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Not Only FAP Other Rare Polyposis Syndromes

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Intestinal Polyps and Polyposis

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Abstract

The rare gastrointestinal polyposis syndromes are a group of disorders characterized by the presence of multiple polyps in the gastrointestinal tract, associated in some cases with extraintestinal manifestations. These syndromes can be inherited or non-inherited. The first group includes, on the basis of polyp histopathology, adenomatous polyposis (familial adenomatous polyposis (FAP) variants like Gardner’s syndrome and Turcot’s syndrome) and hamartomatous polyposis (Juvenile Polyposis syndrome, Peutz-Jeghers syndrome, Cowden’s disease, and Bannayan-Ryley-Ruvalcaba syndrome), while the second group includes Cronkhite-Canada syndrome and Hyperplastic polyposis. The molecular genetics have not been well defined for all the syndromes. However, these conditions present variable risk of gastrointestinal and extraintestinal invasive malignancy. Therefore management and surveillance programs are different for each syndrome.

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Authors and Affiliations

  1. “Magrassi-Lanzara” Department of Clinical and Experimental Medicine, 2nd University of Naples, Naples, Italy

    Gabriele Riegler MD (Gastroenterologist, Unit of Gastroenterology), Annalisa de Leone MD (Gastroenterologist, Unit of Gastroenterology) & Ilaria Esposito MD (Gastroenterologist, Unit of Gastroenterology)

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  1. Gabriele Riegler MD
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  2. Annalisa de Leone MD
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  3. Ilaria Esposito MD
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Editors and Affiliations

  1. Department of Surgery and Gastroenterology, University of Verona, Verona, Italy

    Gian Gaetano Delaini

  2. Department of Surgery, Masaryk Memorial Cancer Institute, Brno, Czech Republic

    Tomáš Skřička

  3. Department of Surgery and Gastroenterology, University of Verona, Verona, Italy

    Gianluca Colucci

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Riegler, G., de Leone, A., Esposito, I. (2009). Not Only FAP Other Rare Polyposis Syndromes. In: Delaini, G.G., Skřička, T., Colucci, G. (eds) Intestinal Polyps and Polyposis. Springer, Milano. https://doi.org/10.1007/978-88-470-1124-3_5

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