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肛周皮肤区域腺癌合并肛周乳腺外佩吉特病1例报告

Perianal extramammary Paget's disease with adenocarcinoma of perianal skin area, a case report.

作者信息

Alhebshi Alhasan H, Aldekhail Mohammed, Abduljabbar Alaa

机构信息

International Medical Center in Jeddah, Kingdom of Saudi Arabia.

Al-Qassim University Medical School, Qassim, Saudi Arabia.

出版信息

J Surg Case Rep. 2023 Jun 16;2023(6):rjad291. doi: 10.1093/jscr/rjad291. eCollection 2023 Jun.

DOI:10.1093/jscr/rjad291
PMID:37337540
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10276950/
Abstract

Extramammary Paget disease (EMPD) is an uncommon slow-growing skin adenocarcinoma originating in the anogenital region and axilla outside the mammary glands, often in regions with apocrine glands. The most common location is the vulva, followed by perineal, perianal, scrotal and penile skin. Here, we report a case of a 63-year-old male with EMPD in the perianal region. He reported 4 years of pain associated with an increasing region of skin irritation and bleeding on defecation that did not improve with topical agents. A biopsy sample revealed poorly differentiated carcinoma consistent with adenocarcinoma and associated with Paget disease. Workup was done. The patient tolerated local excision of the region well with no complications. A rare disease, EMPT, is challenging to diagnose and manage. Histopathological findings can, however, differentiate it from a wide array of similar skin conditions. Thorough investigations should be undertaken before initiating treatment to ensure the best outcomes.

摘要

乳房外佩吉特病(EMPD)是一种罕见的生长缓慢的皮肤腺癌,起源于乳腺外的肛门生殖器区域和腋窝,常见于有顶泌汗腺的区域。最常见的部位是外阴,其次是会阴、肛周、阴囊和阴茎皮肤。在此,我们报告一例63岁男性肛周EMPD病例。他自述4年来排便时疼痛,伴有皮肤刺激区域增大及出血,局部用药无改善。活检样本显示为低分化癌,符合腺癌且与佩吉特病相关。已进行了相关检查。患者对该区域的局部切除耐受良好,无并发症。EMPT是一种罕见疾病,诊断和治疗具有挑战性。然而,组织病理学检查结果可将其与多种类似的皮肤疾病相鉴别。在开始治疗前应进行全面检查以确保最佳治疗效果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/518e/10276950/61094e3f0ceb/rjad291f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/518e/10276950/90b1f05bc1b2/rjad291f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/518e/10276950/364f9b700273/rjad291f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/518e/10276950/d3ba4bbdf3a1/rjad291f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/518e/10276950/61094e3f0ceb/rjad291f4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/518e/10276950/90b1f05bc1b2/rjad291f1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/518e/10276950/364f9b700273/rjad291f2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/518e/10276950/d3ba4bbdf3a1/rjad291f3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/518e/10276950/61094e3f0ceb/rjad291f4.jpg

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本文引用的文献

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Stage 3 perianal extra mammary Paget's disease: A case report of a non-operative approach.3期肛周乳腺外佩吉特病:非手术治疗方法的病例报告
Int J Surg Case Rep. 2022 Feb;91:106796. doi: 10.1016/j.ijscr.2022.106796. Epub 2022 Jan 24.
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Extramammary Paget's Disease in the Genital Area of a Male: A Case Report and Review of the Literature.男性生殖器区域的乳腺外佩吉特病:一例报告并文献复习
Front Oncol. 2021 Nov 4;11:713786. doi: 10.3389/fonc.2021.713786. eCollection 2021.
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A Case Report of Familial Extramammary Paget's Disease in Female Siblings.
女性同胞家族性乳腺外佩吉特病1例报告
Case Rep Dermatol. 2021 Mar 22;13(1):176-183. doi: 10.1159/000514253. eCollection 2021 Jan-Apr.
4
Extramammary Paget disease: five perianal case report and treatment options.乳房外佩吉特病:5例肛周病例报告及治疗选择
J Surg Case Rep. 2021 Jun 17;2021(6):rjab262. doi: 10.1093/jscr/rjab262. eCollection 2021 Jun.
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Metastatic extramammary paget disease, a remarkable clinical case and a brief review of a rare disease.转移性乳腺外佩吉特病:一例典型临床病例及对罕见疾病的简要综述
Dermatol Reports. 2020 Oct 22;12(2):8841. doi: 10.4081/dr.2020.8841. eCollection 2020 Sep 23.
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An advanced case of extramammary Paget disease: Safe and effective treatment in an inoperable elderly patient using extensive en face electron irradiation.一例晚期乳腺外佩吉特病:对一名无法手术的老年患者采用大面积直视电子照射进行安全有效的治疗
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Evidence-Based Screening Recommendations for Occult Cancers in the Setting of Newly Diagnosed Extramammary Paget Disease.基于证据的筛查建议:新诊断出的非乳腺派杰氏病中隐匿性癌症的筛查。
Mayo Clin Proc. 2018 Jul;93(7):877-883. doi: 10.1016/j.mayocp.2018.02.024. Epub 2018 May 24.
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Metastatic Extramammary Paget's Disease: Pathogenesis and Novel Therapeutic Approach.转移性乳腺外佩吉特病:发病机制与新型治疗方法
Front Oncol. 2018 Feb 16;8:38. doi: 10.3389/fonc.2018.00038. eCollection 2018.
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Gastroenterology Res. 2016 Dec;9(6):99-102. doi: 10.14740/gr727e. Epub 2016 Dec 23.
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