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Table 1 Clinical outcome of Radiotherapy in selected studies

From: Primary extramammary invasive Paget’s vulvar disease: what is the standard, what are the challenges and what is the future for radiotherapy?

Author and year publication N Median follow up (mo) RT intent RT total dose (Gy) Toxicity DFS (mo) OS (mo) LC % (mo) Conclusions
Besa et al. 1992 [6] 2 12–66 1: Definitive 44–64 - - - - Dose greater than 50 Gy in who is medically unfit for surgery and for organ preservation could be indicated.
1: Post-operative
Luk et al. 2003 [7] 1 14–174 1: Post-operative 60 TB + 32 IN Acute: confluent wet desquamation, enteritis grade 2 10 15 100 (24) The results confirmed the useful role of radiotherapy in the management of extramammary Paget’s disease.
Late: ≤ grade 2 skin atrophy
Son et al. 2005 [8] 3 6-96 2: Definitive A) 55.8 1ary Acute: Dermatitis grade 2–3 A)12 A) - 100 A)(24) RT is of benefit in some selected cases of EMPD.
B)- B)- B)(6)
C)96 C)- C)(96)
Late: ≤ grade 2 skin atrophy
B) 81.6 1ary + 45.6 IN
1: Post-operative
C) 55.8 TB
Tanaka et al. 2009 [9] 2 18-84 2: Definitive 60 - A) 18 A)- 100 A)(18) EMPD is an uncommon neoplasm without any effective treatment.
B) 84 B)- B)(84)
Hata et al. 2011 [10] 12 8–133 4: Definitive 45–70.2 Gy (60) Acute: ≤ Grade 3 hematologic toxicity, dermatitis, cystitis, enteritis, urethritis 24 (100 %) 24 mo 100 % (2–9) RT is safe and effective for patients with EMPD. It appears to contribute to prolonged survival as a result of good tumor control.
8: Post-operative
Late: telangiectasia
Hata et al. 2012 [11] 7 18–150 7: Definitive 59.4–70.2 Acute: ≤ Grade 3 hematologic toxicity, dermatitis, cystitis, enteritis, urethritis 58 % (36) 92 % (36) 71 % (36) Radiation therapy is effective and safe, and appears to offer a curative treatment option for patients with EMPD.
46 % (60) 79 % (60) (60)
Late: ≤ Grade 3 telangiectasia
Cai et al. 2013 [2] 5 7–169 1: Pre-operative 57–60 Acute, Late: Acceptable ≤ Grade 3 - 70.8 mo (Invasive) 21.3 mo (associated with adnexal adenocarcinoma) - Intraepithelial EMPDV accounted for the majority of primary cases and had a better prognosis.
4: Post-operative
Surgical excision was the standard curative treatment for EMPDV. Radiotherapy was an alternative choice
for patients with medical contradiction or surgical difficulties. Postoperative radiotherapy could be considered
in cases with positive surgical margin or lymph node metastasis. Recurrence was common and repeated excision was often necessary.
Hata et al. 2014 [12] 14 2–174 10: Definitive 45–80.2 (60) Acute: ≤grade 2 hematologic toxicities, dermatitis, colitis, cystitis 54 % (36) 62 % (60) 88 % (36) 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.
46 % (60)
4: Post-operative
Late: ≤ Grade 3 telangiectasia
Itonaga et al. 2014 [15] 7 Median 71.4 2: Definitive 50 Acute, Late: Acceptable ≤ Grade 3 91.7 % (60) 84.3 % (60) 91.7 % (60) Radiotherapy yielded good local control and survival, which suggests that it was effective for patients with EMPD and in particular medically inoperable EMPD.
2: Post-operative
3: after surgical relapse
Hata et al. 2015 [16] 4 2–109 4: Post-operative 45–64.8 Acute: ≤ grade 2 dermatitis, grade 1 colitis and cystitis 92 % (36) 92 % (36) 100 % (38) Postoperative radiation therapy is safe and effective in maintaining local control in patients with EMPD.
71 % (60) 62 % (60)
Late: grade 1 telangiectasia
  1. Abbreviations: N number of patients, DFS Disease free survival, OS Overall survival, LC local control, 1ary Primary Disease, TB tumor bed, IN Inguinal Nodal Areas