# OnCo record pleuropulmonary-blastoma (cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)". Whole corpus: https://onco.cc/api/v1/onco.nt
@prefix schema: <https://schema.org/> .
@prefix onco: <https://onco.cc/ns#> .
@prefix xsd: <http://www.w3.org/2001/XMLSchema#> .

<https://onco.cc/cancers/pleuropulmonary-blastoma/>
  a schema:MedicalCondition ;
  onco:kind "cancer" ;
  schema:identifier "pleuropulmonary-blastoma" ;
  schema:name "Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours)"@en ;
  schema:alternateName "PPB"@en, "DICER1 syndrome"@en, "Bronchial tumours (childhood)"@en, "Tracheobronchial tumours"@en, "Childhood bronchial carcinoid"@en ;
  schema:description "Primary lung tumours in children are rare and unlike adult lung cancer. Pleuropulmonary blastoma starts as a lung cyst in infants and results from a faulty DICER1 gene that also predisposes to thyroid, ovarian and kidney tumours; removing cysts early, guided by an international registry and family gene testing, prevents progression to the aggressive solid forms."@en ;
  schema:url <https://onco.cc/cancers/pleuropulmonary-blastoma/> ;
  schema:dateModified "2026-09-10"^^xsd:date ;
  schema:sameAs <https://en.wikipedia.org/wiki/Pleuropulmonary_blastoma> ;
  schema:citation <https://www.cancer.gov/types/childhood-cancers/patient/rare-childhood-cancers-pdq>, <https://www.ppbregistry.org/>, <https://doi.org/10.1002/cncr.29062>, <https://doi.org/10.1158/1078-0432.CCR-17-3089> ;
  onco:tag "nci-coverage", "paediatric", "thoracic" ;
  onco:related <https://onco.cc/cancers/inflammatory-myofibroblastic-tumour/>, <https://onco.cc/cancers/rhabdomyosarcoma/>, <https://onco.cc/cancers/rare-childhood-cancers/>, <https://onco.cc/cancers/thyroid/>, <https://onco.cc/cancers/wilms-tumor/>, <https://onco.cc/cancers/hepatoblastoma/> ;
  onco:technologies <https://onco.cc/technologies/germline-testing/>, <https://onco.cc/technologies/ct/>, <https://onco.cc/technologies/cytotoxic-chemotherapy/> ;
  onco:drugs <https://onco.cc/drugs/ifosfamide/>, <https://onco.cc/drugs/vincristine/>, <https://onco.cc/drugs/dactinomycin/>, <https://onco.cc/drugs/doxorubicin/> ;
  onco:companies <https://onco.cc/companies/childrens-oncology-group/> ;
  onco:institutions <https://onco.cc/institutions/siop-europe/> ;
  onco:terms <https://onco.cc/terms/hereditary-cancer-syndromes/>, <https://onco.cc/terms/bronchoscopy/> ;
  onco:bottlenecks <https://onco.cc/bottlenecks/b-rare-cancers/>, <https://onco.cc/bottlenecks/b-hereditary-risk/> ;
  onco:keyPapers <https://onco.cc/key-papers/paper-english-cancer/>, <https://onco.cc/key-papers/paper-schultz-clin-cancer-res/> ;
  onco:pipeline <https://onco.cc/technologies/germline-testing/> ;
  onco:standardOfCare <https://onco.cc/technologies/germline-testing/>, <https://onco.cc/drugs/ifosfamide/>, <https://onco.cc/drugs/vincristine/>, <https://onco.cc/drugs/dactinomycin/>, <https://onco.cc/drugs/doxorubicin/>, <https://onco.cc/terms/bronchoscopy/>, <https://onco.cc/terms/hereditary-cancer-syndromes/>, <https://onco.cc/technologies/germline-testing/> .