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肛管腺癌合并肛周佩吉特病:一例报告。

Anal adenocarcinoma with perianal Paget's disease: A case report.

作者信息

Wu Sheng-Wei, Rong Yao, Chen Gui-Jin, Cao Xu-Song, Xie Zheng-Yong, Wu Bei, Huang Hao-Chun, Wang Zhi-Wei, Wu Xiao-Xiang

机构信息

Department of General Surgery, General Hospital of Southern Theater Command, Guangzhou 510000, Guangdong Province, China.

出版信息

World J Gastrointest Surg. 2025 Aug 27;17(8):108963. doi: 10.4240/wjgs.v17.i8.108963.

DOI:10.4240/wjgs.v17.i8.108963
PMID:40949398
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12427061/
Abstract

BACKGROUND

Anal canal adenocarcinoma with secondary perianal Paget's disease (PPD) is clinically rare and exhibits atypical symptoms, often misdiagnosed as benign conditions such as hemorrhoids or perianal eczema, leading to delayed treatment. Further summarization of diagnostic and therapeutic key points, as well as reasons for misdiagnosis, is necessary to enhance clinical awareness.

CASE SUMMARY

A retrospective analysis was conducted on a 72-year-old female patient with a 2-year history of perianal moisture, pruritus, and hematochezia, who was repeatedly misdiagnosed with mixed hemorrhoids. The diagnosis of anal canal adenocarcinoma with secondary PPD was confirmed through colonoscopy, perianal skin biopsy, and immunohistochemical staining (CK7, CK20, ). The patient underwent 3D laparoscopic-assisted abdominoperineal resection (APR) with extended perianal skin excision, achieving negative margins and primary wound healing. No recurrence or metastasis was observed during the 12-month follow-up.

CONCLUSION

Secondary PPD has a high misdiagnosis rate. Clinicians should maintain a high index of suspicion for elderly patients with prolonged perianal symptoms (, pruritus, hematochezia > 6 months) and promptly perform colonoscopy and immunohistochemical testing for definitive diagnosis. APR combined with extended perianal resection is an effective treatment, and standardized long-term follow-up is crucial for prognosis.

摘要

背景

肛管腺癌伴继发性肛周佩吉特病(PPD)临床罕见,症状不典型,常被误诊为痔疮或肛周湿疹等良性疾病,导致治疗延误。进一步总结诊断和治疗要点以及误诊原因,对于提高临床认识很有必要。

病例摘要

对一名72岁女性患者进行回顾性分析,该患者有2年肛周潮湿、瘙痒和便血病史,多次被误诊为混合痔。通过结肠镜检查、肛周皮肤活检及免疫组化染色(CK7、CK20)确诊为肛管腺癌伴继发性PPD。患者接受了3D腹腔镜辅助腹会阴联合切除术(APR)并扩大切除肛周皮肤,切缘阴性,原发伤口愈合。随访12个月期间未观察到复发或转移。

结论

继发性PPD误诊率高。临床医生应对有长期肛周症状(如瘙痒、便血>6个月)的老年患者保持高度怀疑,并及时进行结肠镜检查和免疫组化检测以明确诊断。APR联合扩大肛周切除术是一种有效的治疗方法,规范的长期随访对预后至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e7f/12427061/bad6b15ca7d1/wjgs-17-8-108963-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e7f/12427061/bad6b15ca7d1/wjgs-17-8-108963-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1e7f/12427061/bad6b15ca7d1/wjgs-17-8-108963-g001.jpg

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本文引用的文献

1
Perianal Paget Disease.肛周佩吉特病
JAMA Dermatol. 2024 Nov 1;160(11):1239-1240. doi: 10.1001/jamadermatol.2024.4172.
2
Treatment of high tumor mutation burden metastatic extramammary Paget disease with an anti-PD-1 antibody.用抗程序性死亡蛋白1(PD-1)抗体治疗高肿瘤突变负荷的转移性乳腺外佩吉特病。
J Dermatol. 2023 Nov;50(11):e396-e397. doi: 10.1111/1346-8138.16898. Epub 2023 Jul 17.
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A review of 14 cases of perianal Paget's disease: characteristics of anorectal cancer with pagetoid spread.回顾 14 例肛周派杰病患者:伴派杰样播散的肛门直肠癌特征。
World J Surg Oncol. 2023 Jan 20;21(1):17. doi: 10.1186/s12957-022-02872-z.
4
Treatment of Extramammary Paget Disease and the Role of Reflectance Confocal Microscopy: A Prospective Study.乳腺外 Paget 病的治疗及反射共聚焦显微镜的作用:一项前瞻性研究。
Dermatol Surg. 2021 Apr 1;47(4):473-479. doi: 10.1097/DSS.0000000000002934.
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Perianal Paget's disease as spread from non-invasive colorectal adenomas.肛周派杰病可由非侵袭性结直肠腺瘤播散而来。
Histopathology. 2021 Jan;78(2):276-280. doi: 10.1111/his.14218. Epub 2020 Sep 23.
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PD-L1 Expression in Extramammary Paget Disease: A Case Series.PD-L1 表达在外阴 Paget 病中的研究:病例系列。
Am J Dermatopathol. 2021 Jan 1;43(1):21-26. doi: 10.1097/DAD.0000000000001622.
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Comparison of the biomarkers for targeted therapies in primary extra-mammary and mammary Paget's disease.原发性乳腺外及乳腺派杰氏病中靶向治疗生物标志物的比较。
Cancer Med. 2020 Feb;9(4):1441-1450. doi: 10.1002/cam4.2820. Epub 2020 Jan 3.
8
Extramammary Paget Disease: A Review of the Literature-Part I: History, Epidemiology, Pathogenesis, Presentation, Histopathology, and Diagnostic Work-up.乳房外帕哲病:文献回顾——第一部分:历史、流行病学、发病机制、临床表现、组织病理学和诊断方法。
Dermatol Surg. 2020 Feb;46(2):151-158. doi: 10.1097/DSS.0000000000002064.
9
Sex differences in initial treatment for genital extramammary Paget disease in the United States: A systematic review.美国外生殖器 Paget 病初始治疗的性别差异:系统评价。
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Dermatol Surg. 2018 Mar;44(3):354-364. doi: 10.1097/DSS.0000000000001355.