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Hamartomatous polyposis syndrome associated malignancies: Risk, pathogenesis and endoscopic surveillance
Author(s) -
Liu Shuang,
Ma Ye,
You Wen,
Li Ji,
Li Jing Nan,
Qian Jia Ming
Publication year - 2021
Publication title -
journal of digestive diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.684
H-Index - 51
eISSN - 1751-2980
pISSN - 1751-2972
DOI - 10.1111/1751-2980.13029
Subject(s) - medicine , peutz–jeghers syndrome , malignancy , pten , gastroenterology , hamartoma , pathology , pathogenesis , dermatology , apoptosis , biochemistry , chemistry , pi3k/akt/mtor pathway
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.