Lung Neoplasms in Infants and Children

Frances V. White, Archana Shenoy

Research output: Chapter in Book/Report/Conference proceedingChapterpeer-review

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 languageEnglish
Title of host publicationPulmonary Pathology
Subtitle of host publicationA Volume in the Series Foundations in Diagnostic Pathology, Third Edition
PublisherElsevier
Pages83-96
Number of pages14
ISBN (Electronic)9780323935487
ISBN (Print)9780323935715
DOIs
StatePublished - Jan 1 2024

Keywords

  • congenital peribronchial myofibroblastic tumors
  • fetal lung interstitial tumor
  • pediatric lung tumors
  • pleuropulmonary blastoma

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