Laboratory Medicine Program, Anatomic Pathology, University Health Network, Toronto, Ontario, Canada.
Department of Basic Medical Sciences, Faculty of Medicine, Al-Balqa' Applied University, Al-Salt, Jordan.
J Cutan Pathol. 2022 Jul;49(7):663-668. doi: 10.1111/cup.14223. Epub 2022 Mar 23.
Extramammary Paget disease (EMPD) is an uncommon disease affecting older men and women. Clinically, it appears as a plaque lesion with an erythematous or leukoplakic background in regions with abundant apocrine glands such as female external genitalia, perineum, scrotum, and penis.
We are presenting an 85-year-old patient with recurrent erythematous plaque lesions involving the penis and known to have urothelial carcinoma (UC) in situ of the bladder. A literature review of EMPD secondary to UC has been conducted through PubMed and Google Scholar search engines.
The histopathologic examination revealed a proliferation of Paget cells within the surface squamous epithelium. The lesional cells displayed vesicular nuclei, clear cytoplasm, and a positive staining for CK7, CK20, HER2, GATA3 as well as p40, and negative staining for SOX10, CK5/6, and CDX2. The literature review revealed 18 more cases of EMPD associated with UC, including 4 with non-invasive UC. Most of them were treated surgically, but the disease recurred in nine cases and death of disease was reported in at least four patients, all of them associated with invasive UC.
The prognosis of penile EMPD seems to be dictated by the stage of the underlying UC.
乳房外 Pagets 病（EMPD）是一种罕见的疾病，影响老年男性和女性。临床上，它表现为红斑或白色斑块病变，位于大汗腺丰富的区域，如女性外生殖器、会阴、阴囊和阴茎。
我们介绍了一位 85 岁的患者，他有复发性红斑斑块病变，累及阴茎，已知患有膀胱原位尿路上皮癌（UC）。通过 PubMed 和 Google Scholar 搜索引擎对继发于 UC 的 EMPD 进行了文献回顾。
组织病理学检查显示表面鳞状上皮内有 Paget 细胞增殖。病变细胞显示泡状核、透明细胞质，CK7、CK20、HER2、GATA3 以及 p40 阳性染色，SOX10、CK5/6 和 CDX2 阴性染色。文献回顾显示，与 UC 相关的 EMPD 还有 18 例，其中 4 例为非浸润性 UC。大多数患者接受了手术治疗，但有 9 例疾病复发，至少有 4 例患者死于疾病，均与浸润性 UC 有关。
阴茎 EMPD 的预后似乎取决于基础 UC 的分期。
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