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Juvenile Polyposis Syndrome, Lower Gastrointestinal Tract

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

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

  • 205 Accesses

Synonyms

Combined JP/hereditary hemorrhagic telengiectasia (Osler-Weber-Rendu) syndrome; Gastric JP; Generalized juvenile polyposis (JP); JP coli

Definition

JP is an autosomal dominant hereditary syndrome in which the affected individuals characteristically have multiple juvenile polyps in one or more parts of the GI tract: stomach, small bowel, or colorectum. The patients have increased risk of carcinomas of the stomach and small and large bowel. The juvenile polyps in these patients have the potential to transform to carcinoma.

The syndrome occurs in patients with no family history in roughly half of the cases.

It has been proposed that an individual has JP if at least one of the following criteria is met:

  1. 1.

    >3 or 5 juvenile polyps in the colorectum

  2. 2.

    Juvenile polyps present in throughout the GI tract

  3. 3.

    Any number of juvenile polyps in an individual with positive family history

Clinical Features

  • Incidence

    The incidence is about 0.6–1/100,000 in western countries.

    ...

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References and Further Reading

  • Coburn, M. C., Pricolo, V. E., DeLuca, F. G., & Bland, K. I. (1995). Malignant potential in intestinal juvenile polyposis syndromes. Annals of Surgical Oncology, 2, 386–391.

    Article  CAS  Google Scholar 

  • Howe, J. R., Roth, S., Ringold, J. C., Summers, R. W., Järvinen, H. J., Sistonen, P., Tomlinson, I. P., Houlston, R. S., Bevan, S., Mitros, F. A., Stone, E. M., & Aaltonen, L. A. (1998). Mutations in the SMAD4/DPC4 gene in juvenile polyposis. Science, 280, 1086–1088.

    Article  CAS  Google Scholar 

  • Howe, J. R., Bair, J. L., Sayed, M. G., Anderson, M. E., Mitros, F. A., Petersen, G. M., Velculescu, V. E., Traverso, G., & Vogelstein, B. (2001). Germline mutations of the gene encoding bone morphogenetic protein receptor 1A in juvenile polyposis. Nature Genetics, 28, 184–187.

    Article  CAS  Google Scholar 

  • Jansen, M., de Leng, W. W. J., Baas, A. F., Myoshi, H., Mathus-Vliegen, L., Taketo, M. M., Clevers, H., Giardiello, F. M., & Offerhaus, G. J. A. (2006). Mucosal prolapse in the pathogenesis of Peutz-Jeghers polyposis. Gut, 55, 1–5.

    Article  CAS  Google Scholar 

  • Jass, J. R., Williams, C. B., Bussey, H. J., & Morson, B. C. (1988). Juvenile polyposis – A precancerous condition. Histopathology, 13, 619–630.

    Article  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Pathology, Gazi University, Ankara, Turkey

    Özgür Ekinci

Authors
  1. Özgür Ekinci
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Corresponding author

Correspondence to Özgür Ekinci .

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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Ekinci, Ö. (2017). Juvenile Polyposis Syndrome, Lower Gastrointestinal Tract. 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_1504

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  • DOI: https://doi.org/10.1007/978-3-319-40560-5_1504

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  • 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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