Cardiovascular and Respiratory Systems

  • Bahig M. ShehataEmail author
  • Nasim Khoshnam
  • Aliya N. Husain
Part of the Molecular Pathology Library book series (MPLB)


Primary tumors of the lung and heart are very rare in children as compared to metastatic disease which, although uncommon, is still seen more often. Thus, three entities are discussed here, pleuropulmonary blastoma, inflammatory myofibroblastic tumor, and histiocytoid cardiomyopathy, in each of which there has been recent progress in understanding their molecular pathogenesis. Discussion will also focus on the pathologic and molecular features of cardiac fibroma and NUT midline carcinoma.


Pleuropulmonary blastoma PPB DICER1 DICER1-PPB familial tumor predisposition syndrome Inflammatory myofibroblastic tumor Histiocytoid cardiomyopathy NDUFB1 Cardiac fibroma NUT midline carcinoma NUT BRD4 


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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Bahig M. Shehata
    • 1
    Email author
  • Nasim Khoshnam
    • 1
  • Aliya N. Husain
    • 2
  1. 1.Georgia Institute of Technology and Emory University School of MedicineAtlantaUSA
  2. 2.Department of Pathology, University of ChicagoChicagoUSA

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