摘要目的 探讨肛周Paget病的临床病理特点和诊治方法,提高对此病的认识.方法 回顾性分析17例经手术及病理证实的肛周Paget病患者的临床资料.结果 17例中男11例,女6例,平均年龄62.5岁.12例患者以肛门周围湿疹、瘙痒、糜烂及溃疡就诊;5例以痔疮、结肠炎、尖锐湿疣及慢性肛周感染就诊.术前误诊为湿疹、痔疮和直肠癌等疾病者6例.7例行局部广泛切除+植皮术,其中3例术后辅以放射治疗.10例行Miles术,其中6例行局部淋巴结清扫,并辅以术后化疗.除2例失访外,15例获得随访,随访时间8个月至6年,15例中8例复发,7例死于该病.结论 肛周Paget病缺乏特征性的临床表现,误诊率高;一旦确诊应及早手术,术后应采取放疗及化疗等综合治疗.

abstractsObjective To evaluate clinical characteristics,diagnosis and treatment of perianal Paget's disease.Methods Clinical data of 17 perianal Paget's disease patients confirmed by pathology and immunohistochemistry were retrospectively analysed.Results There were 11 males and 6 females with a median age of 62.5 years.The main manifestations were eczema,itch,erosion and ulcer formation around the anus.Five complained of hemorrhoids,colonitis,condyloma and chronic infection around the anus.Six patients were misdiagnosed as eczema,hemorrhoids and rectum cancer before definite diagnosis was made.All cases received surgery.Seven patients were subject to local extensive resection,three received adjuvant radiotherapy.Ten underwent Miles' operation,six of them with local lymphadenectomy and chemotherapy.Fifteen patients were followed up from 8 months to 6 years.Recurrence occurred in 8 patients causing a death in 7 cases.Conclusions Perianal Paget's disease has no specific clinical characteristics,and the misdiagnosis rate is high.Once the diagnosis of perianal Paget's disease is made,a surgery should be performed as early as possible,adjuvant radiotherapy and chemotherapy helps prolong patient's survival.