Radiotherapy in Patients with Extramammary Paget's Disease - Our Own Experience and Review of the Literature

Abstract

Background: There are few reports on radiotherapy methods for treating extramammary Paget's disease (EMPD). The aim of this study was to explore the outcome of radiotherapy for EMPD. Patients and Methods: Between June 1995 and October 2010, 14 patients with EMPD (7 male and 7 female; median age 77 years) underwent radiotherapy at the Tokyo Medical University Hospital. The median total irradiation dose was 50 Gy, delivered in 20-33 fractions. Case reports of patients with EMPD were obtained from the PubMed database for the period of 1991-2012. 66 patients (median age 73 years) were identified. Radiotherapy outcomes were analyzed. Results: All patients achieved complete response within the irradiated volume during a median observation period of 71.4 months. The 5-year locoregional progression-free survival and overall survival were 91.7% (95% confidence interval (CI) 53.9-98.8%) and 84.3% (95% CI 50.3-95.8%), respectively. From the PubMed database, the 5-year local progression-free survival and locoregional progression-free survival were 84.5% (95% CI 65.3-93.6%) and 77.5% (95% CI 57.3-89.0%), respectively. 12 (18%) patients had a recurrence in the inguinal lymph nodes. Conclusion: Radiotherapy yielded good local control and survival, which suggests that it was effective for patients with EMPD and in particular medically inoperable EMPD.

The Role of Radiotherapy in Extramammary Paget Disease: A Systematic Review

“…8Y25 Doses ranged from 30 to 80.2 Gy delivered in 3 to 43 fractions by means of photons or electrons. A sporadic use of concomitant chemoradiation therapy to treat the EMPD and the concurrent underlying anal cancer was reported by Brown et al 12 Radiotherapy was administered as postoperative adjuvant treatment in 9 articles accounting for 63 (32.3%) patients 9,13,15,17,19,20,22,23,26 with doses ranging between 32 and 64.8 Gy in 20 to 30 fractions delivered by means of photons or electrons.…”

“…9,13,15,17,19,22,23,26 It was not always reported if the relapsing disease was in an in situ or invasive form.…”

“…Concerning the correlation between dose and CR, studies that used a low RT dose (maximum dose of e60 Gy) 10,12,13,16,22,25 showed a CR ranging between 50% and 100% with only 2 studies reaching 100% of CR; whereas the maximum dose administered was greater than 60 Gy, 8,9,15,17Y20,23,24 all but 1 studies reported 100% of CR. Despite an RT dose of 60 Gy or greater seems to be adequate for the treatment of the gross tumor volume, relapses were reported within the radiation field.…”

“…Despite an RT dose of 60 Gy or greater seems to be adequate for the treatment of the gross tumor volume, relapses were reported within the radiation field. 13,17,18,22,23 9 2 vulva, 4 anus, 1 scrotum,1 perineum 8 4 D 3 AD 1 NAD (patient affected by a synchronous rectal adenocarcinoma) Burrows, 1995 10 1 scrotum, 1 vulvar,1 groin, 2 sovrapubic skin 5 D Moreno-Arias, 2001 11 1 scrotum, 1 penis 2 D for in situ EPMD Brown, 2002 12 6 perianal skin 6 5 D 1 P (due to a concomitant anal adenocarcinoma) Luk, 2003 13 4 scrotum, 18 7 vulva, 5 perineum, 2 scrotum 14 D Ito, 2012 19 1 penis, 1 vulva, 1 perineum, 3 lymph node metastasis 6 3 D 1 AD 2 P Cai, 2013 20 Vulva 43 (17 RT) 9 D 6 AD 1 NAD 1 P Carrozzo, 2014 21 4 vulva or perivulvar, 1 glans 5 D Itonaga, 2014 22 7 vulva, 6 scrotum, 1 perianal 14 9 D 5 AD Hata, 2014 23 14 vulva, 14 perineal, 11 scrotum, 2 penis 41 24 D 17 AD Hata, 2014 24 5 scrotum, 1 penis, 1 vulva, 1 perineum 8 D Tackenberg, 2015 25 3 inguinal, 2 penis, 2 perianal region 7 D Hata, 2015 26 7 perineum, 4 vulva, 9 scrotum, 1 penis 21 AD: margin close (5) or positive (16) AD, adjuvant; D, definitive for primary disease or for recurrence after a primary treatment; NAD, neoadjuvant; P, palliative; Ref., reference. Local control data were not reported in 6 studies.…”

“…9,13,15,19,23,26 Disease-free survival data were lacking in 7 studies. 10,16,17,20,22,25,26 Disease-free survival data were different according to clinical setting and time. Patients receiving RT as definitive treatment for primary or recurrent disease after surgery had a 12-, 20-, and 60-month DFS of 66% to 100%, 33% to 100%, and 20% to 63%, respectively.…”