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Abstract：
BACKGROUND: Extramammary Paget\'s disease (EMPD) is a rare malignant disease originating from the apocrine glands involving the perineum, vulva, axilla, scrotum, and penis.
OBJECTIVE: To study the clinical presentation, extent of disease, efficacy of treatment, and survival outcomes of the cases in a single institution.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
OBJECTIVE: To study the clinical presentation, extent of disease, efficacy of treatment, and survival outcomes of the cases in a single institution.
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.
摘要：
背景：乳房外Paget病（EMPD）是一种罕见的恶性疾病，起源于累及会阴的大汗腺，外阴,腋下,阴囊,和阴茎。
目的：为了研究临床表现，疾病的程度,治疗效果，以及单个机构中案件的生存结果。
方法：对19例EMPD患者进行回顾性观察资料分析。人口统计信息,临床管理记录，个别病例的组织病理学数据来自住院患者的数据注册表。
结果：诊断时的平均年龄（岁）为62.4，性别分布相同。同步肿瘤6例(31.5%)。19例患者中有18例接受了广泛局部切除（WLE）和重建手术形式的明确手术治疗。在11例（68.8％）病例中发现了阳性切缘，在这11例中有7例接受了第二次手术干预以实现肿瘤清除或辅助肿瘤治疗。存活患者的随访时间因诊断时间和明确治疗而异。在研究时，19例病例中有10例(52.7%)还活着。在7例癌症死亡病例中，5例死于潜在相关恶性肿瘤进展，仅2例死于EMPD晚期。
结论：EMPD可能相当激进，尤其是在次要形式中，手术边缘肿瘤残留率高，手术治疗具有挑战性。EMPD很容易误导临床医生和患者，导致在确定有效管理之前不必要的延迟。
目的：为了研究临床表现，疾病的程度,治疗效果，以及单个机构中案件的生存结果。
方法：对19例EMPD患者进行回顾性观察资料分析。人口统计信息,临床管理记录，个别病例的组织病理学数据来自住院患者的数据注册表。
结果：诊断时的平均年龄（岁）为62.4，性别分布相同。同步肿瘤6例(31.5%)。19例患者中有18例接受了广泛局部切除（WLE）和重建手术形式的明确手术治疗。在11例（68.8％）病例中发现了阳性切缘，在这11例中有7例接受了第二次手术干预以实现肿瘤清除或辅助肿瘤治疗。存活患者的随访时间因诊断时间和明确治疗而异。在研究时，19例病例中有10例(52.7%)还活着。在7例癌症死亡病例中，5例死于潜在相关恶性肿瘤进展，仅2例死于EMPD晚期。
结论：EMPD可能相当激进，尤其是在次要形式中，手术边缘肿瘤残留率高，手术治疗具有挑战性。EMPD很容易误导临床医生和患者，导致在确定有效管理之前不必要的延迟。
