# Pulmonary blastoma (adult)

Source: https://onco.cc/cancers/pulmonary-blastoma/  
OnCo record `pulmonary-blastoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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).

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).

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: Classic biphasic pulmonary blastoma; Biphasic pulmonary blastoma; Adult pulmonary blastoma; Well-differentiated fetal adenocarcinoma (the monophasic epithelial form, now classed as fetal adenocarcinoma)
- Tags: subtype-page; wave4; lung
- 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)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/pulmonary-blastoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/pulmonary-blastoma/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/pulmonary-blastoma/#what-it-is [3 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/pulmonary-blastoma/#finding-it [3 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/pulmonary-blastoma/#treating-it [1 setting]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/pulmonary-blastoma/#evidence
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/pulmonary-blastoma/#science
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/pulmonary-blastoma/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/pulmonary-blastoma/#living-with-it [7 questions]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/pulmonary-blastoma/coming/
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/pulmonary-blastoma/data/ [1 connected records]

## Standard of care

- All stages: Surgical excision; adjuvant chemotherapy and radiotherapy of unproven benefit; no trial exists. ([Sarcomatoid carcinoma of the lung](https://onco.cc/cancers/pulmonary-sarcomatoid-carcinoma/), [Lobectomy](https://onco.cc/terms/lobectomy/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Pulmonary_blastoma
- Nicholson 2022, JTO: the 2021 WHO classification of lung tumours: https://doi.org/10.1016/j.jtho.2021.11.003
- Lung Cancer 2011: classic biphasic pulmonary blastoma, case report and review: https://doi.org/10.1016/j.lungcan.2011.03.018
- European Respiratory Journal 2016: somatic DICER1 mutations in adult-onset pulmonary blastoma: https://doi.org/10.1183/13993003.00172-2016

## Connected records

- cancers: [Adenocarcinoma of the lung](https://onco.cc/cancers/lung-adenocarcinoma/), [Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours)](https://onco.cc/cancers/pleuropulmonary-blastoma/), [Non-small-cell lung cancer](https://onco.cc/cancers/nsclc/), [Pleuropulmonary blastoma (types I, Ir, II and III)](https://onco.cc/cancers/pleuropulmonary-blastoma-dicer1/), [Sarcomatoid carcinoma of the lung](https://onco.cc/cancers/pulmonary-sarcomatoid-carcinoma/)
- terms: [Lobectomy](https://onco.cc/terms/lobectomy/)

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JSON: https://onco.cc/api/v1/entities/pulmonary-blastoma.json