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Journal of Lower Genital Tract Disease:
doi: 10.1097/LGT.0b013e31825f4b4f
Original Articles

Extramammary Paget Disease: Epidemiology and Association to Cancer in a Quebec-Based Population

Funaro, Deana MD, FRCP, FAAD1; Krasny, Mark MD, FRCP2; Lam, Christina MD, FRCP2; Desy, Delphine MD, FRCP3; Sauthier, Philippe MD, FRCP4; Bouffard, Danielle MD, FRCP3

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Objective: This study aimed to further characterize the epidemiology, clinical manifestations, pathology, immunopathology, outcome from therapy, and associated underlying malignancy in extramammary Paget disease (EMPD).

Materials and Methods: We conducted a retrospective review of patients treated for EMPD in our tertiary care center during a 23-year period ranging from 1985 to 2008.

Results: Sixty-four cases of EMPD were diagnosed during this period. Mean age at diagnosis was 66.8 years. Of the patients, 79.7% were female. Tumors were mostly localized on the vulvoperineal region. Associated cancers were found in 30% of the patients and included breast cancer and urogenital cancers. Of the patients, 42% had a least 1 recurrence. The risk of recurrence could only be associated to tumor location on the vulvoperineal region. The limitations of this study include its retrospective nature and sample size.

Conclusions: Extramammary Paget disease is more commonly found on the vulva of older women and frequently recurs. Recurrence was not associated to margin status, which would support a more conservative therapeutic approach.

©2013The American Society for Colposcopy and Cervical Pathology


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