胃息肉病综合征

Kenrry Chiu, Lik Hang Lee, W. Xiong
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摘要

胃息肉多为基底腺息肉和增生性息肉。腺瘤、炎性肌瘤息肉和神经内分泌肿瘤是其他类型的胃息肉,发生频率较低。很少,胃息肉可能与综合征相关,包括遗传性癌症综合征。其中一些综合征还与胃癌风险增加有关。我们提出一个病例50岁的妇女与已知的考登综合征谁提出了多个胃错构瘤息肉。然后我们回顾与胃息肉相关的各种综合征的临床、内窥镜和病理特征。基底腺息肉显性综合征包括家族性腺瘤性息肉病、胃腺癌及近端胃息肉病、mutyh相关息肉病,均为遗传性癌症综合征。胃错构瘤息肉见于青少年息肉综合征、Peutz-Jeghers综合征、PTEN错构瘤肿瘤综合征(包括Cowden综合征)和cronkite - canada综合征。综合征型胃息肉患者,如家族性腺瘤性息肉病患者,可进行活检。在其他情况下,胃息肉的病理评估可能提示或有助于潜在综合征的诊断工作,并结合其他临床和内镜检查结果。
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Gastric Polyposis Syndromes
Most gastric polyps are fundic gland polyps and hyperplastic polyps. Adenomas, inflammatory fibroid polyps, and neuroendocrine tumors are other types of gastric polyps that occur less frequently. Rarely, a gastric polyp may be associated with a syndrome, including hereditary cancer syndromes. Some of these syndromes are also associated with an increased risk of gastric cancer. We present a case of a 50-year-old woman with known Cowden syndrome who presents with multiple gastric hamartomatous polyps. We then review the clinical, endoscopic, and pathologic features of various syndromes that are associated with gastric polyps. Fundic gland polyp–predominant syndromes include familial adenomatous polyposis, gastric adenocarcinoma and proximal polyposis of the stomach, and MUTYH-associated polyposis, all of which are hereditary cancer syndromes. Gastric hamartomatous polyps are found in patients with juvenile polyposis syndrome, Peutz-Jeghers syndrome, PTEN hamartoma tumor syndrome including Cowden syndrome, and Cronkhite-Canada syndrome. Syndromic gastric polyps may be biopsied in patients with a previously established diagnosis of the underlying syndrome, such as surveillance endoscopies in familial adenomatous polyposis. On other occasions, the pathologic assessment of gastric polyps may prompt or contribute to a diagnostic workup of an underlying syndrome in conjunction with other clinical and endoscopic findings.
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期刊介绍: Each issue of Pathology Case Reviews examines one vital theme in the field with peer-reviewed, clinically oriented case reports that focus on diagnosis, specimen handling and reports generation. Each theme-oriented issue covers both histopathologic and cytopathologic cases, offering a comprehensive perspective that includes editorials and review articles of the newest developments in the field, differential diagnosis hints, applications of new technologies, reviews of current issues and techniques and an emphasis on new approaches.
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