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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Paget病: 文献综述-第一部分: 病史、流行病学学、发病机制、表现、组织病理学和诊断检查。
- 影响因子:1.25
- DOI:10.1097/DSS.0000000000002064
- 作者列表:"Morris CR","Hurst EA
- 发表时间:2020-02-01
Abstract
BACKGROUND:Extramammary Paget disease (EMPD) is a rare malignancy with unclear pathophysiology that occurs predominantly on apocrine rich skin. Surgery is the treatment of choice; however, procedures tend to be extensive and associated with a high rate of recurrence. OBJECTIVE:To review the current literature on EMPD regarding epidemiology, pathogenesis, clinical presentation, histology, diagnostic work-up, treatment, and prognosis. MATERIALS AND METHODS:Literature review using PubMed search for articles related to EMPD. RESULTS:Extramammary Paget disease classically presents as a slowly growing red plaque, which often mimics an inflammatory condition leading to significant delay in diagnosis. Diagnosis requires histopathologic examination and is often supported by immunohistochemical analysis. Once a diagnosis of EMPD is made, the patient must be risk-stratified and evaluated for an underlying malignancy. CONCLUSION:Standard of treatment is surgery, with data suggesting that Mohs micrographic surgery may have superior clinical outcomes and lower recurrence rates. Alternatives such as photodynamic therapy and topicals have been explored and may be appropriate in certain situations. Patients with EMPD generally have a good prognosis with a 5-year overall survival rate of 75% to 95%.
摘要
背景: 乳房外Paget病 (EMPD) 是一种罕见的恶性肿瘤,病理生理学尚不清楚,主要发生在富含大汗腺的皮肤上。手术是首选的治疗方法; 然而,手术往往是广泛的,复发率高。 目的: 回顾目前有关EMPD流行病学学、发病机制、临床表现、组织学、诊断检查、治疗和预后的文献。 材料与方法: 利用PubMed检索与EMPD相关的文章进行文献综述。 结果: 乳房外Paget病经典表现为缓慢生长的红色斑块,常模拟炎症状态,导致诊断显著延迟。诊断需要组织病理学检查,并经常得到免疫组织化学分析的支持。一旦诊断为EMPD,患者必须进行风险分层,并评估潜在的恶性肿瘤。 结论: 治疗标准为手术,数据表明Mohs显微外科手术可能具有较好的临床疗效和较低的复发率。已经探索了光动力疗法和局部药物等替代方法,在某些情况下可能是合适的。EMPD患者一般预后良好,5 年总生存率为 75% ~ 95%。
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皮肤肿瘤是发生在皮肤的细胞增生性疾病,是一种常见病。发生于皮内或皮下组织的新生物,种类很多,临床上分良性肿瘤和恶性肿瘤。恶性肿瘤可以不断增殖,引起转移,威胁生命,称为皮肤癌。