Skip to main content
SpringerLink
Log in

Pleuropulmonary Blastoma

  • Chapter
  • First Online:
Rare Tumors In Children and Adolescents

Part of the book series: Pediatric Oncology ((PEDIATRICO))

Abstract

Pleuropulmonary blastoma (PPB) is a malignant ­sarcoma of lung and pleura in children under age 6 years. PPB is in the family of dysembryonic developmental tumors of early childhood such as WT (nephroblastoma), embryonal rhabdomyosarcoma (ERMS), neuroblastoma, medulloblastoma, retinoblastoma (Rb), and others. Histopathologically, PPB recapitulates primitive pleuropulmonary ­mesenchyme from which it is thought to arise (Dehner 1994; Manivel et al. 1988). Because of its rarity, PPB was recognized as a diagnostic entity only in the 1980s (Dehner 1994; Manivel et al. 1987, 1988). In contrast to ∼500 Wilms tumors (WT) occurring each year in the United States, 25–50 PPBs are estimated to occur annually.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 229.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Bahubeshi A, Bal N, Rio Frio T et al (2010) Germline DICER1 mutations and familial cystic nephroma. J Med Genet 47:863–866

    Article  PubMed  Google Scholar 

  • Belcher E, Lawson MH, Nicholson AG, Davison A, Goldstraw P (2007) Congenital cystic adenomatoid malformation presenting as in-flight systemic air embolisation. Eur Respir J 30:801–804

    Article  PubMed  CAS  Google Scholar 

  • Cummings NM, Desai S, Thway K et al (2010) Cystic primary pulmonary synovial sarcoma presenting as recurrent pneumothorax: report of 4 cases. Am J Surg Pathol 34:1176–1179

    Article  PubMed  Google Scholar 

  • Dehner LP (1994) Pleuropulmonary blastoma is the pulmonary blastoma of childhood. Semin Diagn Pathol 11:144–151

    PubMed  CAS  Google Scholar 

  • Dimaras H, Khetan V, Halliday W et al (2008) Loss of RB1 induces non-proliferative retinoma: increasing genomic instability correlates with progression to retinoblastoma. Hum Mol Genet 17:1363–1372

    Article  PubMed  CAS  Google Scholar 

  • Dishop MK, McKay EM, Kreiger PA et al (2010) Fetal Lung Interstitial Tumor (FLIT): a proposed newly recognized lung tumor of infancy to be differentiated from cystic pleuropulmonary blastoma and other developmental pulmonary lesions. Am J Surg Pathol 34:1762–1772

    Article  PubMed  Google Scholar 

  • Hill DA, Jarzembowski JA, Priest JR, Williams G, Schoettler P, Dehner LP (2008) Type I pleuropulmonary blastoma: pathology and biology study of 51 cases from the international pleuropulmonary blastoma registry. Am J Surg Pathol 32:282–295

    Article  PubMed  Google Scholar 

  • Hill DA, Ivanovich J, Priest JR et al (2009) DICER1 mutations in familial pleuropulmonary blastoma. Science 325:965

    Article  PubMed  CAS  Google Scholar 

  • Hill DA, Wang JD, Schoettler P et al (2010) Germline DICER1 mutations are common in both hereditary and presumed sporadic pleuropulmonary blastoma [abstract]. Lab Invest 90:311

    Google Scholar 

  • Indolfi P, Bisogno G, Casale F et al (2007) Prognostic factors in pleuro-pulmonary blastoma. Pediatr Blood Cancer 48:318–323

    Article  PubMed  Google Scholar 

  • Kirsch S, Leuschner I, Int-Veen C et al (2005) Sixteen children with pleuropulmonary blastoma – results of the German Cooperative Soft Tissue Sarcoma Group [abstract]. Sarcoma 9:88

    Google Scholar 

  • Manivel C, Wick M, Dehner L (1987) Thoracopulmonary blastoma – reassessment of an embryonal neoplasm. Lab Invest 56:48

    Google Scholar 

  • Manivel JC, Priest JR, Watterson J et al (1988) Pleuropulmonary blastoma. The so-called pulmonary blastoma of childhood. Cancer 62:1516–1526

    Article  PubMed  CAS  Google Scholar 

  • Miniati DN, Chintagumpala M, Langston C et al (2006) Prenatal presentation and outcome of children with pleuropulmonary blastoma. J Pediatr Surg 41:66–71

    Article  PubMed  Google Scholar 

  • Plon SE, Malkin D (2005) Childhood cancer and heredity. In: Pizzo PA (ed) Principles and practice of pediatric oncology, vol 5. Lippincott Williams & Wilkins, Philadelphia, pp 14–37

    Google Scholar 

  • Priest JR, Watterson J, Strong L et al (1996) Pleuropulmonary blastoma: a marker for familial disease. J Pediatr 128:220–224

    Article  PubMed  CAS  Google Scholar 

  • Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP (1997) Pleuropulmonary blastoma: a clinicopathologic study of 50 cases. Cancer 80:147–161

    Article  PubMed  CAS  Google Scholar 

  • Priest JR, Hill DA, Williams GM et al (2006) Type I pleuropulmonary blastoma: a report from the International Pleuropulmonary Blastoma Registry. J Clin Oncol 24:4492–4498

    Article  PubMed  Google Scholar 

  • Priest JR, Magnuson J, Williams GM et al (2007) Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma. Pediatr Blood Cancer 49:266–273

    Article  PubMed  Google Scholar 

  • Priest JR, Williams GM, Hill DA, Dehner LP, Jaffe A (2009) Pulmonary cysts in early childhood and the risk of malignancy. Pediatr Pulmonol 44:14–30

    Article  PubMed  Google Scholar 

  • Priest JR, Andic D, Arbuckle S, Gonzalez-Gomez I, Hill DA, Williams G (2011a) Great vessel/cardiac extension and tumor embolism in pleuropulmonary blastoma: a report from the International Pleuropulmonary Blastoma Registry. Pediatr Blood Cancer 56(4):604–609

    Article  PubMed  Google Scholar 

  • Priest JR, Williams GM, Manera R et al (2011b) Ciliary body medulloepithelioma: four cases associated with pleuropulmonary blastoma–a report from the International Pleuropulmonary Blastoma Registry. Br J Ophthalmol 95(7):1001–1005

    Article  PubMed  Google Scholar 

  • Radulescu VC, Gerrard M, Moertel C et al (2008) Treatment of recurrent clear cell sarcoma of the kidney with brain metastasis. Pediatr Blood Cancer 50:246–249

    Article  PubMed  Google Scholar 

  • Rio Frio T, Bahubeshi A, Kanellopoulou C et al (2011) DICER1 mutations in familial multinodular goiter with and without ovarian sertoli-leydig cell tumors. JAMA 305:68–77

    Article  PubMed  CAS  Google Scholar 

  • Scott RH, Stiller CA, Walker L, Rahman N (2006) Syndromes and constitutional chromosomal abnormalities associated with Wilms tumour. J Med Genet 43:705–715

    Article  PubMed  CAS  Google Scholar 

  • Seitz H (2010) siRNAs: the hidden face of the small RNA world. Curr Biol 20:R108–R110

    Article  PubMed  CAS  Google Scholar 

  • Slade I, Bacchelli C, Davies H et al (2011) DICER1 syndrome – clarifying the diagnosis, clinical features and management implications of a pleiotropic tumor predisposition syndrome. J Med Genet 48(4):273–278

    Article  PubMed  CAS  Google Scholar 

  • Stocker JT (2002) Congenital pulmonary airway malformation – a new name for and an expanded classification of congenital cystic adenomatoid malformation of the lung. Histopathology 41:424–431

    Article  Google Scholar 

Download references

Acknowledgments

The International PPB Registry has been supported by the Pine Tree Apple Tennis Classic since 1987 and by the Theodora H. Lang Charitable Trust and Children’s Hospitals and Clinics of Minnesota foundation. Appreciation is expressed to Gretchen Williams and Marsha Finkelstein for the help in preparation of this material.

Author information

Authors and Affiliations

  1. International Pleuropulmonary Blastoma Registry, Children’s Hospitals and Clinics of Minnesota, 2545 Chicago Ave. S., Suite 412, 55404, Minneapolis, MN, USA

    John R. Priest

Authors
  1. John R. Priest
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to John R. Priest .

Editor information

Editors and Affiliations

  1. Klinik für Kinder- und Jugendmedizin, Klinikum Dortmund gGmbH, Beurhausstr. 40, Dortmund, 44137, Germany

    Dominik T. Schneider

  2. Klinik für Kinder und Jugendliche, Universitätsklinikum Erlangen-Nürnberg, Loschgestr. 15, Erlangen, 91054, Germany

    Ines B. Brecht

  3. at Egleston, Dept. Pediatric Hematology/Oncology, Children's Healthcare of Atlanta, Uppergate Drive NE., 2015, Atlanta, 30322, Georgia, USA

    Thomas A. Olson

  4. Unità di Oncologia Pediatrica, Istituto Nazionale Tumori, Via G. Venezian 1, Milano, 20133, Italy

    Andrea Ferrari

Rights and permissions

Reprints and permissions

Copyright information

© 2012 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Priest, J.R. (2012). Pleuropulmonary Blastoma. In: Schneider, D., Brecht, I., Olson, T., Ferrari, A. (eds) Rare Tumors In Children and Adolescents. Pediatric Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-04197-6_25

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-04197-6_25

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-04196-9

  • Online ISBN: 978-3-642-04197-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation