Lung

Sarcomatoid carcinoma

Pulmonary blastoma


Editorial Board Member: Jefree J. Schulte, M.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Hongxing
Ihab Lamzabi, M.D.

Last author update: 4 November 2022
Last staff update: 4 November 2022

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PubMed Search: Pulmonary blastoma

Hongxing
Ihab Lamzabi, M.D.
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Cite this page: Gui H, Lamzabi I. Pulmonary blastoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lungtumorpulmonaryblastoma.html. Accessed December 7th, 2022.
Definition / general
  • Rare lung malignancy, 0.25 - 1% of lung cancers
  • Biphasic components with admixed epithelium and primitive stroma
  • Highly aggressive type of sarcomatoid carcinomas (BMC Res Notes 2014;7:294)
Essential features
  • Peripheral solitary large mass, well circumscribed, unencapsulated
  • Well differentiated glandular component and blastematous stroma
Terminology
  • Separated from fetal adenocarcinoma (epithelium only) and pleuropulmonary blastoma (mesenchymal only)
ICD coding
  • ICD-O: 8972/3 - pulmonary blastoma
Epidemiology
Sites
Pathophysiology
Etiology
Clinical features
Diagnosis
Radiology description
Radiology images

Images hosted on other servers:
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Mass near hilum

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Compression of vena cava

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Uptake within mass

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Right mass

Prognostic factors
Case reports
Treatment
Gross description
  • Mostly peripheral, large, well circumscribed, unencapsulated mass, average 9.1 cm (Lung Cancer 2011;73:127)
  • Lobulated with cystic, necrotic and hemorrhagic degeneration
Gross images

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Solid mass

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Hemorrhagic mass

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Hongxing Gui, M.D., Ph.D. and University of Michigan Virtual Slide Box
Epithelial and mesenchymal components Epithelial and mesenchymal components

Epithelial and mesenchymal components

Glandular epithelium and blastematous stroma Glandular epithelium and blastematous stroma

Glandular epithelium and blastematous stroma


TTF1

TTF1

Vimentin

Vimentin

Beta catenin

Beta catenin

Positive stains
Negative stains
Electron microscopy description
Molecular / cytogenetics description
Sample pathology report
  • Lung, right lower lobe, lobectomy:
    • Pulmonary blastoma, 9 cm, margins negative for tumor (see comment)
    • Comment: H&E sections demonstrate an admixture of malignant epithelial and mesenchymal components. Immunostains performed with adequate controls reveal that the glandular cells are strongly positive for pancytokeratin and TTF1. The stromal cells are vimentin positive. Beta catenin reveals nuclear staining in both epithelial and stromal components. The findings are consistent with the diagnosis of pulmonary blastoma, a subgroup of sarcomatoid carcinoma.
Differential diagnosis
  • Fetal type adenocarcinoma:
    • Epithelial component only, no blastematous stroma
  • Pleuropulmonary blastoma:
    • Pediatric tumor, most diagnosed before 4 years of age
    • No epithelial component; admixture of blastematous and sarcomatoid components
    • Epithelium (if seen) is benign entrapped respiratory type
    • 40% with DICER1 mutation
  • Biphasic synovial sarcoma:
  • Carcinosarcoma:
    • Non-small cell carcinoma (squamous cell carcinoma or adenocarcinoma) and heterologous sarcomatous component
    • Older age
    • Absence of morules and no nuclear staining of beta catenin
Gene mutations commonly seen in different tumors
CTNNB1 mutation P53 mutation DICER1 mutation
 Fetal adenocarcinoma   +   -   - 
 Pulmonary blastoma   +   +   Rare 
 Carcinosarcoma   -   +   - 
 Pleuropulmonary blastoma   -   +   + 
Note: +, present; -, absent
Board review style question #1
    Which of the following is true for pulmonary blastoma?

  1. Associated with smoking
  2. Low to moderate grade tumor
  3. Lung counterpart of Wilms tumor
  4. Most common in young patients < 15 years old
  5. Mostly in a central location
Board review style answer #1
A. Smoking is a risk factor of pulmonary blastoma

Comment Here

Reference: Pulmonary blastoma
Board review style question #2
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    What distinguishes pulmonary blastoma, shown here, from primary lung carcinosarcoma?

  1. Fewer mitotic figures
  2. Less common for metastasis
  3. Less cytologic atypia
  4. Less necrosis
  5. Nuclear immunostaining of beta catenin
Board review style answer #2
E. Pulmonary blastoma is positive for beta catenin, which is negative in lung carcinosarcoma

Comment Here

Reference: Pulmonary blastoma
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