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  • Title: Hamartomatous polyposis syndrome associated malignancies: Risk, pathogenesis and endoscopic surveillance.
    Author: Liu S, Ma Y, You W, Li J, Li JN, Qian JM.
    Journal: J Dig Dis; 2021 Aug; 22(8):444-451. PubMed ID: 34145757.
    Abstract:
    Hamartomatous polyposis syndromes (HPS) are a heterogeneous spectrum of diseases that are characterized by diffuse hamartomatous polyps lining the gastrointestinal (GI) tract together with extra-GI manifestations. Classical HPS includes juvenile polyposis syndrome, Peutz-Jeghers syndrome, PTEN hamartoma tumor syndrome and hereditary mixed polyposis syndrome. Patients with HPS have a higher risk of GI and extra-GI malignancies than the general population, although the underlying mechanisms remain unclear and are obviously different from the carcinogenesis of classical adenocarcinoma and colitis-associated malignancy. In this review we aimed to clarify the risks, possible mechanism and endoscopic surveillance of HPS-associated GI malignancies.
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