Type I Pleuropulmonary Blastoma

Preferred Label : Type I Pleuropulmonary Blastoma;

NCIt related terms : Type I PPB;

NCIt definition : A subtype of pleuropulmonary blastoma characterized by the presence of cysts with a microscopic population of primitive malignant small cells. There is no evidence of a malignant solid mesenchymal component. It usually has a good prognosis compared to types II and III pleuropulmonary blastoma.;

Alternative definition : NICHD: A pleuropulmonary blastoma comprising malignant small cells in a cystic pattern.;

Neoplastic status : Malignant;

NCI Metathesaurus CUI : CL497419;

Details

Origin ID

C45626;

UMLS CUI

C1710501;

Automatic exact mappings (from CISMeF team)
- Pleuro-pulmonary blastoma type I [ICD-11 More detail]
- Pleuropulmonary blastoma type 1 [ORDO Disease]
- Pleuropulmonary blastoma type I (disorder) [SNOMED CT 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 type I (disorder) [SNOMED CT concept]
concept_is_in_subset
- NCIt Neoplasm Core Terminology [NCIt concept]
- NICHD Terminology [NCIt concept]
- Pediatric Hematology-Oncology Terminology [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Solid Growth Pattern [NCIt concept]
disease_excludes_normal_tissue_origin
- Germinal Layer [NCIt concept]
disease_has_abnormal_cell
- Malignant Blastemal Cell [NCIt concept]
- Malignant Cell [NCIt concept]
- Malignant Small Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
disease_has_associated_disease
- DICER1 Syndrome [NCIt concept]
disease_has_finding
- Childhood Lesion [NCIt concept]
- Cystic Change [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]

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