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阴囊侵袭性乳腺外佩吉特病:一例报告。

Invasive extramammary Paget's disease of the scrotum: A case report.

作者信息

Li Heidi Oi-Yee, Van Alicia, Lipson Jennifer

机构信息

Division of Dermatology, The Ottawa Hospital, University of Ottawa, ON, Canada.

Faculty of Medicine, University of Ottawa, ON, Canada.

出版信息

SAGE Open Med Case Rep. 2025 Jul 8;13:2050313X251350334. doi: 10.1177/2050313X251350334. eCollection 2025.

DOI:10.1177/2050313X251350334
PMID:40657443
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12246521/
Abstract

Extramammary Paget's disease is a rare cutaneous malignancy that typically presents as carcinoma in situ, and instances of invasive extramammary Paget's disease are exceptionally rare. Herein, we describe a rare case of invasive extramammary Paget's disease of the scrotum. A 91-year-old male presented with an over 10-year history of a granulomatous rash with serous discharge, refractory to various topical therapies and weekly wound care. Physical examination revealed a well-demarcated, erythematous, mamillated, eroded plaque with arcuate borders involving the left inguinal crease and scrotum. A punch biopsy showed intra-epidermal and invasive carcinoma with focal pagetoid spread and intracytoplasmic mucin. The favored diagnosis was primary, invasive extramammary Paget's disease. The patient underwent a wide local excision and routine imaging surveillance and remains recurrence-free at 1-year follow-up. Given that extramammary Paget's disease remains commonly misdiagnosed, leading to delays in treatment, dermatologists should be aware of this diagnosis, particularly for elderly patients presenting with non-specific genital lesions recalcitrant to routine treatment.

摘要

乳房外佩吉特病是一种罕见的皮肤恶性肿瘤,通常表现为原位癌,而侵袭性乳房外佩吉特病的病例极为罕见。在此,我们描述一例罕见的阴囊侵袭性乳房外佩吉特病病例。一名91岁男性,有超过10年的肉芽肿性皮疹伴浆液性渗出病史,对各种局部治疗和每周一次的伤口护理均无效。体格检查发现一个边界清晰、红斑状、乳头状、糜烂的斑块,边界呈弧形,累及左腹股沟皱襞和阴囊。穿刺活检显示表皮内和侵袭性癌,伴有局灶性派杰样扩散和胞质内黏液。最可能的诊断是原发性侵袭性乳房外佩吉特病。患者接受了广泛局部切除和常规影像学监测,在1年随访时无复发。鉴于乳房外佩吉特病仍常被误诊,导致治疗延误,皮肤科医生应了解这一诊断,尤其是对于出现对常规治疗无效的非特异性生殖器病变的老年患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad52/12246521/33c921a74c13/10.1177_2050313X251350334-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad52/12246521/33c921a74c13/10.1177_2050313X251350334-fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ad52/12246521/33c921a74c13/10.1177_2050313X251350334-fig1.jpg

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本文引用的文献

1
Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease.乳腺外佩吉特病的循证临床实践指南
JAMA Oncol. 2022 Apr 1;8(4):618-628. doi: 10.1001/jamaoncol.2021.7148.
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Curr Oncol. 2021 Aug 5;28(4):2969-2986. doi: 10.3390/curroncol28040260.
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Dermatol Surg. 2020 Mar;46(3):305-311. doi: 10.1097/DSS.0000000000002240.
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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.
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Extramammary Paget disease.乳房外佩吉特病
Dermatol Online J. 2019 Apr 15;25(4):13030/qt7qg8g292.
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Evaluation of positron emission tomography imaging to detect lymph node metastases in patients with extramammary Paget's disease.正电子发射断层扫描成像用于检测乳腺外佩吉特病患者淋巴结转移的评估
J Dermatol. 2017 Aug;44(8):939-943. doi: 10.1111/1346-8138.13833. Epub 2017 Mar 11.
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Treatment outcomes in a large cohort of patients with invasive Extramammary Paget's disease.大样本侵袭性乳房外 Paget 病患者的治疗结果。
Gynecol Oncol. 2012 May;125(2):346-51. doi: 10.1016/j.ygyno.2012.01.032. Epub 2012 Jan 28.
8
Extramammary Paget's disease: Summary of current knowledge.乳房外 Paget 病:现有知识概述。
Dermatol Clin. 2010 Oct;28(4):807-26. doi: 10.1016/j.det.2010.08.002.
9
Vulvar toker cells: the long-awaited missing link: a proposal for an origin-based histogenetic classification of extramammary paget disease.外阴托克尔细胞:期待已久的缺失环节:关于乳腺外佩吉特病基于起源的组织发生学分类的提议
Am J Dermatopathol. 2006 Feb;28(1):84-6. doi: 10.1097/01.dad.0000194052.65695.f1.
10
Vulvar clear cells of Toker: precursors of extramammary Paget's disease.托克尔外阴透明细胞:乳腺外佩吉特病的前驱病变。
Am J Dermatopathol. 2005 Jun;27(3):185-8. doi: 10.1097/01.dad.0000158291.20440.13.