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Lung tumor

Other malignancies

Pulmonary blastoma

Reviewer: Deepali Jain, M.D. (see Reviewers page)
Revised: 22 January 2013, last major update September 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.

General
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● Rare tumor composed of mixture of immature epithelial and mesenchymal tissue resembling fetal lung tissue
● Also called embryoma
● Usually adults (mean age 43 years), 20% occur in patients < age 20 years
● Metastases common
● 2/3 die within 2 years
● See also pleuropulmonary blastoma - cystic tumor of pleura and lung

Case reports
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● 16 year old boy with biphasic tumor (Arch Pathol Lab Med 2002;126:875)
● 16 year old girl (Case Report Med 2012;2012:471613)

Gross description
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● Peripheral, solitary, well circumscribed, large
● Type I (purely cystic), type II (cystic and solid), type III (purely solid)

Micro description
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● Biphasic tumor in which epithelial and mesenchymal components have a primitive, “fetal-type” appearance
● Well formed tubular glands surrounded by cellular stroma of “embryonal” appearance
● Resembles Wilm’s tumor and fetal lung at 10-16 weeks
● Glandular cells are tall, columnar, often with clear cytoplasm and subnuclear and supranuclear cytoplasmic vacuoles
● Morules with ground-glass nuclei are common
● Stroma may differentiate towards striated muscle, smooth muscle, cartilage
● In infants/children, epithelial component is benign appearing or minimal
● Stroma may be rhabdomyoblastic or chondroid

Micro images
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Biphasic tumor

Positive stains
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● PAS (glycogen in epithelial cells)

Molecular description
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● CTNNB1 mutation (J Clin Pathol 2011;64:349), DICER1 mutation

Differential diagnosis
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● Fetal lung interstitial tumor (FLIT): see Am J Surg Pathol 2010;34:1762

End of Lung tumor > Other malignancies > Pulmonary blastoma

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