Abstract：
BACKGROUND: Perianal Paget\'s disease (PPD) is an intraepithelial invasion of the perianal skin and is frequently associated with underlying anorectal carcinoma. The relatively rare nature of this disease has made it difficult to develop treatment recommendations. This study aims to analyze the clinical and pathological features of perianal Paget\'s disease (PPD) and to explore rational treatment options and follow-up for this disease.
METHODS: The National Cancer Center Hospital database was searched for all cases of perianal Paget\'s disease diagnosed between 2006 and 2021. In the 14 patients identified, we reviewed the diagnosis, management, and outcomes of adenocarcinoma with pagetoid spread, including suspected or recurrent cases.
RESULTS: All 14 cases met the inclusion criteria. The median follow-up period after diagnosis was 4.5 (range, 0.1-13.0) years. Pagetoid spread before initial treatment was suspected in 12 cases (85.7%). Underlying rectal cancer was identified in 6 cases, and no primary tumor was detected in the other 6 cases. Seven patients had recurrent disease, with the median time to recurrence of 34.6 (range, 19.2-81.7) months. The time to the first relapse was 3 months, and that to the second relapse was 6 months. The overall 5-year survival rate was 90.0%.
CONCLUSIONS: Endoscopic and radiologic evaluation, as well as immunohistologic examination, should be performed. is to differentiate PPD with and without underlying anorectal carcinoma. The time to first recurrence varies widely, and long-term and regular follow-up for more than 5 years is considered necessary for local recurrence and distant metastasis.
METHODS: The National Cancer Center Hospital database was searched for all cases of perianal Paget\'s disease diagnosed between 2006 and 2021. In the 14 patients identified, we reviewed the diagnosis, management, and outcomes of adenocarcinoma with pagetoid spread, including suspected or recurrent cases.
RESULTS: All 14 cases met the inclusion criteria. The median follow-up period after diagnosis was 4.5 (range, 0.1-13.0) years. Pagetoid spread before initial treatment was suspected in 12 cases (85.7%). Underlying rectal cancer was identified in 6 cases, and no primary tumor was detected in the other 6 cases. Seven patients had recurrent disease, with the median time to recurrence of 34.6 (range, 19.2-81.7) months. The time to the first relapse was 3 months, and that to the second relapse was 6 months. The overall 5-year survival rate was 90.0%.
CONCLUSIONS: Endoscopic and radiologic evaluation, as well as immunohistologic examination, should be performed. is to differentiate PPD with and without underlying anorectal carcinoma. The time to first recurrence varies widely, and long-term and regular follow-up for more than 5 years is considered necessary for local recurrence and distant metastasis.
摘要：
背景：肛周Paget病（PPD）是肛周皮肤的上皮内侵袭，通常与潜在的肛门直肠癌有关。这种疾病的相对罕见的性质使得难以制定治疗建议。本研究旨在分析肛周Paget病(PPD)的临床和病理特征,探讨合理的治疗方案及随访情况。
方法：在国家癌症中心医院数据库中检索了2006年至2021年间诊断的所有肛周Paget病病例。在确定的14名患者中，我们回顾了诊断结果,管理,以及腺癌伴Pagetoid扩散的结果，包括可疑或复发病例。
结果：14例符合纳入标准。诊断后的中位随访期为4.5（范围，0.1-13.0）年。12例（85.7％）在初次治疗前怀疑类黄酮扩散。在6例中发现了潜在的直肠癌，其余6例未检出原发肿瘤。七个病人有反复发作的疾病,中位复发时间为34.6(范围，19.2-81.7）个月。第一次复发的时间是3个月，第二次复发是6个月。5年生存率为90.0%。
结论：内窥镜和放射学评估，以及免疫组织学检查，应该执行。是区分有和没有潜在的肛门直肠癌的PPD。首次复发的时间差异很大，认为局部复发和远处转移有必要进行5年以上的长期定期随访。
方法：在国家癌症中心医院数据库中检索了2006年至2021年间诊断的所有肛周Paget病病例。在确定的14名患者中，我们回顾了诊断结果,管理,以及腺癌伴Pagetoid扩散的结果，包括可疑或复发病例。
结果：14例符合纳入标准。诊断后的中位随访期为4.5（范围，0.1-13.0）年。12例（85.7％）在初次治疗前怀疑类黄酮扩散。在6例中发现了潜在的直肠癌，其余6例未检出原发肿瘤。七个病人有反复发作的疾病,中位复发时间为34.6(范围，19.2-81.7）个月。第一次复发的时间是3个月，第二次复发是6个月。5年生存率为90.0%。
结论：内窥镜和放射学评估，以及免疫组织学检查，应该执行。是区分有和没有潜在的肛门直肠癌的PPD。首次复发的时间差异很大，认为局部复发和远处转移有必要进行5年以上的长期定期随访。
