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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.
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).
How 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.
How common: 0.25 to 0.5 percent of malignant lung tumours (Lung Cancer 2011).
Treatment: 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).
Averages across everyone diagnosed, often years ago. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
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, 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
Surgical excision; adjuvant chemotherapy and radiotherapy of unproven benefit; no trial exists.
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Query for this cancer: (TITLE:"Pulmonary blastoma" OR ABSTRACT:"Pulmonary blastoma" OR TITLE:"adult" OR ABSTRACT:"adult" OR TITLE:"Classic biphasic pulmonary blastoma" OR ABSTRACT:"Classic biphasic pulmonary blastoma" OR TITLE:"Biphasic pulmonary blastoma" OR ABSTRACT:"Biphasic pulmonary blastoma" OR TITLE:"Adult pulmonary blastoma" OR ABSTRACT:"Adult pulmonary blastoma" OR TITLE:"Well-differentiated fetal adenocarcinoma the monophasic epithelial form, now classed as fetal adenocarcinoma" OR ABSTRACT:"Well-differentiated fetal adenocarcinoma the monophasic epithelial form, now classed as fetal adenocarcinoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Pulmonary blastoma (adult), not a curated reading list.
No targets or pathways are linked to this cancer yet. Browse the gene hub →
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