Chang Yu-Wei, Ma Hsu, Liao Wen-Chieh
Division of Plastic and Reconstructive Surgery, Department of Surgery, Taipei Veterans General Hospital, 19F, No.201 Shih-Pai RD Sec 2, Taipei, Taiwan.
School of Medicine, National Yang-Ming University, Taipei, Taiwan.
World J Surg Oncol. 2021 Apr 12;19(1):110. doi: 10.1186/s12957-021-02228-z.
This study aimed to investigate the survival analysis of extramammary Paget's disease (EMPD) in a Taiwanese population and to provide data for comparison with other studies in various locations and racial populations.
We retrospectively analyzed the medical records of 63 patients with EMPD who were surgically treated from 2002 to 2019 at a single institution. The primary endpoint was the 5-year overall survival rate of EMPD, and the secondary endpoint was recurrence-free 5-year survival. Independent variables included patients' demographic data, concurrent malignancy (i.e., non-EMPD-related cancers), tumor size, distant metastasis, and surgery and/or radiation.
Of all the 63 patients, 8 cases were excluded. A total of 43 patients (78.18%) were male, and 12 were female, with a mean age of 72.67 years (range 44-89 years). The most common affected anatomic site was the penoscrotal region (22 patients, 40.00%), followed by the perianal and perineal regions (17 patients, 30.91%). Among the 55 patients, 41 patients (74.55%) were diagnosed with at least one underlying disease, whereas the most common underlying disease was cardiovascular disease (30 patients, 54.55%). The overall survival rate was 80.00% at 36 months and 65.45% at the end of follow-up. EMPD with deep dermal invasion was a significant poor prognostic factor of overall survival in cause-specific hazard model (sub-hazard ratio (HR) 5.167, p = 0.0015, 95% confidence interval (CI) 1.876-14.230). Patients with regional metastasis or distant metastasis had poorer prognosis of 5-year survival (sub-HR 4.513, p = 0.0028, CI 1.683-12.103). The limitations of this study include its retrospective nature and sample size.
In our series, EMPD with metastasis and deep dermal invasion was the significant harmful factors in both overall 5-year survival and 5-year recurrence-free survival. The surgical excision is not associated with a low risk of local recurrence or overall survival, and long-term follow-up is still needed.
本研究旨在调查台湾人群中外阴佩吉特病（EMPD）的生存分析，并提供数据以便与其他不同地区和种族人群的研究进行比较。
我们回顾性分析了2002年至2019年在单一机构接受手术治疗的63例EMPD患者的病历。主要终点是EMPD的5年总生存率，次要终点是5年无复发生存率。自变量包括患者的人口统计学数据、并发恶性肿瘤（即与EMPD无关的癌症）、肿瘤大小、远处转移以及手术和/或放疗。
63例患者中，8例被排除。共有43例患者（78.18%）为男性，12例为女性，平均年龄72.67岁（范围44 - 89岁）。最常受累的解剖部位是阴茎阴囊区域（22例患者，40.00%），其次是肛周和会阴区域（17例患者，30.91%）。在这55例患者中，41例患者（74.55%）被诊断患有至少一种基础疾病，而最常见的基础疾病是心血管疾病（30例患者，54.55%）。36个月时总生存率为80.00%，随访结束时为65.45%。在病因特异性风险模型中，伴有真皮深层浸润的EMPD是总生存的显著不良预后因素（亚风险比（HR）5.167，p = 0.0015，95%置信区间（CI）1.876 - 14.230）。有区域转移或远处转移的患者5年生存率预后较差（亚HR 4.513，p = 0.0028，CI 1.683 - 12.103）。本研究的局限性包括其回顾性性质和样本量。
在我们的系列研究中，伴有转移和真皮深层浸润的EMPD是5年总生存和5年无复发生存的显著有害因素。手术切除与局部复发风险低或总生存无关，仍需要长期随访。
