Additional file 3 - Studies of photodynamic therapy (PDT) for Extramammary Paget's disease: lesion response Study Patient ID Age (years) Gender Prior treatment* Lesion location Type of PDT Number of treatments Response Length of followup** (months) Prospective case series*** 1 55 female 2 60 female 3 57 female Raspagliesi et al. (2006) surgical and laser excision surgical and laser excision laser excision surgical and laser excision surgical and laser excision surgical excision surgical excision surgical and laser excision surgical and laser excision laser excision laser excision vulva topical MAL-PDT 3 Complete Response 5 perianal topical MAL-PDT 3 No Response 5 vulva topical MAL-PDT 3 No Response 4 vulva topical MAL-PDT 3 Complete Response 4 perianal topical MAL-PDT 3 Complete Response 4 vulva perianal topical MAL-PDT topical MAL-PDT 3 3 Complete Response Complete Response 4 4 vulva topical MAL-PDT 3 Complete Response 2 vulva topical MAL-PDT 3 Complete Response 1 vulva axilla topical MAL-PDT topical MAL-PDT 3 3 Partial Response Complete Response 1 1 topical ALA-PDT 1st 4 treatments: topical ALA-PDT; last treatment: intravenous Porfimer sodium-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT 1 Partial Response 19 5 Complete Response 96 3 3 3 1 1 1 2 2 2 2 2 1 3 1 Partial Response Partial Response Partial Response Complete Response Complete Response Complete Response Complete Response Complete Response Complete Response Partial Response Partial Response Complete Response Partial Response Complete Response 20 20 20 9 9 9 6 6 6 6 6 88 4 47 1 Complete Response 68 4 67 female 5 59 female 6 69 female 7 75 female 8 75 male surgical excision axilla 9 50 male none groin 10 61 male 11 65 male 12 72 male pubis penis scrotum pubis buttock pubis scrotum scrotum scrotum scrotum scrotum penis penis penis 13 78 female laser excision laser excision laser excision laser excision laser excision laser excision laser excision laser excision none MMS MMS none none none surgical excision and topical ALAPDT Retrospective case reports/case series Housel et al. (2010) and Shieh et al. (2002) perianal intravenous Porfimer sodium-PDT 1 Study Patient ID 14 15 Thaler et al. (2010) Age (years) 80 52 Gender female female Prior treatment* Lesion location topical ALA-PDT perianal topical ALA-PDT perianal none perianal none buttock none perianal surgical and laser excision surgical and laser excision ? topical imiquimod none none none topical ALA-PDT topical ALA-PDT surgical excision surgical excision surgical excision surgical excision surgical excision surgical excision perianal perianal 16 17 18 19 20 21 22 69 68 76 73 67 81 84 male male male female female male female 23 66 female 24 76 female 25 83 female Li et al. (2009) 26 75 female none perianal Wang et al. (2008) 27 28 29 66 ? ? male ? ? cryotherapy ? ? penis/scrotum unspecified EM unspecified EM 31 ? male ? scrotum 32 ? male ? penis 33 ? male ? crissum 34 ? female ? pubis 35 ? female ? vulva Andretta-Tanaka et al. (2009) Fukui et al. (2009) Liu et al. (2007) perianal axilla scrotum vulva vulva penis vulva vulva perianal vulva extra-urethra vulva vulva Type of PDT intravenous Porfimer sodium-PDT intravenous Porfimer sodium-PDT intravenous PorfimerSodium PDT intravenous Porfimer sodium-PDT intravenous Porfimer sodium-PDT intravenous Porfimer sodium-PDT intravenous Porfimer sodium-PDT topical ALA-PDT topical MAL-PDT topical MAL-PDT topical MAL-PDT topical MAL-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT 1st 2 treatments: topical ALA-PDT; 3rd treatment: topical ALA-PDT + intravenous HpDa-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT intravenous Porfimer sodium-PDT intravenous Porfimer sodium-PDT intravenous Porfimer sodium-PDT intravenous Porfimer sodium-PDT intravenous Porfimer sodium-PDT Number of treatments Response Length of followup** (months) 1 Partial Response 48 1 Partial Response 48 1 Complete Response 12 1 Complete Response 12 1 Complete Response 12 1 Complete Response 62 1 Complete Response 62 4 8 3 3 3 6 9 9 9 ? ? ? ? Partial Response Partial Response Complete Response Partial Response Partial Response Complete Response Complete Response Complete Response Complete Response Complete Response Complete Response Complete Response Complete Response ? 12 6 6 6 18 17 15 15 12 12 3 3 3 Complete Response 7 3 ? ? Partial Response Partial Response Partial Response 12 12 12 3 ?b 3 3 ?b 3 3 ?b 3 3 ?b 3 3 ?b 3 2 Study T’Kint & Roseeuw (2006) Kim et al. (2005) Madan et al. (2005) Mikasa et al. (2005) Tulchinsky et al. (2004) Zawislak et al. (2004) Zhu et al. (2004) Patient ID Age (years) Gender Lesion location 36 64 female MMS MMS vulva perianal 37 54 male ? scrotum 38 69 male ? penis/scrotum 39 56 male ? scrotum 40 73 male ? penis/scrotum 41 58 female ? vulva 42 54 male ? penis/scrotum 43 50 male ? penis/scrotum 44 80 male none groin/scrotum 45 46 47 48 49 50 51 52 53 54 55 56 57 92 73 74 49 66 ? ? ? ? ? ? ? ? male female female female female ? ? ? ? ? ? ? ? 58 78 female 59 51 male 60 61 62 63 ? ? ? ? male male male male surgical excision none none surgical excision none ? ? ? ? ? ? ? ? none NR NR NR NR NR ? ? ? ? penis vulva perianal perianal vulva unspecified PD unspecified PD unspecified PD unspecified PD unspecified PD unspecified PD unspecified PD unspecified PD pubis vulva pubis penis penis scrotum unspecified EM unspecified EM unspecified EM unspecified EM Song et al. (2003) Xu et al. (2002) Prior treatment* Type of PDT topical MAL-PDT topical MAL-PDT combined PDT and surgical excisionc combined PDT and surgical excisionc combined PDT and surgical excisionc combined PDT and surgical excisionc combined PDT and surgical excisionc combined PDT and surgical excisionc combined PDT and surgical excisionc 1st 5 and last treatments: topical ALA-PDT; 6th treatment: intravenous Porfimer sodium-PDT topical ALA-PDT topical ALA-PDT ? ? topical ALA-PDT intravenous HpDa-PDT intravenous HpDa-PDT intravenous HpDa-PDT intravenous HpDa-PDT intravenous HpDa-PDT intravenous HpDa-PDT intravenous HpDa-PDT intravenous HpDa-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT Number of treatments Response 4 4 Complete Response Complete Response Length of followup** (months) 14 14 ? Complete Response ? ? Complete Response ? ? Complete Response ? ? Complete Response ? ? Complete Response ? ? Complete Response ? ? Complete Response ? 7 Complete Response 12 2 5 1 2 4 1 1 2 2 2 2 2 2 4 4 4 4 4 4 ? ? ? ? Complete Response Complete Response Partial Response Partial Response Complete Response Complete Response Complete Response Complete Response Complete Response Complete Response Complete Response Complete Response Complete Response Complete Response Complete Response Complete Response Partial Response Complete Response Complete Response Complete Response Complete Response Complete Response Complete Response 2 2 12 ? 3 ? ? ? ? ? ? ? ? 12 12 12 12 12 12 ? ? ? ? 3 Study Patient ID 64 65 66 67 Age (years) ? ? ? ? Gender Prior treatment* Lesion location male male male male ? ? ? ? unspecified EM unspecified EM unspecified EM unspecified EM 70 68 male ? penis/scrotum 71 56 male ? scrotum 72 54 male ? penis/scrotum 73 73 male ? penis/scrotum 74 53 male ? scrotum ? L vulva ? R vulva Chang et al. (2001) 75 58 female 76 50 male ? penis/scrotum Runfola et al. (2000) 77 79 male surgical excision perianal Henta et al. (1999) 78 74 female electron beam irradiation and chemotherapy vulva Wang et al. (1991) 79 80 81 82 ? ? ? ? ? ? ? ? none none none none unspecified EM unspecified EM unspecified EM unspecified EM Type of PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT topical ALA-PDT combined topical ALAPDT, intralesional injection HpDa-PDT and intravenous HpDa-PDT combined topical ALAPDT, intralesional injection HpDa-PDT and intravenous HpDa-PDT combined topical ALAPDT, intralesional injection HpDa-PDT and intravenous HpDa-PDT combined topical ALAPDT, intralesional injection HpDa-PDT and intravenous HpDa-PDT combined topical ALAPDT, intralesional injection HpDa-PDT and intravenous HpDa-PDT combined topical ALAPDT, intralesional injection HpDa-PDT and intravenous HpDa-PDT combined topical ALAPDT, intralesional injection HpDa-PDT and intravenous HpDa-PDT combined topical ALAPDT, intralesional injection HpDa-PDT and intravenous HpDa-PDT intravenous Porfimer sodium-PDT 1st treatment: topical ALAPDT; 2nd treatment: intralesional injection ALAPDT intravenous HpDa-PDT intravenous HpDa-PDT intravenous HpDa-PDT intravenous HpDa-PDT Number of treatments Response Length of followup** (months) ? ? ? ? Partial Response Partial Response Partial Response Partial Response ? ? ? ? ? Partial Response 18 ? Partial Response 12 ? Partial Response 24 ? Partial Response 18 ? Partial Response 22 ? Partial Response 18 ? Partial Response 18 ? Partial Response 20 1 Partial Response 48 10 Complete Response ? ? ? ? ? Complete Response Complete Response Partial Response Partial Response ? ? ? ? 4 Study Kubota et al. (1986) Patient ID 83 84 85 Age (years) ? ? ? Gender Prior treatment* Lesion location ? ? ? none none none unspecified PD unspecified PD unspecified PD 86 77 female ? vulva Type of PDT intravenous HpDa-PDT intravenous HpDa-PDT intravenous HpDa-PDT intravenous Porfimer sodium-PDT Number of treatments Response Length of followup** (months) ? ? ? Complete Response Complete Response Partial Response ? ? ? ? No Response 6 * history of prior treatment for Paget’s disease ** length of follow-up after last treatment *** an additional prospective case series study was included in the review (Li et al. (2010)) but not included in this table as outcomes were not reported by patient a unspecified hematoporphyrin derivative (HpD) used as photosensitizing agent b Liu et al. (2007) did not report outcome data by patient; overall outcomes: 1/5 lesions complete response, 3/5 partial response, 1/5 minimal response c order of treatment not reported (i.e. whether surgery or PDT was performed first) ? = not specified, ALA = 5-aminolevulinic acid, MAL = methyl aminolevulinate, MMS = Mohs Micrographic surgery, HpD = hematoporphyrin derivative 5