Hamartomatous polyposis syndrome associated malignancies: Risk, pathogenesis and endoscopic surveillance,Journal of Digestive Diseases

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Hamartomatous polyposis syndrome associated malignancies: Risk, pathogenesis and endoscopic surveillance
Journal of Digestive Diseases ( IF 2.3 ) Pub Date : 2021-06-18 , DOI: 10.1111/1751-2980.13029
Shuang Liu ₁ , Ye Ma ₁ , Wen You ₁ , Ji Li ₁ , Jing Nan Li ₁ , Jia Ming Qian ₁

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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, Peutzâ€“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.

中文翻译：

错构瘤性息肉病综合征相关恶性肿瘤：风险、发病机制和内窥镜监测

错构瘤息肉病综合征 (HPS) 是一组异质性疾病，其特征是胃肠道 (GI) 道内的弥漫性错构瘤息肉以及胃肠道外表现。经典的 HPS 包括幼年性息肉病综合征、Peutz-Jeghers 综合征、PTEN错构瘤综合征和遗传性混合性息肉病综合征。HPS 患者比一般人群有更高的胃肠道和胃肠道外恶性肿瘤的风险，尽管其潜在机制尚不清楚，并且与经典腺癌和结肠炎相关恶性肿瘤的致癌作用明显不同。在这篇综述中，我们旨在阐明 HPS 相关胃肠道恶性肿瘤的风险、可能的机制和内镜监测。

更新日期：2021-08-17

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