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黑斑息肉综合征并发小肠套叠及乙状结肠息肉恶变

Small bowel intussusception and concurrent sigmoid polyp with malignant transformation in Peutz-Jeghers syndrome.

作者信息

Algarni Maidah, Raml Enas, Trabulsi Nora, Nassif Mohammed

机构信息

Department of General Surgery, King Abdulaziz University, Saudi Arabia.

出版信息

J Surg Case Rep. 2019 Jan 31;2019(1):rjz004. doi: 10.1093/jscr/rjz004. eCollection 2019 Jan.

Abstract

Peutz-Jeghers syndrome (PJS) is an autosomal dominant condition characterized by the association of gastrointestinal polyposis, mucocutaneous pigmentation and cancer predisposition McGarrity, Amos, Baker (Peutz-Jeghers Syndrome, , .). Intussusception and malignant polyps are not rare complications of PJS, where the lifetime risk of intussusception is 48% and the possibility of developing any cancer by age 65 years is 37% Kopacova, Tacheci, Rejchrt, Bures (Peutz-Jeghers syndrome: diagnostic and therapeutic approach. 2009;:5397-408.). Very few cases of malignant polyps causing intussusception associated with synchronous malignant polyp in PJS have been reported to date Cai, Tian, Zhou, He, Hu, Deng (Jejunal intussusception and polyps with different types of malignant transformation in Peutz-Jeghers syndrome: report of a case. 2013;:239-41.). We describe a case of a patient with PJS presenting with symptomatic jejunal intussusception and an incidentally found malignant hamartoma in the sigmoid colon.

摘要

黑斑息肉综合征(PJS)是一种常染色体显性遗传病,其特征为胃肠道息肉病、黏膜皮肤色素沉着和癌症易感性(麦加里蒂、阿莫斯、贝克,《黑斑息肉综合征》,……)。肠套叠和恶性息肉是PJS并不罕见的并发症,其中肠套叠的终生风险为48%,65岁时发生任何癌症的可能性为37%(科帕乔娃、塔切奇、雷赫尔特、布雷什,《黑斑息肉综合征:诊断和治疗方法》。2009年;:5397 - 408)。迄今为止,仅有极少数关于PJS中恶性息肉导致肠套叠并伴有同步恶性息肉的病例报道(蔡、田、周、何、胡、邓,《黑斑息肉综合征中不同类型恶性转化的空肠套叠和息肉:一例报告》。2013年;:239 - 41)。我们报告一例患有PJS的患者,该患者出现有症状的空肠套叠,并在乙状结肠偶然发现了一个恶性错构瘤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d4f7/6354681/6f307ea5380d/rjz004f01.jpg

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