Extramammary Paget disease: epidemiology and association to cancer in a Quebec-based population.
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).


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.
Division of Vulvar Disease, Centre Hospitalier Universitaire de Montréal, University of Montreal, Québec, Canada H2X 3J4. rouleaufunaro@videotron.ca
Deana
D
Funaro
Mark
M
Krasny
Christina
C
Lam
Delphine
D
Desy
Philippe
P
Sauthier
Danielle
D
Bouffard
D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001943:Breast Neoplasms / Q000453:epidemiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010145:Paget Disease, Extramammary / Q000150:complications / Q000453:epidemiology* / Q000473:pathology*; D010502:Perineum / Q000473:pathology; D011792:Quebec / Q000453:epidemiology; D012008:Recurrence; D012189:Retrospective Studies; D014565:Urogenital Neoplasms / Q000453:epidemiology; D014844:Vulva / Q000473:pathology