Surgical Treatment and Outcomes of Patients With Extramammary Paget Disease: A Cohort Study.
BACKGROUND
Extramammary Paget disease (EMPD) is a rare intraepithelial adenocarcinoma occurring mainly in the anogenital region. Traditional management with wide local excision has shown high recurrence rates, thus Mohs micrographic surgery (MMS) has emerged as a promising treatment option.


OBJECTIVE
To compare long-term outcomes after treatment with MMS or excision for primary EMPD.


METHODS
Retrospective cohort review was conducted for 207 patients with EMPD treated at Mayo Clinic in Rochester, MN, between 1961 and 2012.


RESULTS
Of the 25 patients treated with MMS, 19 primary tumors were included for outcome analysis, with an estimated 5-year recurrence-free survival rate of 91% (95% confidence interval [CI], 75-100) using Kaplan-Meier curve analysis. Of 158 patients treated with local excision, 124 were included for the analysis, with an estimated 5-year recurrence-free survival rate of 66% (95% CI, 56-78). The hazard ratio (HR) for association of treatment was 0.4 (95% CI, 0.10-1.65; p = .20). Estimated 5-year overall survival rates were 79% for MMS (95% CI, 61-100) and 68% for excision (95% CI, 59-78) (HR, 1.39 [95% CI, 0.69-2.82]; p = .36).


CONCLUSIONS
Although treatment of primary EMPD with MMS versus excision did not show statistical difference, MMS demonstrated favorable long-term outcomes and was associated with a higher recurrence-free survival rate.
*Mayo Medical School, Mayo Clinic College of Medicine, Rochester, Minnesota; †Department of Dermatology, Mayo Clinic, Rochester, Minnesota; ‡Division of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota.
Sharon J
SJ
Kim
Agnieszka K
AK
Thompson
Adeel S
AS
Zubair
Clark C
CC
Otley
Christopher J
CJ
Arpey
Christian L
CL
Baum
Randall K
RK
Roenigk
Christine M
CM
Lohse
Jerry D
JD
Brewer
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018572:Disease-Free Survival; D005260:Female; D005834:Genital Neoplasms, Male / Q000601:surgery; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D015580:Mohs Surgery; D009364:Neoplasm Recurrence, Local; D010145:Paget Disease, Extramammary / Q000401:mortality / Q000601:surgery*; D012189:Retrospective Studies; D012611:Scrotum / Q000601:surgery; D016896:Treatment Outcome; D014846:Vulvar Neoplasms / Q000601:surgery