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.
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。