Retrospective analysis of perianal Paget's disease with underlying anorectal carcinoma.
OBJECTIVE
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.


CONCLUSIONS
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.
Lian
Peng
P
Department of Colorectal Surgery, Fudan University, Shanghai Cancer Center, Shanghai 200032, China.
Gu
Wei-Lie
WL
Zhang
Zhen
Z
Cai
Guo-Xiang
GX
Wang
Ming-He
MH
Xu
Ye
Y
Sheng
Wei-Qi
WQ
Cai
San-Jun
SJ
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001005:Anus Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D002681:China; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010145:Paget Disease, Extramammary / Q000175:diagnosis* / Q000628:therapy; D012004:Rectal Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D012189:Retrospective Studies