Loading
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.
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).
How 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.
How common: no incidence figure; 350 confirmed cases in the international registry (Messinger 2015).
Treatment: 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).
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).
Central tumours arise in the large airways, peripheral ones in the alveoli; both drain to hilar then mediastinal nodes, and the pleural lining is a separate cancer site.
Same organ: Mediastinal germ cell tumour, Type A and type AB thymoma, Type B1 and type B2 thymoma, Type B3 thymoma, Micronodular thymoma with lymphoid stroma, Adenocarcinoma of the lung, Squamous cell carcinoma of the lung, Large cell carcinoma of the lung, Sarcomatoid carcinoma of the lung, Adenosquamous carcinoma of the lung, Invasive mucinous adenocarcinoma of the lung, Adenocarcinoma in situ and minimally invasive adenocarcinoma of the lung, Basaloid squamous cell carcinoma of the lung, Lymphoepithelial carcinoma of the lung, Pulmonary blastoma (adult), Non-small-cell lung cancer, Lung cancer (all types), Small-cell lung cancer, Mesothelioma, Pleural mesothelioma, Thymoma and thymic carcinoma, Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours), Inflammatory myofibroblastic tumour (IMT), EGFR-mutated non-small-cell lung cancer, ALK-positive non-small-cell lung cancer, KRAS G12C-mutant non-small-cell lung cancer, ROS1-positive non-small-cell lung cancer, MET exon 14 and MET-amplified non-small-cell lung cancer, RET fusion-positive non-small-cell lung cancer, BRAF V600E-mutant non-small-cell lung cancer, HER2-mutant non-small-cell lung cancer, NTRK fusion-positive non-small-cell lung cancer, PD-L1-high non-small-cell lung cancer without a driver mutation, Resectable stage I to III non-small-cell lung cancer, Unresectable stage III non-small-cell lung cancer, Limited-stage small-cell lung cancer, Extensive-stage small-cell lung cancer, Lung neuroendocrine tumours (typical and atypical carcinoid), Thymoma (WHO types A, AB, B1, B2 and B3), Thymic carcinoma
Complete resection of the cyst; chemotherapy debated; DICER1 testing and surveillance.
Resection and multi-agent chemotherapy (ifosfamide, vincristine, actinomycin, doxorubicin), radiotherapy for residual disease.
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Query for this cancer: (TITLE:"Pleuropulmonary blastoma" OR ABSTRACT:"Pleuropulmonary blastoma" OR TITLE:"types I, Ir, II and III" OR ABSTRACT:"types I, Ir, II and III" OR TITLE:"PPB" OR ABSTRACT:"PPB" OR TITLE:"Pleuropulmonary blastoma type I" OR ABSTRACT:"Pleuropulmonary blastoma type I" OR TITLE:"Pleuropulmonary blastoma type Ir" OR ABSTRACT:"Pleuropulmonary blastoma type Ir" OR TITLE:"Pleuropulmonary blastoma type II" OR ABSTRACT:"Pleuropulmonary blastoma type II") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Pleuropulmonary blastoma (types I, Ir, II and III), not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Fatal if given intrathecally: label all syringes.
Reduce by 50% for bilirubin 20-50 µmol/L and 75% for 50-85 µmol/L.
Heart damage from cancer treatment: anthracyclines weaken the heart muscle permanently in a dose-related way, trastuzumab does so reversibly, and some kinase inhibitors raise blood pressure or disturb rhythm. Heart function (LVEF) is monitored by ultrasound during treatment.
Nerve damage from chemotherapy that causes numbness, tingling and pain in the hands and feet, and sometimes weakness or hearing loss. It builds up with each dose, can be permanent, and is the main reason oxaliplatin, taxanes and vincristine have to be stopped or reduced.
See all on the product pages:DoxorubicinIfosfamideVincristine·Printable cards in the navigator
Newly diagnosed? Read the first 60 days with Pleuropulmonary blastoma, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.