Radiation therapy for extramammary Paget's disease: treatment outcomes and prognostic factors

Abstract

Background: Extramammary Paget's disease (EMPD) is a relatively rare malignancy, and there are few reports related to radiation therapy. In the present study, we investigated the outcome of radiation therapy for EMPD.Patients and methods: Forty-one patients with EMPD in the genitalia underwent radiation therapy with curative intent.Fifteen patients had regional lymph node metastases before radiation therapy, but none had distant metastasis. Total doses of 45-80.2 Gy (median, 60 Gy) were delivered to tumor sites in 23-43 fractions (median, 33 fractions).Results: At a median follow-up period of 41 months, 16 patients had developed recurrences, including 5 with local progression within the radiation field and 12 with lymph node or/and distant metastases outside the radiation field. The local progression-free and disease-free rates were 88% and 55% at 3 years, and 82% and 46% at 5 years, respectively. Nine patients died at 6-73 months after irradiation; the causes of death were tumor progression in five patients, infectious pneumonia in two, renal failure in one and old age in one. The overall and cause-specific survival rates were 93% and 96% at 3 years, and 68% and 84% at 5 years, respectively. Tumor invasion into the dermis and regional lymph node metastasis were significant prognostic factors for both distant metastasis and survival. No therapy-related toxicities of grade ≥3 were observed.Conclusions: Radiation therapy is safe and effective for patients with EMPD. It appeared to contribute to prolonged survival owing to good tumor control, and to be a promising curative treatment option.

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

“…12,19 Twelve studies failed to mention the used toxicity scale, 8Y11,13Y16,20Y22,25 whereas 5 studies graded toxicity by the Radiation Therapy Oncology Group/European Organization for Research and Treatment of Cancer (RTOG/ EORTC) scale. 17,18,23,24,26 In most of the reported studies, the RT treatment seems to be well tolerated. Cai et al 20 reported about 1 patient discontinuing RT because of toxicity, and Luk et al 13 reported a long suspension of RT (40 days) in 1 patient.…”

“…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.…”

“…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.…”

“…8,9,11Y14,18,19,21,23,24 Patients receiving adjuvant RT had a 12-, 20-, and 60-month LC of 100%, 0% to 100%, and 0% to 100%, respectively. 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.…”