{"entity":{"id":"pulmonary-blastoma","kind":"cancer","name":"Pulmonary blastoma (adult)","aka":["Classic biphasic pulmonary blastoma","Biphasic pulmonary blastoma","Adult pulmonary blastoma","Well-differentiated fetal adenocarcinoma (the monophasic epithelial form, now classed as fetal adenocarcinoma)"],"tldr":"Pulmonary blastoma is a very rare lung cancer of adults that mixes a fetal-looking gland component with a primitive sarcoma-like component, and so belongs with the sarcomatoid lung cancers. It is different from the pleuropulmonary blastoma of young children. Surgery is the treatment; chemotherapy has no proven benefit and the outlook is poor.","summary":"The WHO classification places classic biphasic pulmonary blastoma among the sarcomatoid carcinomas: a tumour with fetal-type adenocarcinoma glands and a primitive blastematous stroma, distinct from the pure fetal adenocarcinoma (formerly well-differentiated fetal adenocarcinoma) that sits with adenocarcinoma variants, and from pleuropulmonary blastoma, a childhood DICER1-related tumour with its own page (Nicholson 2022). Pulmonary blastomas make up 0.25 to 0.5 percent of malignant lung neoplasms; most patients are adults with an average age of 43, tobacco use is a causative factor, 40 percent are asymptomatic and the usual finding is a large peripheral nodule (Lung Cancer 2011). Somatic DICER1 mutations have been reported in adult-onset pulmonary blastoma, linking it biologically to the childhood tumour (European Respiratory Journal 2016).\n\nHow it differs from its parent: it is the only non-small-cell lung cancer with embryonic-type tissue, its patients are two decades younger than the lung cancer average, and it is not treated with the parent's driver or immunotherapy pathways because no evidence exists for them.\n\nHow common: 0.25 to 0.5 percent of malignant lung tumours (Lung Cancer 2011).\n\nTreatment: surgical excision is the treatment of choice; the efficacy of adjuvant chemotherapy and radiotherapy is not established; overall five-year survival is 16 percent, better for the fetal adenocarcinoma form, and worse with biphasic type, recurrence, metastasis at presentation, size over 5 cm and node involvement (Lung Cancer 2011).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Pulmonary_blastoma","links":[{"label":"Nicholson 2022, JTO: the 2021 WHO classification of lung tumours","url":"https://doi.org/10.1016/j.jtho.2021.11.003"},{"label":"Lung Cancer 2011: classic biphasic pulmonary blastoma, case report and review","url":"https://doi.org/10.1016/j.lungcan.2011.03.018"},{"label":"European Respiratory Journal 2016: somatic DICER1 mutations in adult-onset pulmonary blastoma","url":"https://doi.org/10.1183/13993003.00172-2016"}],"tags":["subtype-page","wave4","lung"],"related":["nsclc","pulmonary-sarcomatoid-carcinoma","pleuropulmonary-blastoma","lung-adenocarcinoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":["lobectomy"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"lung","burden":"0.25 to 0.5 percent of malignant lung tumours; average age 43; overall five-year survival 16 percent (Lung Cancer 2011 review).","subtypes":["Classic biphasic pulmonary blastoma (fetal adenocarcinoma glands with blastematous stroma)","Fetal adenocarcinoma, low and high grade (the monophasic epithelial form, classed with adenocarcinoma)","DICER1-mutated adult pulmonary blastoma"],"biomarkers":["Biphasic histology with fetal-type glands","Somatic DICER1 mutation (reported)","Tumour size over 5 cm and node status (prognostic)"],"standardOfCare":[{"setting":"All stages","approach":"Surgical excision; adjuvant chemotherapy and radiotherapy of unproven benefit; no trial exists.","refs":["pulmonary-sarcomatoid-carcinoma","lobectomy"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"nsclc"},"route":"/cancers/pulmonary-blastoma/","neighbours":{"cancer":[{"id":"lung-adenocarcinoma","kind":"cancer","name":"Adenocarcinoma of the lung","route":"/cancers/lung-adenocarcinoma/"},{"id":"pleuropulmonary-blastoma","kind":"cancer","name":"Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours)","route":"/cancers/pleuropulmonary-blastoma/"},{"id":"nsclc","kind":"cancer","name":"Non-small-cell lung cancer","route":"/cancers/nsclc/"},{"id":"pleuropulmonary-blastoma-dicer1","kind":"cancer","name":"Pleuropulmonary blastoma (types I, Ir, II and III)","route":"/cancers/pleuropulmonary-blastoma-dicer1/"},{"id":"pulmonary-sarcomatoid-carcinoma","kind":"cancer","name":"Sarcomatoid carcinoma of the lung","route":"/cancers/pulmonary-sarcomatoid-carcinoma/"}],"term":[{"id":"lobectomy","kind":"term","name":"Lobectomy","route":"/terms/lobectomy/"}]}}