Phyo Aung Kyaw, Mun Kein-Seong, Kwan Kong Chee, Ann Chai Chu, Kuppusamy Shanggar
Division of Urology, Department of Surgery, University of Malaya Kuala Lumpur, Malaysia.
Department of Pathology, University Malaya Kuala Lumpur, Malaysia.
Int J Clin Exp Pathol. 2020 Sep 1;13(9):2369-2376. eCollection 2020.
Extramammary Paget's disease (EMPD) is a rare malignant disease originating from the apocrine glands involving the perineum, vulva, axilla, scrotum, and penis.
To study the clinical presentation, extent of disease, efficacy of treatment, and survival outcomes of the cases in a single institution.
Retrospective observation data analysis of 19 EMPD cases was performed. Demographic information, clinical management records, and histopathologic data of individual cases were obtained from the inpatient hospital data registry.
The mean age (years) at time of diagnosis was 62.4 with equal gender distribution. Synchronous tumors were detected in 6 cases (31.5%). 18 out of 19 patients underwent definitive surgical management in the form of wide local excision (WLE) and reconstructive surgery. Positive margins were found in 11 (68.8%) cases and 7 out of these 11 cases underwent second look surgical intervention to achieve oncological clearance or adjuvant oncology treatment. Follow-up period for living patients varied depending on time of diagnosis and definitive treatment. 10 out 19 cases (52.7%) were alive at the time of the study. Among the 7 cases of mortality from cancer, 5 cases died from progression of underlying associated malignancy and only 2 cases died with advanced stage of EMPD.
EMPD can be quite aggressive, especially in the secondary form, and surgical management is challenging with a high rate of residual tumor at the surgical margin. EMPD can easily mislead the clinician and patient, leading to unnecessary delay prior to definitive effective management.
乳腺外佩吉特病（EMPD）是一种罕见的恶性疾病，起源于顶泌汗腺，累及会阴、外阴、腋窝、阴囊和阴茎。
研究单一机构中病例的临床表现、疾病范围、治疗效果和生存结果。
对19例EMPD病例进行回顾性观察数据分析。从住院患者医院数据登记处获取个体病例的人口统计学信息、临床管理记录和组织病理学数据。
诊断时的平均年龄为62.4岁，性别分布均衡。6例（31.5%）检测到同步肿瘤。19例患者中有18例接受了以广泛局部切除（WLE）和重建手术形式的确定性手术治疗。11例（68.8%）病例发现手术切缘阳性，其中11例中的7例接受了二次手术干预以实现肿瘤清除或辅助肿瘤治疗。存活患者的随访期因诊断时间和确定性治疗而异。研究时19例中有10例（52.7%）存活。在7例癌症死亡病例中，5例死于潜在相关恶性肿瘤的进展，仅2例死于EMPD晚期。
EMPD可能具有相当的侵袭性，尤其是在继发性形式中，手术治疗具有挑战性，手术切缘残留肿瘤率高。EMPD很容易误导临床医生和患者，导致在确定性有效治疗之前出现不必要的延误。
