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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.
Pleuropulmonary blastoma (PPB) is the archetypal DICER1-related tumour: a mesenchymal malignancy of the lung and pleura in children under about six years. It evolves through recognised stages: type I (purely cystic, infants, excellent outcome with surgery), type Ir (regressed cystic), type II (cystic and solid) and type III (solid), with progressively worse prognosis. About two thirds of children carry a germline loss-of-function DICER1 variant, with a second somatic hotspot mutation in the RNase IIIb domain in the tumour. The same syndrome causes cystic nephroma, Sertoli-Leydig cell tumour of the ovary, multinodular goitre and differentiated thyroid carcinoma, pituitary blastoma, pineoblastoma, embryonal rhabdomyosarcoma of the cervix and nasal chondromesenchymal hamartoma, so a PPB diagnosis triggers testing and surveillance across the family.
Treatment is surgical for type I disease, with adjuvant chemotherapy debated and studied in the registry; types II and III receive multi-agent chemotherapy (ifosfamide, vincristine, dactinomycin and doxorubicin, the IVADo backbone used in rhabdomyosarcoma) plus complete resection, with radiotherapy for residual disease. The International PPB/DICER1 Registry (since 1988) has defined the staging, treatment recommendations and surveillance guidance, including chest imaging for infants known to carry DICER1 variants so that cysts are removed before they become solid tumours.
Other childhood lung and airway tumours are grouped with PPB by the NCI: tracheobronchial carcinoid tumours (the most common primary lung malignancy in children, usually cured by sleeve or lobar resection), mucoepidermoid carcinoma of the bronchus, inflammatory myofibroblastic tumour (ALK-rearranged, see its own record) and rare adult-type carcinomas. The recurring themes are delayed diagnosis because symptoms mimic asthma or pneumonia, the value of bronchoscopy and cross-sectional imaging, and the need for rare-tumour registries rather than randomised trials.
Very rare: pleuropulmonary blastoma is reported in a few dozen children a year worldwide through the International PPB/DICER1 Registry; tracheobronchial tumours in children are mostly carcinoid and mucoepidermoid tumours (NCI PDQ).
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, Pleuropulmonary blastoma (types I, Ir, II and III), 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, 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), Large cell neuroendocrine carcinoma of the lung, Thymoma (WHO types A, AB, B1, B2 and B3), Thymic carcinoma
Complete surgical resection of the cystic lesion; adjuvant chemotherapy is optional and registry-guided; germline DICER1 testing for the child and first-degree relatives.
Neoadjuvant or adjuvant multi-agent chemotherapy (ifosfamide, vincristine, dactinomycin, doxorubicin) with complete resection; radiotherapy for unresectable residual disease.
Bronchoscopic assessment and parenchyma-sparing surgical resection (sleeve resection); systemic therapy rarely needed.
Surveillance per international consensus: chest imaging in infancy and early childhood, abdominal ultrasound, thyroid ultrasound, and awareness of ovarian and other syndrome tumours.
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One cancer page on OnCo cites this paper by its DOI; this record gives the citation a page of its own so a reader can follow it without leaving OnCo. Read the abstract above alongside the citing page listed under Related; the record was created automatically from the Europe PMC entry and its figures have not been checked by hand.
One cancer page on OnCo cites this paper by its DOI; this record gives the citation a page of its own so a reader can follow it without leaving OnCo. Read the abstract above alongside the citing page listed under Related; the record was created automatically from the Europe PMC entry and its figures have not been checked by hand.
Query for this cancer: (TITLE:"Childhood lung and airway tumours" OR ABSTRACT:"Childhood lung and airway tumours" OR TITLE:"pleuropulmonary blastoma, tracheobronchial tumours" OR ABSTRACT:"pleuropulmonary blastoma, tracheobronchial tumours" OR TITLE:"PPB" OR ABSTRACT:"PPB" OR TITLE:"DICER1 syndrome" OR ABSTRACT:"DICER1 syndrome" OR TITLE:"Bronchial tumours childhood" OR ABSTRACT:"Bronchial tumours childhood" OR TITLE:"Tracheobronchial tumours" OR ABSTRACT:"Tracheobronchial tumours") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours), not a curated reading list.
Manivel and colleagues separate PPB from adult pulmonary blastoma; the International PPB Registry is founded.
Hill and colleagues (Science) identify the first microRNA-processing gene tumour syndrome.
Messinger and colleagues (Cancer 2015) report outcomes by type.
No targets or pathways are linked to this cancer yet. Browse the gene hub →
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
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.
Alkylating chemotherapy can damage a blood stem cell in a way that shows up years later as myelodysplastic syndrome or acute myeloid leukaemia. It is uncommon, it depends on the total dose, and the risk falls away after about ten years. Knowing the cumulative dose you were given is the single most useful thing on your treatment summary.
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.
See all on the product pages:Dactinomycin (actinomycin D)DoxorubicinIfosfamideVincristine·Printable cards in the navigator
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