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Peutz-Jeghers Syndrome

  • Reference work entry
  • First Online:
Pathology of the Gastrointestinal Tract

Part of the book series: Encyclopedia of Pathology ((EP))

  • 174 Accesses

Synonyms

Periorificial lentiginosis; Polyps and spots syndrome

Definition

Peutz-Jeghers polyposis is a hereditary cancer syndrome which belongs to hamartomatous polyposis syndromes. It is characterized by mucocutaneous melanotic hyperpigmentation and hamartomatous polyps in gastrointestinal tract with an increased risk of intestinal and also extraintestinal malignancies.

Hamartoma defines an overgrowth of normal tissues in their own native localisations. Hamartomatous polyps in gastrointestinal tract can be seen sporadically, but multiple polyps can occur as a component of genetically inherited or acquired hamartomatous polyposis syndromes. Hamartomatous polyposis syndromes include: (1) Juvenile polyposis, (2) Peutz-Jeghers polyposis (PJP), (3) Cowden syndrome and Bannayan-Ruvalcaba-Riley syndrome (these two are also called as “PTEN hamartoma tumor syndrome” because of the same affected gene locus), (4) Cronkhite-Canada syndrome (the latter is different from the former three because of...

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References and Further Reading

  • Cauchin, E., Touchefeu, Y., & Matysiak-Budnik, T. (2015). Hamartomatous tumors in gastrointestinal tract. Gastrointestinal Tumors, 2, 65–74.

    Article  CAS  Google Scholar 

  • Hornick, J. L., & Odze, R. D. (2015). Polyps of the large intestine. In R. D. Odze & J. R. Goldblum (Eds.), Surgical pathology of the GI tract, liver, biliary tract and pancreas (pp. 607–655). Philadelphia: Elsevier.

    Google Scholar 

  • Jung, I., Gurzu, S., & Turdean, G. S. (2015). Current status of familial gastrointestinal polyposis syndromes. World Journal of Gastrointestinal Oncology, 7, 347–355.

    Article  Google Scholar 

  • Offerhaus, G. J. A., Billaud, M., & Gruber, S. B. (2010). Peutz-Jeghers syndrome. In F. T. Bosman, F. Carneiro, R. H. Hruban, & N. D. Theise (Eds.), WHO classification of tumours of the digestive system (pp. 168–170). Lyon: IARC.

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  • Shaco-Levy, R., Jasperson, K. W., Martin, K., Samadder, N. J., Burt, R. W., Ying, J., & Bronner, M. P. (2016). Morphologic characterization of hamartomatous gastrointestinal polyps in Cowden syndrome, Peutz-Jeghers syndrome and juvenile polyposis syndrome. Human Pathology, 49, 39–48.

    Article  Google Scholar 

  • Turner, J. R. (2015). The gastrointestinal tract. In V. Kumar, A. K. Abbas, & J. C. Aster (Eds.), Pathologic basis of disease (pp. 750–817). Philadelphia: Elsevier.

    Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Pathology, Gulhane Military Medical Academy, Etlik, Ankara, Turkey

    Armagan Gunal

Authors
  1. Armagan Gunal
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Corresponding author

Correspondence to Armagan Gunal .

Editor information

Editors and Affiliations

  1. Faculty of Medicine of Porto University, Centro Hospitalar São João and Ipatimup/i3S, Porto, Portugal

    Fátima Carneiro

  2. Serviço de Anatomia Patológica, Instituto Português de Oncologia de Lisboa de Francisco Gentil, Lisbon, Portugal

    Paula Chaves

  3. Faculdade de Ciências da Saúde, Universidade da Beira Interior, Covilhã, Portugal

    Paula Chaves

  4. Department of Pathology, Ankara University Medical School, Sihhiye, Ankara, Turkey

    Arzu Ensari

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Gunal, A. (2017). Peutz-Jeghers Syndrome. In: Carneiro, F., Chaves, P., Ensari, A. (eds) Pathology of the Gastrointestinal Tract. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-40560-5_1667

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  • DOI: https://doi.org/10.1007/978-3-319-40560-5_1667

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-40559-9

  • Online ISBN: 978-3-319-40560-5

  • eBook Packages: MedicineReference Module Medicine

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