The Role of Radiation Therapy for the Extramammary Paget's Disease of the Vulva ; Experience of 3 Cases

Abstract

W e have experienced three cases of extramammary Paget's disease (EMPD) of the vulva that received radiation therapy (RT).Here, we analyze the efficacy of RT and include a literature survey.Three patients with EM PD of the vulva were treated with curative RT between 1993 and 1998. One of the patients had associated underlying adenocarcinoma of the vulva. The total doses of radiation administered were 54～78 Gy/6～8 weeks. Radiation fields encompassed 2 to 3 cm outer margins free from all visible disease including or not including the inguinal area using a 9 MeV electron or a 6 MV photon beam. Follow-up durations after radiotherapy were 0.6～11 years. Complete response was obtained in all three patients. Marginal failure occurred in one patient, and another patient with underlying adenocarcinoma treated by vulvectomy with bilateral inguinal lymph node dissection followed by external RT showed no relapse. Radiation induced side effects were transient acute confluent wet desquamation in the treated area resulting in mild late atrophic skin changes.Although surgery is currently considered the preferred primary treatment for EMPD, it has a high relapse rate due to the multifocal nature of the disease. W e conclude that RT is of benefit in some selected cases of 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.…”

“…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.…”

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

“…Many patients who underwent radiotherapy achieved complete response [15,16,17,18,19,21,22,24,25,26,27,28,29]. Summarizing the data from 66 patients from the literature, the local progression-free survival at 5 years was 85%.…”

“…This data was collected by searching English-language journals included in the PubMed database and published between 1991 and 2012, using the keywords ‘EMPD' and ‘radiotherapy'. 15 available articles were identified [15,16,17,18,19,20,21,22,24,25,26,27,28,29,30]; however, 1 article [30] was excluded from this analysis because the applied radiotherapy methods were not described in detail. Therefore, 66 patients (21 male, 45 female; median age 73 years (range 38-92 years)) from 14 articles were analyzed (table 3).…”