Role of radiotherapy as curative treatment of extramammary Paget's disease.
OBJECTIVE
Extramammary Paget's disease (EMPD) is a relatively rare malignancy that usually arises in the genital areas. Wide surgical excision remains the standard and most reliable curative treatment of EMPD. However, surgery is sometimes not possible, because many patients are elderly, and complete excision can be difficult owing to the tumor location. We, therefore, performed a review to determine the role of radiotherapy (RT) for EMPD.


METHODS
A total of 22 patients with EMPD in their external genitalia (4 men and 18 women, age 52-94 years at RT) underwent RT with curative intent. Nine patients had regional lymph node metastases. A total dose of 45-70.2 Gy (median, 60) was delivered to the pelvis, including the tumors, in 25-39 fractions (median, 33).


RESULTS
In all but 3 patients, the irradiated tumors were controlled during a follow-up period of 8-133 months (median, 42). Of the 22 patients, 13 developed recurrences, including local progression within the radiation field in 3 and lymph node and/or distant metastases outside the radiation field in 10, at 3-43 months after treatment. The 2- and 5-year local progression-free rates were 91% and 84%, respectively. Of the 22 patients, 7 patients had died at 33-73 months after RT. The cause of death was tumor progression in 4, infectious pneumonia in 2, and renal failure in 1 patient. The overall and cause-specific survival rates were 100% for both at 2 years and 53% and 73% at 5 years, respectively. No therapy-related Grade 3 or greater toxicity was observed.


CONCLUSIONS
RT is safe and effective for patients with EMPD. It appears to contribute to prolonged survival as a result of good tumor control.
Department of Radiology, Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa, Japan. mhata@syd.odn.ne.jp
Masaharu
M
Hata
Motoko
M
Omura
Izumi
I
Koike
Hidefumi
H
Wada
Etsuko
E
Miyagi
Yoshibumi
Y
Tayama
Kazumasa
K
Odagiri
Yumiko
Y
Minagawa
Ichiro
I
Ogino
Tomio
T
Inoue
D000368:Aged; D000369:Aged, 80 and over; D018572:Disease-Free Survival; D019583:Dose Fractionation, Radiation; D005260:Female; D005833:Genital Neoplasms, Female / Q000473:pathology / Q000532:radiotherapy*; D005834:Genital Neoplasms, Male / Q000473:pathology / Q000532:radiotherapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000401:mortality / Q000532:radiotherapy; D010145:Paget Disease, Extramammary / Q000401:mortality / Q000532:radiotherapy*; D012189:Retrospective Studies