{"entity":{"id":"pleuropulmonary-blastoma-dicer1","kind":"cancer","name":"Pleuropulmonary blastoma (types I, Ir, II and III)","aka":["PPB","Pleuropulmonary blastoma type I","Pleuropulmonary blastoma type Ir","Pleuropulmonary blastoma type II","Pleuropulmonary blastoma type III","DICER1-related pleuropulmonary blastoma"],"tldr":"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.","summary":"Pleuropulmonary blastoma is a dysontogenetic mesenchymal tumour of the lung and pleura of early childhood with three subtypes on a progression pathway, from type I (purely cystic) to type II (cystic and solid) and type III (solid), plus type Ir, a regressed cystic form; a germline DICER1 mutation is the cause in the majority (Messinger 2015). In the 350 registry-confirmed cases (85 of 435 submitted cases, 20 percent, proved to be another entity on review), five-year overall survival was 91 percent for type I and Ir, with all deaths in that group from progression to type II or III, and survival was significantly better for type II than type III (Messinger 2015). The DICER1 syndrome also produces ovarian Sertoli-Leydig cell tumours, cystic nephroma, thyroid nodules and other tumours in the same children and families (Gynecologic Oncology 2011).\n\nHow it differs from its parent: the parent page is the corpus's umbrella for all childhood lung and airway tumours; this page is the entity itself, with its subtype pathway, its registry survival figures and its syndrome. Adult pulmonary blastoma is a different tumour with its own page.\n\nHow common: no incidence figure; 350 confirmed cases in the international registry (Messinger 2015).\n\nTreatment: complete resection for type I and Ir cysts, with chemotherapy debated for type I; resection and multi-agent chemotherapy (registry regimens based on ifosfamide, vincristine, actinomycin and doxorubicin) for types II and III, with radiotherapy for residual disease; germline DICER1 testing and surveillance for the child and relatives (Messinger 2015; NCI PDQ rare cancers of childhood).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Pleuropulmonary_blastoma","links":[{"label":"NCI PDQ: rare cancers of childhood","url":"https://www.cancer.gov/types/childhood-cancers/patient/rare-childhood-cancers-pdq"},{"label":"Messinger 2015, Cancer: 350 central pathology-confirmed pleuropulmonary blastoma cases, International PPB Registry","url":"https://doi.org/10.1002/cncr.29032"},{"label":"Gynecologic Oncology 2011: ovarian sex cord-stromal tumours, pleuropulmonary blastoma and DICER1, International PPB Registry","url":"https://doi.org/10.1016/j.ygyno.2011.03.024"}],"tags":["subtype-page","wave4","rare"],"related":["pleuropulmonary-blastoma","pulmonary-blastoma","pineoblastoma","childhood-cancers","granulosa-cell-tumour"],"cancers":[],"sections":[],"technologies":["germline-testing"],"targets":["dicer1"],"drugs":["ifosfamide","vincristine","doxorubicin"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"paediatric","burden":"Very rare: the International Pleuropulmonary Blastoma Registry confirmed 350 cases by central pathology review, 33 percent type I or Ir, 35 percent type II and 32 percent type III, with median ages at diagnosis of 8, 35 and 41 months (Messinger 2015).","subtypes":["Pleuropulmonary blastoma type I (cystic; median age 8 months; 91 percent five-year survival with type Ir)","Pleuropulmonary blastoma type Ir (regressed cystic)","Pleuropulmonary blastoma type II (cystic and solid; median age 35 months)","Pleuropulmonary blastoma type III (solid; median age 41 months; worst outcome)","Pleuropulmonary blastoma in DICER1 syndrome families"],"biomarkers":["Germline DICER1 mutation (the majority)","Subtype I, Ir, II or III on central pathology review","Somatic DICER1 RNase IIIb hotspot mutation","Family history of DICER1 syndrome tumours"],"standardOfCare":[{"setting":"Type I and Ir","approach":"Complete resection of the cyst; chemotherapy debated; DICER1 testing and surveillance.","refs":["pleuropulmonary-blastoma","germline-testing"]},{"setting":"Type II and III","approach":"Resection and multi-agent chemotherapy (ifosfamide, vincristine, actinomycin, doxorubicin), radiotherapy for residual disease.","refs":["ifosfamide","vincristine","doxorubicin","pleuropulmonary-blastoma"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"pleuropulmonary-blastoma"},"route":"/cancers/pleuropulmonary-blastoma-dicer1/","neighbours":{"cancer":[{"id":"granulosa-cell-tumour","kind":"cancer","name":"Adult granulosa cell tumour of the ovary","route":"/cancers/granulosa-cell-tumour/"},{"id":"childhood-cancers","kind":"cancer","name":"Childhood cancers (all types)","route":"/cancers/childhood-cancers/"},{"id":"pleuropulmonary-blastoma","kind":"cancer","name":"Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours)","route":"/cancers/pleuropulmonary-blastoma/"},{"id":"pineoblastoma","kind":"cancer","name":"Pineoblastoma","route":"/cancers/pineoblastoma/"},{"id":"pulmonary-blastoma","kind":"cancer","name":"Pulmonary blastoma (adult)","route":"/cancers/pulmonary-blastoma/"}],"technology":[{"id":"germline-testing","kind":"technology","name":"Germline (hereditary) testing","route":"/technologies/germline-testing/"}],"target":[{"id":"dicer1","kind":"target","name":"DICER1","route":"/targets/dicer1/"}],"drug":[{"id":"doxorubicin","kind":"drug","name":"Doxorubicin","route":"/drugs/doxorubicin/"},{"id":"ifosfamide","kind":"drug","name":"Ifosfamide","route":"/drugs/ifosfamide/"},{"id":"vincristine","kind":"drug","name":"Vincristine","route":"/drugs/vincristine/"}]}}