pleuropulmonary blastoma
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Epidemiology
- rare
Pathology
- pediatric tumor of the lung that arises during fetal lung development
- often part of an inherited cancer syndrome
- tumors contain both epithelial cells & mesenchymal cells
- early in tumorigenesis, cysts form in lung airspaces, & these cysts are lined with benign-appearing epithelium
- mesenchymal cells susceptible to malignant transformation reside within the cyst walls & form a dense layer beneath the epithelial lining
- in a subset of patients, overgrowth of mesenchymal cells produces a sarcoma & a worse prognosis
Genetics
- associated with defects in DICER1