Table of Contents
Definition / general | Case reports | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Molecular / cytogenetics descriptionCite this page: Pulmonary blastoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lungtumorpulmonaryblastoma.html. Accessed April 25th, 2018.
Definition / general
- 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
- 16 year old boy with biphasic tumor (Arch Pathol Lab Med 2002;126:875)
- 16 year old girl (Case Rep Med 2012;2012:471613)
Gross description
- Peripheral, solitary, well circumscribed, large
Microscopic (histologic) description
- 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 Wilms' 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
Positive stains
- PAS (glycogen in epithelial cells)
Molecular / cytogenetics description
- CTNNB1 mutation (J Clin Pathol 2011;64:349)