Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York.
Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York.
Dis Colon Rectum. 2014 Jun;57(6):747-751. doi: 10.1097/DCR.0000000000000100.
Perianal Paget's disease (intraepithelial adenocarcinoma) is rare and sometimes difficult to diagnose because symptoms are nonspecific. It is often noninvasive but frequently recurs locally. Invasive disease can metastasize to distant sites.
The purpose of this work was to review the diagnosis, management, and outcomes of patients with perianal Paget's disease.
Institutional databases were queried for all of the cases of perianal Paget's disease at Memorial Sloan-Kettering Cancer Center between 1950 and 2011. Clinicopathologic factors were investigated for association with recurrence and survival.
The study was conducted at a tertiary care center.
Sixty-five patients with perianal Paget's disease were included in the study (35 women [54%]; median age at diagnosis, 66 years [range, 60-72 years]; and 41 with invasive disease/24 with noninvasive disease). A total of 56% with invasive disease were men.
Measures included median follow-up, disease status, local and distant recurrence, sites of recurrence, disease-specific survival, overall survival, and treatment modality.
A total of 95% with invasive disease and 87% with noninvasive disease were symptomatic at presentation. The most common symptoms were pruritus and perianal bleeding. The duration of symptoms was longer in patients with invasive (12.0 months; range, 4.0-18.0 months) versus noninvasive (3.5 months; range, 1.0-10.0 months) disease. Synchronous malignancies unrelated to the primary disease were noted in 5 patients with invasive disease and 3 with noninvasive disease. Noninvasive disease was treated with a wide local excision and invasive disease with a wide local excision (n = 32, 78%) or abdominoperineal resection (n = 9, 22%). Forty-one patients (27 invasive and 14 noninvasive) required multiple operations for tumor clearance. In those with invasive disease, the median time to recurrence was 5 years, and the median tumor-specific survival rate was 10 years.
This was a retrospective study, limited by selection bias.
Perianal Paget's disease is associated with nonspecific symptoms, frequently delaying diagnosis. Wide local excision is the treatment of choice if negative margins can be obtained. Abdominoperineal resection should be considered for invasive disease. Local recurrence is common; follow-up includes periodic proctoscopy and digital examination. Invasive disease can metastasize to distant sites; follow-up should include the examination of inguinal lymph nodes and the imaging of liver and lungs.
肛周派杰病（上皮内腺癌）较为罕见，有时难以诊断，因为其症状不具有特异性。该病通常为非浸润性，但常局部复发。浸润性疾病可发生远处转移。
本研究旨在回顾分析肛周派杰病患者的诊断、治疗和结局。
回顾性分析纪念斯隆-凯特琳癌症中心 1950 年至 2011 年间的所有肛周派杰病病例。分析了临床病理因素与复发和生存的关系。
该研究在一家三级护理中心进行。
本研究共纳入 65 例肛周派杰病患者（35 例女性[54%]；中位诊断年龄 66 岁[范围 60-72 岁]；41 例为浸润性疾病，24 例为非浸润性疾病）。浸润性疾病患者中，56%为男性。
中位随访时间、疾病状态、局部和远处复发、复发部位、疾病特异性生存率、总生存率和治疗方式。
95%的浸润性疾病患者和 87%的非浸润性疾病患者在就诊时均有症状。最常见的症状是瘙痒和肛周出血。与非浸润性疾病患者（3.5 个月[范围 1.0-10.0 个月]）相比，浸润性疾病患者（12.0 个月[范围 4.0-18.0 个月]）的症状持续时间更长。5 例浸润性疾病患者和 3 例非浸润性疾病患者同时存在与原发性疾病无关的其他恶性肿瘤。非浸润性疾病患者采用广泛局部切除术治疗，浸润性疾病患者采用广泛局部切除术（n=32，78%）或腹会阴联合切除术（n=9，22%）治疗。41 例患者（27 例浸润性疾病，14 例非浸润性疾病）需要多次手术以清除肿瘤。浸润性疾病患者的中位复发时间为 5 年，中位肿瘤特异性生存率为 10 年。
这是一项回顾性研究，存在选择偏倚。
肛周派杰病常伴有非特异性症状，导致诊断延迟。如果能够获得阴性切缘，广泛局部切除术是首选治疗方法。对于浸润性疾病，应考虑行腹会阴联合切除术。局部复发常见；随访包括定期直肠镜检查和直肠指检。浸润性疾病可发生远处转移；随访应包括腹股沟淋巴结检查以及肝脏和肺部影像学检查。
