Mucinous carcinoma of the duodenum associated with hereditary nonpolyposis colorectal cancer: Report of a case

Mucinous carcinoma of the duodenum associated with hereditary nonpolyposis colorectal cancer: Report of a case
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DOI:
10.1007/s00595-006-3321-7
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发表时间:
2006-12-01
期刊:
影响因子:
2.5
通讯作者:
Mochizuki, Hidetaka
Mochizuki, Hidetaka
中科院分区:
医学4区
文献类型:
--
作者:
Yagyu, Toshihiko;Aihara, Tsukasa;Mochizuki, Hidetaka

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我们在此报告一个罕见的病例,原发性十二指肠黏液癌合并遗传性非息肉病性大肠癌。一名50岁的男子,已知有HNPCC的基础上,阿姆斯特丹标准,我是因为十二指肠肿瘤的存在而入院。十二指肠镜检查显示在十二指肠第二部分的后壁有一溃疡性肿瘤,并经活组织检查证实为恶性肿瘤。他接受了保留幽门的胰管切除术和区域淋巴结清扫术。组织学诊断为十二指肠粘液癌伴淋巴结转移。高频微卫星不稳定性(MSI-H)被确定在结肠和十二指肠标本的基础上的微卫星检测。还鉴定了hMSH 2基因的种系突变。尽管结肠外恶性肿瘤与HNPCC相关,但十二指肠癌仍然非常罕见,在过去20年中仅报告了两例病例。因此,目前的情况下,只有第三个这样的情况下,病人在本文中描述与简要回顾的文献。
We herein report a rare case of primary mucinous carcinoma of the duodenum associated with hereditary nonpolyposis colorectal cancer (HNPCC). A 50-year-old man known to have HNPCC based on the Amsterdam criteria I was admitted because of the presence of a duodenal tumor. Duodenoscopy revealed an ulcerated tumor in the posterior wall of the second portion of the duodenum and the malignancy was confirmed by a biopsy. He underwent a pylorus-preserving pancreaticoduodenectomy with a regional lymph node dissection. The histological diagnosis was mucinous carcinoma of the duodenum with lymph node metastasis. High-frequency microsatellite instability (MSI-H) was identified in both the colon and a duodenal specimen based on a microsatellite assay. A germline mutation in the hMSH2 gene was also identified. Even though extracolonic malignancies are associated with HNPCC, duodenal cancer is nevertheless very rare, and only two cases have been reported over the past 20 years. The present case is therefore only the third such case and the patient is herein described with a brief review of the literature.