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

机译:小肠套叠并发乙状结肠息肉伴恶变的Peutz-Jeghers综合征

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摘要

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, GeneReviews(R), National Center of Biotechnology Information.). 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. World J Gastroenterol 2009;>15:5397–408.). 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. Oncol Lett 2013;>5:239–41.). We describe a case of a patient with PJS presenting with symptomatic jejunal intussusception and an incidentally found malignant hamartoma in the sigmoid colon.
机译:Peutz-Jeghers综合征(PJS)是常染色体显性疾病,其特征是胃肠道息肉病,皮肤粘膜色素沉着和癌症易感性之间的联系McGarrity,Amos,Baker(Peutz-Jeghers综合征,GeneReviews(R),国家生物技术信息中心)。肠套叠和恶性息肉并不是PJS的罕见并发症,终生肠套叠风险为48%,到65岁时患任何癌症的可能性为37%Kopacova,Tacheci,Rejchrt,Bures(Peutz-Jeghers综合征:诊断和治疗) World J Gastroenterol 2009; > 15 :5397–408。)。迄今为止,极少有PJS同步性恶性息肉引起肠套叠致恶性息肉的病例报道(蔡,田,周,何,胡,邓(空肠套叠和Peutz-Jeghers综合征具有不同类型恶性转化的息肉: Oncol Lett 2013; > 5 :239–41。)。我们描述了一例伴有症状性空肠肠套叠并在乙状结肠中偶然发现恶性错构瘤的PJS患者。

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