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家族性腺瘤性息肉病(FAP)行回肠储袋肛管吻合术后在意外部位发生的癌症。

Cancer in an unexpected site post pouch surgery for familial adenomatous polyposis (FAP).

作者信息

Alwahbi Omar A, Abduljabbar Alaa S, Anwer Lucman A

机构信息

King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia.

Mayoclinic, Rochester, MN, United States.

出版信息

Int J Surg Case Rep. 2018;42:266-268. doi: 10.1016/j.ijscr.2017.12.037. Epub 2017 Dec 28.

Abstract

INTRODUCTION

Familial Adenomatous Polyposis (FAP) is a hereditary condition characterized by multiple colorectal adenomatous polyps. FAP is the most common adenomatous polyposis syndrome. Restorative proctocolectomy is the most commonly performed surgical procedure performed for patients suffering from FAP with different options for anastomosis, namely ileorectal anastomosis (IRA) or ileal pouch anal anastomosis (IPAA). The occurrence of adenomas is a common finding during follow up and surveillance post surgery for these patients. Although there are a few cases of carcinoma that were namely at the anal transitional zone (ATZ), there are only a few cases of ileal pouch related adenocarcinoma reported. This work has been reported in line with the SCARE criteria (Agha et al., 2016) [1].

PRESENTATION OF CASE

We report a case of a 34-year-old man diagnosed with FAP who underwent proctocolectomy with IPAA, and subsequently referred to our center, who, despite appropriate measures and surveillance, developed adenocarcinoma in the ileal pouch.

DISCUSSION

Restorative proctocolectomy for Familial Adenomatous Polyposis (FAP) is the mainstay of treatment. There are different surgical options, each with its own set of advantages and disadvantages. The most favored option is proctocolectomy with ileal pouch anal anastomosis (IPAA) due to because it involves resection of the rectum. Despite these interventions, adenomas and/or carcinomas have been reported on follow up post surgery.

CONCLUSION

Although the risk of developing adenomas or carcinomas in the ileal pouch post proctocolectomy with IPAA is low it should not be neglected as cancer occurrence or recurrence is unpredictable even with appropriate measures.

摘要

引言

家族性腺瘤性息肉病(FAP)是一种遗传性疾病,其特征为多个结直肠腺瘤性息肉。FAP是最常见的腺瘤性息肉病综合征。直肠结肠全切除回肠储袋肛管吻合术是为FAP患者实施的最常见手术,吻合方式有不同选择,即回肠直肠吻合术(IRA)或回肠储袋肛管吻合术(IPAA)。腺瘤的发生是这些患者术后随访和监测期间的常见发现。虽然有少数病例的癌发生在肛管移行区(ATZ),但回肠储袋相关腺癌的报道仅有少数几例。本病例报告符合SCARE标准(Agha等人,2016年)[1]。

病例介绍

我们报告一例34岁男性,诊断为FAP,接受了直肠结肠全切除回肠储袋肛管吻合术,随后转诊至我院,尽管采取了适当措施并进行了监测,但回肠储袋仍发生了腺癌。

讨论

家族性腺瘤性息肉病(FAP)的直肠结肠全切除回肠储袋肛管吻合术是主要治疗方法。有不同的手术选择,每种都有其自身的优缺点。最受青睐的选择是直肠结肠全切除回肠储袋肛管吻合术(IPAA),因为它涉及直肠切除。尽管采取了这些干预措施,但术后随访中仍有腺瘤和/或癌的报道。

结论

尽管直肠结肠全切除回肠储袋肛管吻合术后回肠储袋发生腺瘤或癌的风险较低,但不应忽视,因为即使采取了适当措施,癌症的发生或复发也是不可预测的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4d34/5766745/a25e23d49086/gr1.jpg

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