# OnCo record pleuropulmonary-blastoma-dicer1 (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-dicer1/>
  a schema:MedicalCondition ;
  onco:kind "cancer" ;
  schema:identifier "pleuropulmonary-blastoma-dicer1" ;
  schema:name "Pleuropulmonary blastoma (types I, Ir, II and III)"@en ;
  schema:alternateName "PPB"@en, "Pleuropulmonary blastoma type I"@en, "Pleuropulmonary blastoma type Ir"@en, "Pleuropulmonary blastoma type II"@en, "Pleuropulmonary blastoma type III"@en, "DICER1-related pleuropulmonary blastoma"@en ;
  schema:description "Pleuropulmonary blastoma is a rare lung cancer of young children, and the signature tumour of the inherited DICER1 syndrome. It starts as a lung cyst (type I) that can turn into a solid, aggressive tumour (types II and III). Cysts are removed surgically with an excellent outlook; solid tumours need chemotherapy as well, and every family is offered DICER1 testing."@en ;
  schema:url <https://onco.cc/cancers/pleuropulmonary-blastoma-dicer1/> ;
  schema:dateModified "2026-09-24"^^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://doi.org/10.1002/cncr.29032>, <https://doi.org/10.1016/j.ygyno.2011.03.024> ;
  onco:tag "subtype-page", "wave4", "rare" ;
  onco:related <https://onco.cc/cancers/pleuropulmonary-blastoma/>, <https://onco.cc/cancers/pulmonary-blastoma/>, <https://onco.cc/cancers/pineoblastoma/>, <https://onco.cc/cancers/childhood-cancers/>, <https://onco.cc/cancers/granulosa-cell-tumour/> ;
  onco:technologies <https://onco.cc/technologies/germline-testing/> ;
  onco:targets <https://onco.cc/targets/dicer1/> ;
  onco:drugs <https://onco.cc/drugs/ifosfamide/>, <https://onco.cc/drugs/vincristine/>, <https://onco.cc/drugs/doxorubicin/> ;
  onco:standardOfCare <https://onco.cc/cancers/pleuropulmonary-blastoma/>, <https://onco.cc/technologies/germline-testing/>, <https://onco.cc/drugs/ifosfamide/>, <https://onco.cc/drugs/vincristine/>, <https://onco.cc/drugs/doxorubicin/>, <https://onco.cc/cancers/pleuropulmonary-blastoma/> .