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与胃肠道癌相关的黑斑息肉综合征。一家族中两例报告。

Peutz-Jeghers syndrome associated with gastrointestinal carcinoma. Report of two cases in a family.

作者信息

Cochet B, Carrel J, Desbaillets L, Widgren S

出版信息

Gut. 1979 Feb;20(2):169-75. doi: 10.1136/gut.20.2.169.

DOI:10.1136/gut.20.2.169
PMID:428830
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1419453/
Abstract

Patients with the Peutz-Jeghers syndrome carry a slight, though definite, increased risk of gastrointestinal carcinoma. The malignant potentiality of Peutz-Jeghers hamartomatous polyps, generally considered benign, is supported by this report. Two cases of metastasising gastrointestinal carcinoma associated with the Peutz-Jeghers syndrome are described in a 56 year old female and her 29 year 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. These features indicate that hamartomatous polyps may, in some cases, be the precursors of digestive tract carcinomas.

摘要

患有黑斑息肉综合征的患者患胃肠道癌的风险虽小,但确实有所增加。本报告支持了通常被认为是良性的黑斑息肉综合征错构瘤性息肉的恶性潜能。本文描述了一名56岁女性及其29岁儿子患与黑斑息肉综合征相关的转移性胃肠道癌的两个病例。母亲和儿子分别死于十二指肠癌和胃癌,这些癌症均发生于有广泛转移的错构瘤性息肉中。两个病例的错构瘤性息肉内均出现了发育异常区域。这些特征表明,错构瘤性息肉在某些情况下可能是消化道癌的先兆。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/cbe47d2217e0/gut00447-0102-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/6e7e174d6d69/gut00447-0098-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/ed239c6ad03b/gut00447-0099-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/c0bbe2f03a79/gut00447-0099-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/c2c8d1c25dbe/gut00447-0099-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/cfb80d0635f7/gut00447-0100-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/14964202825b/gut00447-0101-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/cbe47d2217e0/gut00447-0102-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/6e7e174d6d69/gut00447-0098-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/ed239c6ad03b/gut00447-0099-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/c0bbe2f03a79/gut00447-0099-b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/c2c8d1c25dbe/gut00447-0099-c.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/cfb80d0635f7/gut00447-0100-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/14964202825b/gut00447-0101-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d441/1419453/cbe47d2217e0/gut00447-0102-a.jpg

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本文引用的文献

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Generalized intestinal polyposis and melanin spots of the oral mucosa, lips and digits; a syndrome of diagnostic significance.全身肠道息肉病以及口腔黏膜、嘴唇和手指的黑色素斑;一种具有诊断意义的综合征。
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Br Med J. 1957 Aug 24;2(5042):433-9. doi: 10.1136/bmj.2.5042.433.
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Intestinal polyposis associated with mucocutaneous melanin pigmentation Peutz-Jeghers syndrome; review of literature and report of six cases with special reference to pathologic findings.
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