Abstract
Primary lung neoplasms in the pediatric age group are rare in comparison to malformations and pulmonary metastases. Pleuropulmonary blastoma (PPB), congenital peribronchial myofibroblastic tumor (CPMT), and fetal lung interstitial tumor (FLIT) are three primary lung tumors that almost exclusively present in children. PPB is the most common malignant primary tumor and the archetypal tumor associated with DICER1 predisposition syndrome. Morphologic features of PPB varies from purely cystic (type I) and solid and cystic (type II) to a solid tumor with sarcomatous and blastemal elements (type III). CPMT and FLIT are rare benign tumors seen in early infancy. CPMT is a mesenchymal myofibroblastic tumor involving the hilum, while FLIT is well-circumscribed mass that morphologically resembles fetal lung in the canalicular phase of development. In the post-transplant setting, Epstein-Barr virus–associated smooth muscle tumor can occur in the lung.
Original language | English |
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Title of host publication | Pulmonary Pathology |
Subtitle of host publication | A Volume in the Series Foundations in Diagnostic Pathology, Third Edition |
Publisher | Elsevier |
Pages | 83-96 |
Number of pages | 14 |
ISBN (Electronic) | 9780323935487 |
ISBN (Print) | 9780323935715 |
DOIs | |
State | Published - Jan 1 2024 |
Keywords
- congenital peribronchial myofibroblastic tumors
- fetal lung interstitial tumor
- pediatric lung tumors
- pleuropulmonary blastoma