoma [ICD-11 Extension code]
- Pleuropulmonary blastoma [MedDRA Preferred Term]
- Polybrominated biphenyl [LOINC component]
- Polybrominated biphenyl (substance) [SNOMED CT concept]
Currated CISMeF NLP mapping
- Pleuropulmonary blastoma [HRDO Sign]
- Pleuropulmonary blastoma [ICD-O code]
- Pleuropulmonary blastoma [HPO term]
- Pleuropulmonary blastoma [MeSH concept]
- Pleuropulmonary blastoma [OMIM Phenotype]
- Pleuropulmonary blastoma [ORDO Disease]
- Pleuropulmonary blastoma (disorder) [SNOMED CT concept]
- Pleuropulmonary blastoma (morphologic abnormality) [SNOMED CT concept]
- pleuropulmonary blastoma [Disease (Nov.)]
- pleuropulmonary blastoma [DO Concept]
- pleuropulmonary blastoma [MeSH Supplementary Concept]
DO Cross reference
- pleuropulmonary blastoma [DO Concept]
Disease may have findings
- Chest Pain [NCIt concept]
- Cough [NCIt concept]
- Fever [NCIt concept]
- Pneumothorax [NCIt concept]
- Respiratory Distress [NCIt concept]
Excludes anatomical site(s)
- Neck [NCIt concept]
Has associated anatomic sites
- Lung [NCIt concept]
- Pleural Cavity [NCIt concept]
- Respiratory System [NCIt concept]
- Thoracic Cavity [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- Pleuropulmonary blastoma [MeSH concept]
- Pleuropulmonary blastoma [OMIM Phenotype]
- Pleuropulmonary blastoma [HPO term]
- Pleuropulmonary blastoma [ORDO Disease]
- Pleuropulmonary blastoma [HRDO Sign]
- Pleuropulmonary blastoma [ICD-O code]
- Pleuropulmonary blastoma [MedDRA Preferred Term]
- Pleuropulmonary blastoma (disorder) [SNOMED CT concept]
- Pleuropulmonary blastoma (morphologic abnormality) [SNOMED CT concept]
- pleuropulmonary blastoma [MeSH Supplementary Concept]
- pleuropulmonary blastoma [DO Concept]
- pleuropulmonary blastoma [Disease (Nov.)]
concept_is_in_subset
- CPTAC Codelists Terminology [NCIt concept]
- CPTAC Neoplasms Codelist [NCIt concept]
- CPTAC Terminology [NCIt concept]
- CTRP Disease Terminology [NCIt concept]
- CTRP Terminology [NCIt concept]
- CTS-API Disease Main Type Terminology [NCIt concept]
- Clinical Trials Search-Application Programming Interface Disease Category Terminology [NCIt concept]
- GDC Terminology [NCIt concept]
- GDC Value Terminology [NCIt concept]
- Mapped ICDO3.1 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.1 Morphology Terminology [NCIt concept]
- Mapped ICDO3.1 Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology Terminology [NCIt concept]
- Mapped ICDO3.2 Terminology [NCIt concept]
- NCIt Neoplasm Core Terminology [NCIt concept]
- NICHD Terminology [NCIt concept]
- Pediatric Hematology-Oncology Terminology [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
disease_excludes_normal_tissue_origin
- Germinal Layer [NCIt concept]
disease_has_abnormal_cell
- Malignant Blastemal Cell [NCIt concept]
- Malignant Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
disease_has_associated_disease
- DICER1 Syndrome [NCIt concept]
disease_has_finding
- Childhood Lesion [NCIt concept]
- DICER1 Gene Alteration Positive [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
disease_has_molecular_abnormality
- DICER1 Gene Mutation [NCIt concept]
disease_has_normal_cell_origin
- Embryonic Cell [NCIt concept]
disease_has_normal_tissue_origin
- Embryonic Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Thoracic Cavity [NCIt concept]
disease_mapped_to_gene
- DICER1 Gene [NCIt concept]
disease_may_have_associated_disease
- Adult Cystic Nephroma [NCIt concept]
- Colon Polyp [NCIt concept]
- Ovarian Teratoma [NCIt concept]
disease_may_have_cytogenetic_abnormality
- Gain of Chromosome 8 [NCIt concept]
disease_may_have_molecular_abnormality
- TP53 Gene Inactivation [NCIt concept]
neoplasm_has_special_category
- Solid Neoplasm [NCIt concept]

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