AIM:To analyze clinical and pathological characteristics of an aggressive subtype of perianal Paget’s disease(PPD) and explore its rational treatment modalities.METHODS:PPD patients were retrospectively collected in the institutional colorectal database of the Fudan University Shanghai Cancer Center.Detailed patient histories of past medical condition,diagnosis,treatment,and pathological findings were reviewed.Surgical specimen from diagnosis and surgery were reviewed by two independent pathologists for confirmation of diagnoses.Follow up was accomplished by clinical interview by cellphone.RESULTS:In total,eight cases of PPD were analyzed.All patients had underlying anorectal adenocarcinoma,including seven with synchronous lesions and one with metachronous lesions.Moreover,all anorectal lesions had a mucin-producing component.The median age at diagnosis was 65(range 29-81 years),and the male/female ratio was 7:1.The Median follow-up time of all patients was 61.5 mo(range 10-204 mo).One patient treated with abdominoperineal resection(APR) died from lung metastases 10 mo after the APR operation.The other patients are still free of disease at the time of this analysis.CONCLUSION:PPD is a rare malignancy and is easily misdiagnosed.Underlying anorectal cancer was not unusual and was a significant prognostic factor.Rational treatment of both anorectal cancer and PPD lesion is essential for long-term survival. AIM: To analyze clinical and pathological characteristics of an aggressive subtype of perianal Paget’s disease (PPD) and explore its rational treatment modalities. METHODS: PPD patients were retrospectively collected in the institutional colorectal database of the Fudan University Shanghai Cancer Center. Dtailed patient histories of past medical condition, diagnosis, treatment, and pathological findings were reviewed. Surgical specimens from diagnosis and surgery were reviewed by two independent pathologists for confirmation of diagnoses. Vollow up was accomplished by clinical interview by cellphone .RESULTS: In total, eight cases of PPD were analyzed. All patients had the underlying anorectal adenocarcinoma, including seven with synchronous lesions and one with metachronous lesions. More over, all anorectal lesions had a mucin-producing component. The median age at diagnosis was 65 (range 29-81 years), and the male / female ratio was 7: 1.The Median follow-up time of all patients was 61.5 mo (range 10-204 mo) .One patient treated with abdominoperineal resection (APR) died from lung metastases 10 months after the APR operation. the other patients are still free of disease at the time of this analysis. CONCLUSION: PPD is a rare malignancy and is easily misdiagnosed. Underlying anorectal cancer was not unusual and was a significant prognostic factor. Rational treatment of both anorectal cancer and PPD lesion is essential for long-term survival.