PEUTZ-JEGHERS SYNDROME ASSOCIATED WITH GASTRO-INTESTINAL CARCINOMA - REPORT OF 2 CASES IN A FAMILY
PEUTZ-JEGHERS SYNDROME ASSOCIATED WITH GASTRO-INTESTINAL CARCINOMA - REPORT OF 2 CASES IN A FAMILY
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DOI：
10.1136/gut.20.2.169
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发表时间：
1979-01-01
期刊：
影响因子：
24.5
通讯作者：
WIDGREN, S
中科院分区：
文献类型：
--
作者：
COCHET, B;CARREL, J;WIDGREN, S
患有Peutz-Jeghers综合征的患者患胃肠癌的风险轻微增加，但确切增加。恶性潜力的Peutz-Jerghers错构瘤性息肉，通常被认为是良性的进行了讨论。两例转移性胃肠癌与Peutz-Jeghers综合征的描述在一个56岁的女性和她的29岁的儿子。母亲和儿子分别死于十二指肠癌和胃癌，这些癌是在错构瘤性息肉中发展的，并伴有广泛的转移。两例病例均显示错构瘤性息肉内的发育不良区域。在某些情况下，错构瘤性息肉可能是消化道癌的前兆。
Patients with the Peutz-Jeghers syndrome carry a slight, though definite, increased risk of gastrointestinal carcinoma. The malignant potential of Peutz-Jerghers hamartomatous polyps, generally considered benign is discussed. Two cases of metastasizing gastrointestinal carcinoma associated with the Peutz-Jeghers syndrome are described in a 56 yr old female and her 29 yr old son. Both mother and son died from duodenal and gastric carcinomas respectively, which developed in hamartomatous polyps with extensive metastases. Both cases also showed dysplastic areas within hamartomatous polyps. Hamartomatous polyps may, in some cases, be the precursors of digestive tract carcinomas.