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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

Journal of Lower Genital Tract Disease: April 2013 - Volume 17 - Issue 2 - p 167–174
doi: 10.1097/LGT.0b013e31825f4b4f
Original Articles

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.

Recurrence of extramammary Paget disease was not associated to margin status, which would support a more conservative therapeutic approach.

1Division of Vulvar Disease, 2Department of Dermatology, 3Department of Pathology, and 4Department of Gynecology-Oncology, Centre Hospitalier Universitaire de Montreal, University of Montreal, Quebec, Canada

Reprint requests to: Deana Funaro, MD, Department of Dermatology, St-Luc Hospital, Centre Hospitalier Universitaire de Montreal, 1058, rue Saint-Denis, Montréal, Québec, Canada H2X 3J4. E-mail:

A bursary granted through the 2008 to 2009 Astellas research competition supported this study.

The authors have no financial and conflict of interest to disclose.

The results of this study were presented at the Annual Quebec dermatologists’ conference in May 2011.

©2013The American Society for Colposcopy and Cervical Pathology