Brief Article
Copyright ©2010 Baishideng. All rights reserved.
World J Gastroenterol. Jun 21, 2010; 16(23): 2943-2948
Published online Jun 21, 2010. doi: 10.3748/wjg.v16.i23.2943
Retrospective analysis of perianal Paget’s disease with underlying anorectal carcinoma
Peng Lian, Wei-Lie Gu, Zhen Zhang, Guo-Xiang Cai, Ming-He Wang, Ye Xu, Wei-Qi Sheng, San-Jun Cai
Peng Lian, Wei-Lie Gu, Guo-Xiang Cai, Ming-He Wang, Ye Xu, San-Jun Cai, Department of Colorectal Surgery, Fudan University, Shanghai Cancer Center, Shanghai 200032, China
Zhen Zhang, Department of Radiation Oncology, Fudan University, Shanghai Cancer Center, Shanghai 200032, China
Wei-Qi Sheng, Department of Pathology, Fudan University, Shanghai Cancer Center, Shanghai 200032, China
Author contributions: Gu WL, Cai GX, Wang MH and Xu Y contributed to the collection of clinicopathological information and follow up; Zhang Z performed some of the irradiation treatment; Sheng WQ and colleagues performed the pathological diagnoses; Cai SJ and Lian P designed the study; Lian P wrote the manuscript; Cai SJ provided financial support for this work.
Supported by Key Project of Science and technology commission of Shanghai Municipality, Shanghai, China, No. 07DZ19505
Correspondence to: Dr. San-Jun Cai, Department of Colorectal Surgery, Fudan University, Shanghai Cancer Center, Shanghai 200032, China. caisanjun1208@yahoo.cn
Telephone: +86-21-64175590 Fax: +86-21-64035387
Received: February 20, 2010
Revised: April 6, 2010
Accepted: April 13, 2010
Published online: June 21, 2010
Abstract

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.

Keywords: Perianal Paget’s disease; Anorectal cancer; Treatment; Prognosis