Vulvar Paget’s Disease: A Systematic Review of the MITO Rare Cancer Group
Abstract
:Simple Summary
Abstract
1. Introduction
2. Materials and Methods
2.1. Search Strategy
2.2. Study Selection
2.3. Data Extraction and Analysis
2.4. Statistical Analysis
3. Results
3.1. Study Selection
3.2. Study and Population Characteristics
3.3. Treatment Approches
3.4. Outcomes
4. Discussion
5. Conclusions
Author Contributions
Funding
Acknowledgments
Conflicts of Interest
References
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Reference | Type of Study | Sample Size, n | Mean Age (Years, Range) | Symptoms (%) | Signs (%) | Tumor Invasion |
---|---|---|---|---|---|---|
Kodama et al. 1995 [16] | Cs | 30 | 67.6 (41–82) | Vulvar itching (100%) | Erythema (63%) | 10 ni-VPD (33.3%); 9 mi-VPD (30%); 11 i-VPD (36.7%) |
Fishman et al. 1995 [17] | Cs | 14 | 70.5 (57–83) | Vulvar itching (100%) | NR | NR |
Yoshitatsu et al. 1997 [18] | Cr | 1 | 68 | Vulvar itching | Vulvar erythema with a nodule on the left labium major | ni-VPD |
Goldblum et al. 1997 [19] | Cs | 19 | 65 (56–86) | NR | NR | 5 i-VPD (26.3%) |
Fanning et al. 1999 [20] | Ra | 100 | 70 (35–100) | Vulvar itching (NR) | NR | 7 mi-VPD (7%); 5 i-VPD (5%) |
Henta et al. 1999 [21] | Cr | 1 | 74 | Pain | Irregular border dark red mass | ni-VPD |
Murata et al. 1999 [22] | Cs | 1 (extracted from a total of 6 pts) | 78 | NR | Erythema | i-VPD |
Louis-Sylvestre et al. 2001 [23] | Ra | 52 | 67.4 (39.6–94.7) | NR | NR | NR |
Wilkinson et al. 2002 [6] | Cs | 3 | 76 (76–81) | Vulvar inflammatory lesion (66%); urinary urgency (33%) | Erythematous and eczematoid lesion (66%); periurethral induration and scar tissue surrounding the urethral meatus (33%) | NR |
Tebes et al. 2002 [24] | Cs | 23 | 69(46–84) | Itching (78%), burning sensation (52%), dysuria (4%), watery discharge (4%) | Vulvar lesion (87%) | 6 i-VPD (26.1%) |
Luk et al. 2003 [25] | Cs | 1 (extracted from a total of 6 pts) | 55 | NR | NR | NR |
Wang et al. 2003 [26] | Cr | 1 | 75 | Itching and burning | Hypopigmented to pink plaque in labium major | NR |
Chin et al. 2004 [27] | Cr | 1 | 65 | NR | NR | NR |
Zawislak et al. 2004 [28] | Cr | 1 | 66 | 8 years of persistent vulvar itching | Purple-red, well-demarcated, moist lesion on the left labia majora | NR |
Bhattacharya et al. 2005 [29] | Cr | 1 | 59 | vulvar irritation | NR | ni-VPD |
Raspagliesi et al. 2006 [30] | Prospective pilot study | 7 | 63 (50–75) | Severe vulvar itching (NR) | NR | NR |
Yanagi et al. 2007 [31] | Cr | 2 | 84 (82–86) | NR | Infiltrated reddish plaque on the genital region | NR |
Hatch et al. 2008 [32] | Cs | 2 | 64 (60–68) | Pain (100%), urinary retention (50%) | Vulvar erythema | NR |
Karam et al. 2008 [33] | Cr | 1 | 34 | NR | NR | NR |
Challenor et al. 2009 [34] | Cs | 2 | 57 (48–66) | Itching (100%), soreness (50%) | Vulvar erythema | NR |
Sendagorta et al. 2010 [35] | Cs | 3 | 66 (58–82) | Itching (66.6%), burning (33.3%) | Vulvar lichenified plaque (33.3%), vulvar erythematous plaque (66.6%), vulvar erosions (1–33.3%) | NR |
Shaco-Levy et al. 2010 [36] | Cs | 56 | 69 (42–89) | Vulvar itching (91%), vulvar pain (11%), drainage (5%), bleeding (2%) | Erythematous-white plaque (98%) | 0.2–6 mm |
Roh et al. 2010 [37] | Cs | 11 | 66.6 | Pruritis | NR | NR |
Anton et al. 2011 [38] | Cr | 1 | 84 | Itching | Hyper-pigmented to the pink plaque from the pubic area to the left great labia | NR |
Feldmeyer et al. 2011 [39] | Cr | 1 | 59 | Fluctuating soreness and itching | Poorly delimited erythematous–squamous patch on the right labia major | NR |
Hanawa et al. 2011 [40] | Cr | 1 | 70 | Asymptomatic | Increasing vulvar erythema, slightly raised erythematous lesion with ulcers, hemorrhagic granulomatous lesion | NR |
Jones et al. 2011 [41] | Ra | 50 | 67.6 | Itching (54%) | NR | NR |
Tonguc et al. 2011 [42] | Cr | 1 | 65 | Itching | Hyperkeratotic erythematous lesion from right labium to the clitoris | NR |
Mendivil et al. 2012 [43] | Cs | 16 | 75 (53–84) | Pruritis (100%) | NR | 3 i-VPD |
Al Yousef et al. 2012 [44] | Cr | 1 | 78 | Pruritis | Erythema | NR |
Baiocchi et al. 2012 [45] | Cs | 4 | 62.2 (56–80) | Itching (100%) | Vulvar erythematous plaque (100%), vulvar erosions (100%) | NR |
Wakabayashi et al. 2012 [46] | Cr | 1 | 68 | NR | NR | NR |
Cai et al. 2013 [47] | Ra | 43 | 68.6 (52–85) | Itching (95.3%), vulvar pain (18.6%), bleeding (16.3%), and discharge (13.9%) | Erythematous lesions (81.4%), ulceration (32.6%), erosion (30.2%) | NR |
Choi et al. 2013 [48] | Cs | 3 (extracted from a total of 10 pts) | 73 (65–81) | NR | Vulvar erythema | NR |
Gavriilidis et al. 2013 [49] | Cr | 1 | 75 | Rash and vulvar itching | Eczematoid and erythematous area of the genitalia | NR |
Sanderson et al. 2013 [50] | Cs | 6 | 71.5 (58–85) | Soreness (50%), itching (50%), irritation (16,6%), inflammation (16.6%) pain (16.6%) | Plaque | NR |
De Magnis et al. 2013 [51] | Ra | 34 | 68.7 | Itching (76.5%), burning (58.8%) | NR | i-VPD: 11.7% |
Treglia et al. 2013 [52] | Cr | 1 | 61 | NR | NR | NR |
Magnano et al. 2013 [53] | Cr | 1 | 84 | Slight pruritus | Erythematous ulcerated and well-demarcated oval plaque (5 × 3 cm) | ni-VPD |
Marchitelli et al. 2014 [54] | Cs | 10 | 71.9 (60–92) | NR | Erythematous plaque | NR |
Liu et al. 2014 [55] | Ra | 85 | 64.4 (33–82) | Pruritis (74.1%), pain (5.9%) | NR | i-VPD: 20% |
Luyten et al. 2014 [56] | Ra | 20 | 66.4 (41–84) | NR | NR | NR |
Carrozzo et al. 2014 [57] | Cs | 4 (based on a total of 5 pts) | 69 (59–79) | NR |
| NR |
Frances et al. 2014 [58] | Cr | 1 | 80 | NR | Itching eczematous plaques | ni-VPD |
Hata et al., et a. 2015 [59] | Ra | 21 | 69 (53–88) | NR | NR | NR |
Asaka et al. 2015 [60] | Cr | 1 | 80 | Vulvar itching, pain | Pigmented vulvar, perianal erythematous plague, and a subcutaneous nodule in the left major labia | Intradermal invasion |
Sopracordevole et al. 2016 [61] | Ra | 27 | 66.5 (36–88) | Itching (64.3%), burning + itching (14.3%), pain (14.3%), burning (7.2%) | Erythema (93.7%), hyperkeratosis (31.6%) | 8 mi-VPD (29.6%) 3 i-VPD (11.1%) |
Cowan et al. 2016 [62] | Prospective pilot study | 8 | 71.5 (47–78) | Itching (63%), burning (25%), pain (25%) | Visible lesions (100%), erythema (75%), anal involvement (13%) | NR |
Fan et al. 2016 [63] | Ra | 18 | 65 | Pruritus, erosions, burning sensation, pain | Erythematous patches (50%), hypopigmentation (50%), hyperpigmentation (27.8%) | NR |
Nagai et al. 2016 [64] | Cs | 2 | 75 (69–81) | Itching | Eczema | NR |
Onaiwu et al. 2016 [65] | Ra | 89 | 67(32–89) | Pruritus (48.3%) | NR | 7 i-VPD (7.9%) |
Hillmann et al. 2016 [66] | Cr | 1 | 48 | Itching | Acetowhite areas in left minora and majora labia, perineal, and anterior perianal regions | NR |
Liau et al. 2016 [67] | Ra | 3 | 74.7 (66–83) | Pruritus, pain | Erythematous patch or plaque | NR |
Jeon et al. 2016 [68] | Cr | 1 | 71 | Pruritus, pain | Erythematous plaque on both labia majora | ni-VPD |
Dogan et al. 2017 [69] | Cr | 1 | 73 | Itching | Left vulvar erythema | Intradermal pagetoid cells |
Vicentini et al. 2017 [70] | Cr | 1 | 62 | Itching, pain, discomfort | Erythematous plaque | ni-VPD |
Parashurama et al. 2017 [71] | Cs | 18 | 76.9 | Vulvar itching, soreness | Ulcer, mass, pigmented lesion | NR |
Konstantinova et al. 2017 [72] | Cs | 181 | 65 | NR | NR | NR |
Mota et al. 2017 [73] | Cr | 1 | 78 | Pruritus | Cutaneous plaque with eczema | NR |
Long et al. 2017 [74] | Cs | 90 | 70.3 | NR | NR | NR |
Kato et al. 2018 [75] | Cr | 2 | 72.5 (64–81) | Nodules and skin infiltrations | Both inguinal lymph nodes | ni-VPD |
Borghi et al. 2018 [76] | Ra | 79 | 67.5 (53–77) | NR | NR | mi-VPD/i-VPD: 12.7% |
Sawada et al. 2018 [77] | Ra | 9 | 82 (61–94) | NR | NR | ni-VPD |
Hiratsuka et al. 2018 [78] | Cs | 1 (extracted from a total of 4 pts) | 69 | NR | NR | i-VPD |
Hsieh et al. 2018 [79] | Cr | 1 | 54 | NR | NR | NR |
Nitecki et al. 2018 [80] | Cs | 44 | 67 | Pain (10–22%), pruritis (10–22%), pain + pruritis (6–13%) | NR | i-VPD: 20% |
Rioli et al. 2018 [81] | Ra | 13 | 70 (52–84) | NR | NR | 3% |
Kato et al. 2018 [82] | Ra | 17 (7 women) | 76.9 | NR | NR | i-VPD |
Cai et al. 2018 [83] | Cs | 8 | 64.4 | NR | NR | NR |
Bouceiro-Mendes et al. 2019 [84] | Cr | 1 | 72 | Vulvar itching | Erythematous plaque | NR |
Loiacono et al. 2019 [85] | Cs | 24 | 69.3 (38–84) | Itching + burning + pruritus + vulvar lesions (21%), pain + pruritus (13%), vulvar itching (4%), pruritus (4%), unknown (58%) | NR | NR |
Mujukian et al. 2019 [86] | Cr | 1 | 84 | NR | Large erythematous plaque affecting the right labia majora | NR |
Molina et al. 2019 [87] | Cs | 3 | 50 (50–70) | Fatigue, weakness, pruritus, fever | Erythematous plaque, scattered, pink-red plaques | NR |
Hirai et al. 2019 [88] | Cs | 5 | 65 (53–74) | NR | NR | NR |
van der Linden et al. 2019 [89] | Ra | 113 | 73 (41–97) | NR | NR | ni-VPD: 77% mi-VPD: 8.8% i-VPD: 14.2% |
Panoskaltsis et al. 2019 [90] | Cr | 1 | 75 | Itching, irritation, and burning | Erythematous plaque with white scaling | i-VPD |
Nasioudis et al. 2020 [91] | Ra | 2602 | 72 (31–90) | NR | NR | i-VPD: 61.8% |
Bartoletti et al. 2020 [92] | Cs | 4 | 62.5 (45–74) | Vulvar itching | NR | NR |
Bruce et al. 2020 [93] | Cr | 1 | 37 | Vulvar irritation at 7 weeks gestation with an erythematous, edematous left labia | NR | NR |
Noel et al. 2020 [94] | Cr | 1 | 65 | Vulvar itching | Vulvar reddish lesion | NR |
Kilts et al. 2020 [95] | Ra | 1268 | 73 (29–100) | NR | NR | NR |
Rathore et al. 2020 [96] | Cr | 1 | 59 | Vulvar itching | Vulvar redness, erythematous indurated plaque with multiple papules and nodules | NR |
Sarkar et al. 2020 [97] | Cr | 1 | 51 | Vulvar itching for 3 years | Erythematous patchy lesion | NR |
Sopracordevole et al. 2020 [98] | Ra | 10 | 65.5 (40–80) | Vulvar itching | Red asymmetrical areas, ulcerations, hyperkeratotic red areas, whitish areas | mi-VPD |
Stasenko et al. 2020 [99] | Ra | 26 | 62 (29–77) | NR | NR | mi-VPD |
Liang et al. 2021 [100] | Cs | 38 | 64.4 (39–79) | Vulvar itching and pain | NR | NR |
Hirata et al. 2021 [101] | Cr | 1 | 55 | Recurrent fever for 10 months, inguinal and intraperitoneal lymphadenopathies | NR | NR |
Kosmidis et al. 2021 [102] | Cr | 2 | 75 (69–81) | Vulvar itching and burning sensation; perineal and vulvar itching and swelling | Erythematous plaque Eczematous plaque + erosion | ni-VPD |
Mazzilli et al. 2021 [103] | Cr | 1 | 60 | NR | NR | NR |
Preti et al. 2021 [104] | Ra | 122 | 65 (36–92) | Itching (59–61%), burning sensation (18%), itching + burning (20–21%) | NR | mi-VPD: 21% i-VPD: 16.8% |
Liu et al. 2021 [105] | Ra | 54 | 72 ± 7 (SD) | NR | NR | NR |
Ferrara et al. 2021 [106] | Prospective study | 10 | 79 (67–92) | NR | NR | NR |
Bajracharya et al. 2022 [107] | Cr | 1 | 70 | Vulvar itching and a gradually progressive reddish lesion | NR | NR |
Wang et al. 2022 [108] | Prospective pilot study | 2 (extracted from a total of 11 pts) | 73 (67–79) | NR | NR | NR |
Borella et al. 2022 [109] | Ra | 55 | 63 (36–92) | Itching (29–59%), burning (15–27%) | NR | NR |
Van der Linden et al. 2022 [110] | Prospective study | 24 | 67 (42–84) | Itching (83%), pain (45.8%), burning sensation (70.8%), strangury (29.2%), dyspareunia (38.5%) | Erythema (100%), scaling (62.5%), ulceration (25%) | NR |
Reference | Surgery | Radiotherapy | Systemic Chemotherapy | Other Therapy | ||||||
---|---|---|---|---|---|---|---|---|---|---|
N | Technique Margin Status | Complications | N | Aim Total Dose/Fr Technique Volumes | Toxicity | N | Aim Scheme | Toxicity | N | |
Kodama et al. 1995 [16] | 29 | ni-VPD: RV + bilateral LA + skin graft (30%), RV + bilateral LA (20%), SV (30%), SV + skin graft (10%), RV + bilateral LA + skin graft + perineal resection and artificial anus (10%) Margin status: R+ in 4 cases (40%) mi-VPD: RV + bilateral LA + skin graft (44.4%), SV (33.3%), RV + bilateral LA (22.2%) Margin status: R+ in 5 cases (55.6%) i-VPD: RV + bilateral LA + skin graft (27.3%), RV + bilateral LA (27.3%), RV + bilateral LA + skin graft + total hysterectomy (9%), exploratory laparotomy (9%), resection (9%), RV + bilateral LA + skin graft + perineal resection and artificial anus (9%) Margin status: R+ in 4 cases (36.4%) | NR | 3 | NR | NR | 11 | Aim: palliative Scheme: bleomycin, mitomycin, FCAP (5-FU, cyclophosphamide doxorubicin, cisplatinum) | NR | NR |
Fishman et al. 1995 [17] | 14 | Primary surgery: WLE (57.1%), SV (21.4%), RV (21.4%) Recurrent surgery: WLE (78.6%), SV (7.1%), WLE + bilateral LA + rectus abdominal myocutaneous reconstruction (7.1%) Margin status: R+ in 5 cases (35.7%) | NR | 1 | Aim: adjuvant Total dose/Fr: NR Volumes: vulvar, pelvic and groin RT | NR | 0 | - | - | 0 |
Yoshitatsu et al. 1997 [18] | 1 | Lesion resection + LA + gracilis musculocutaneous flap → WLE + split-thickness skin graft Margin status: NR | NR | 0 | - | - | 1 | Aim: adjuvant Scheme: mitomycin C and Tegafur | NR | 0 |
Goldblum et al. 1997 [19] | 19 | ni-VPD: SV (10), RV (2), RV + LA (1) Margin status: NR i-VPD: RV (3), SV (2), RV + LA (1) Margin status: R+ | NR | 0 | - | - | 0 | - | - | 0 |
Fanning et al. 1999 [20] | 100 | WLE (32%) RV (58%) HV (10%) Margin status: NR | NR | 0 | - | - | 0 | - | - | 0 |
Henta et al. 1999 [21] | 0 | - | - | 1 | Aim: palliative, electron beam irradiation Total dose/Fr: 5000 cGy Volumes: vulvar lesion | NR | 1 | Aim: palliative Scheme: etoposide (VP16) 100 mg for 5 days concomitant with RT | NR | 1 (photodynamic therapy with aminolevulinic acid) |
Murata et al. 1999 [22] | 0 | - | - | 1 | NR | NR | 0 | - | - | 0 |
Louis-Sylvestre et al. 2001 [23] | 31 | WLE (67.7%) Vu (22.6%) HV (9.7%) Margin status: R+ in 6 cases of Vu | NR | 0 | - | - | 0 | - | - | 21 |
Wilkinson et al. 2002 [6] | 3 | Case 1: RV Case 2: RV, with excision to the fascia and partial vaginal excision, including excision of the hymen Case 3: 2 subsequent partial Vus ➝ periurethral scar tissue excision Margin status: Case 1: R0 Case 2: R0 Case 3: NR | NR | 0 | - | - | 0 | - | - | 0 |
Tebes et al. 2002 [24] | 23 | WLE o PV (73.9%) Radical resection + LA (26.1%) Margin status: R+ in 13 cases (50%) | NR | 0 | - | - | 0 | - | - | 0 |
Luk et al. 2003 [25] | 1 | RV + LA Margin status: NR | NR | 1 | Aim: adjuvant Total dose/Fr: 60 Gy⁄30 fr⁄6 weeks to primary site and right inguinal nodal site 32 Gy⁄8 fr⁄2 weeks at 90% isodose level for prophylactic left inguinal nodal RT Volumes: anteroposterior opposed photon fields to pelvis and vulva, then electron boost to right vulva, matched 10 MeV electron fields to bilateral inguinal nodal sites | Confluent wet desquamation, resolved at 4 weeks post-RT, mild acute small bowel irritation | 0 | - | - | 0 |
Wang et al. 2003 [26] | 1 | WLE Margin status: NR | NR | 0 | - | - | 0 | - | - | 1 |
Chin et al. 2004 [27] | 1 | SV → WLE + rectus abdominal flap Margin status: NR | NR | 0 | - | - | 0 | - | - | 0 |
Zawislak et al. 2004 [28] | 0 | - | - | 0 | - | - | 0 | - | - | 1 (photodynamic therapy) |
Bhattacharya et al. 2005 [29] | 1 | PV → 4 excisions → hysterectomy + salpingo-oophorectomy + LA + SV + partial vaginectomy Margin status: NR | NR | 0 | - | - | 0 | - | - | 0 |
Raspagliesi et al. 2006 [30] | 5 | Vu or WLE Margin status: R+ | NR | 0 | - | - | 0 | - | - | 2 |
Yanagi et al. 2007 [31] | 0 | - | - | 2 | Aim: radical Total dose/Fr: electron beam of 4 MeV, 2.25 Gy per day, 4 days/week to a total dose of 45 Gy. Volumes: a 10 mm margin surrounding the lesion Aim: radical Total dose/Fr: X-rays of 4 MV, 2.5 Gy per day, 4 days/week to a total dose of 45 Gy + electron beam therapy (4 MeV, 3.0 Gy per day, for 5 fractions to a total dose of 15 Gy) Volumes: thick lesions on both sides of the genitals | mild acute mucositis (2) | 0 | - | - | 0 |
Hatch et al. 2008 [32] | 2 | Local resection (1), RV (1) Margin status: NR | NR | 0 | - | - | 0 | - | - | 2 |
Karam et al. 2008 [33] | 1 | Surgical excisions of the vulva and perineum (8 times) Margin status: NR | NR | - | - | - | 1 | Aim: palliative Scheme: 3-w-TZB (14 cycles) | None | 0 |
Challenor et al. 2009 [34] | 2 | Skinning Vu and reconstruction with split skin graft (1), skinning Vu with reconstruction via V-Y advancement flaps (1) Margin status: R+ | NR | 0 | - | - | 0 | - | - | 2 |
Sendagorta et al. 2010 [35] | 1 | Skinning posterior vulvectomy with reconstruction (1) | NR | 0 | - | - | 0 | - | - | 3 |
Shaco-Levy et al. 2010 [36] | 56 | Conservative procedures: WLE, SV, PV. Radical procedures: complete Vus and RVs Margin status: R1 in 44% | Skin flap/graft breakdown and necrosis (6); wound infection (2); hypertrophic scar (2); thigh cellulitis (1) | 6 | Aim: adjuvant Details on RT available only for 1 case Total dose/Fr: 2340 cGy to the groins, pelvis, and anus, with a 4500 cGy boost to the groins and anus and 6100 cGy electron boost to the peri-anal region | NR | 0 | - | - | 0 |
Roh et al. 2010 [37] | 11 | WLE: 63.6% RV: 18.1% SV: 18.1% Margin status: R+ in 72.7% | - | 0 | . | . | 0 | - | 0 | - |
Anton et al. 2011 [38] | 0 | - | - | 0 | - | - | 0 | - | - | 1 |
Feldmeyer et al. 2011 [39] | 0 | - | - | 0 | - | - | 0 | - | - | 1 |
Hanawa et al. 2011 [40] | 0 | - | - | - | Aim: palliative Total dose/Fr: NR Technique: NR Volumes: NR | NR | - | Aim: palliative Scheme: w-TZB for 5 weeks ➝ w-paclitaxel plus w-TZB 6 cycles | NR | 0 |
Jones et al. 2011 [41] | 50 | WLE: 24% HV: 28% RV: 28% Anterior vulvectomy: 14% Skinning vulvectomy: 2% SV: 2% Margin status: R+ in 54% | - | 0 | - | - | 2 | NR | - | 0 |
Tonguc et al. 2011 [42] | 1 | WLE → WLE Margin status: R+ → R0 | NR | 0 | - | - | 0 | - | - | 1 |
Mendivil et al. 2012 [43] | 16 | SV: 62.5% RV: 12.5 % Only biopsy: 12.5% Margin status: R+ in 68.8% | NR | 0 | - | - | 0 | - | - | - |
Al Yousef et al. 2012 [44] | 0 | - | - | 0 | - | - | 0 | - | - | 1 |
Baiocchi et al. 2012 [45] | 2 | SV (1), WLE (1) Margin status: R+ | NR | 1 | Aim: adjuvant Total dose/Fr: 54 Gy Technique: external RT Volumes NR | NR | 4 | |||
Wakabayashi et al. 2012 [46] | 1 | WLE and radical hysterectomy ➝ resection of recurrence in the right pelvic floor Margin status: NR | NR | 0 | - | - | 1 | Aim: palliative Scheme: 3-w-TZB (17 cycles) | Moderate headache and flushing during the first infusion, no other adverse effects | 0 |
Cai et al. 2013 [47] | 35 | (1) Radical surgery (RV +/− inguinal LA): EMPDV + adnexal adenocarcinoma (100%), intraepithelial EMPDV (44%), i-VPD (42.8%); (2) Conservative surgery (WLE and SV): i-VPD (57.2%), intraepithelial EMPDV (56%) Margin status: R+: 45.8% in intraepithelial EMPDV, 42.8% in i-VPD and 66.7% in EMPDV + adnexal adenocarcinoma | NR | 17 | Median total dose/fr: primary radical RT (8): median total dose 60 Gy/20 fr (60–63 Gy)/20–29 fr); Adjuvant RT after surgery (6): median total dose 57 Gy (range = 39–60 Gy); Preoperative RT (1): total dose 43.30 Gy; Rescue RT (1): 63 Gy Palliative RT in tibial metastasis (1): NR Technique: Co60 or 9 MeV electrons Volume: pelvic, para-aortic, vulvar, inguinal, tibial | NR | 1 | Aim: neoadjuvant before surgery Scheme: 2 cycles of unspecified CT | NR | 0 |
Choi et al. 2013 [48] | 3 | WLE Margin status: NR | NR | 0 | - | - | 0 | - | - | 3 (topical imiquimod) |
Gavriilidis et al. 2013 [49] | 1 | RV with bilateral inguinal LN dissection Margin status: R0 | NR | 0 | - | - | 0 | - | - | 0 |
Sanderson et al. 2013 [50] | 2 | Vu (1), skinning posterior Vu with reconstruction (1) | NR | 0 | - | - | 0 | - | - | 6 (topical imiquimod) |
De Magnis et al. 2013 [51] | 34 | WLE (61.7%) Partial SV (29.4%) RV (5.9%) Total SV (2.9%) Margin status: R+ in 44.1% | NR | 0 | - | - | 0 | - | - | 0 |
Treglia et al. 2013 [52] | 1 | Radical excision Margin status: R- | NR | 0 | - | - | 0 | - | - | 0 |
Magnano et al. 2013 [53] | 0 | - | - | 0 | - | - | 0 | - | - | Photodynamic therapy with aminolevulinic acid |
Marchitelli et al. 2014 [54] | 0 | - | - | 0 | - | - | 0 | - | - | 10 |
Liu et al. 2014 [55] | 69 | WLE (17.4%), PV (2.9%), SV (37.6%), RV (34.7%) Margin status: R+ in 34.5% | NR | 4 | NR | - | 0 | - | - | 0 |
Luyten et al. 2014 [56] | 0 | - | - | 0 | - | - | 0 | - | - | 20 |
Carrozzo et al. 2014 [57] | 0 | - | - | 4 | Radical HDR brachytherapy with 188Re (Dermo-Beta-Brachytherapy, DBBT)
| Burning sensation and superficial erosions, followed by small crusts, which resolved in 2–3 weeks after DBBT session | 0 | - | - | 0 |
Frances et al. 2014 [58] | 0 | - | - | 0 | - | - | 0 | - | - | Topical imiquimod 5% |
Hata et al., et a. 2015 [59] | 21 | Surgical excision (no other details) Margin status: R+ in 16 cases (76.2%) | NR | 16 | Aim: adjuvant Volume 1: tumor bed Median total dose: 59.4 Gy (range, 45–648 Gy) in 30 fractions (range, 23–36 fractions) Volume 2: pelvic and inguinal lymph node area; total dose: 44–50. 4 Gy. In 3 cases (who had not undergone lymph node removal): total doses of 56, 60 and 612 Gy were delivered with the shrunken field to the enlarged inguinal lymph nodes of the three patients | Acute: 8 cases grade 1 leukopenia;16/16 ≤ grade 2 dermatitis, 9 grade 1 colitis 3 grade 1 cystitis no grade ≥ 3. Late telangiectasia grade 1 | 0 | - | - | 0 |
Asaka et al. 2015 [60] | 1 | Excisional procedures Wide local excision Margin status: NR | NR | 0 | - | - | 0 | - | - | - |
Sopracordevole et al. 2016 [61] | 25 | WLE (5–20%) Partial SV (8–32%) Total SV (9–36%) Skinning (1–4%) Total vulvectomy (2–8%) Total vulvectomy with inguinofemoral lymphadenectomy (11–40.7%) Margin status: R0 | NR | 0 | - | - | 0 | - | - | 2: CO2 laser excision |
Cowan et al. 2016 [62] | 8 | Unilateral PV (5), bilateral PV (1), multiple vulvectomies and skin flaps (1) Margin status: R1 microscopic | NR | 0 | - | - | 0 | - | - | 0 |
Fan et al. 2016 [63] | 18 | 10 local excision (55.5%) 3 WLE (16.7%) 5 SV (27.8%) Margin status: R1 in 3 cases (16.7%) | NR | 0 | - | - | 0 | - | - | 0 |
Nagai et al. 2016 [64] | 2 | Wide local excision + split-thickness skin graft | Graft infection | 0 | - | - | 0 | - | - | 0 |
Onaiwu et al. 2016 [65] | 74 | WLE (61.8%) RV (14.6%) Skinning vulvectomy (4.5%) Mohs surgery (2.3%) Margin status: R1 in 47 cases (63.5%) | NR | 0 | - | - | 0 | - | - | 15 |
Hillmann et al. 2016 [66] | 1 | Radical vulvectomy and bilateral inguinal lymphadenectomy Margin status: R0 | NR | 0 | - | - | 0 | - | - | 0 |
Liau et al. 2016 [67] | 2 | 1 WLE 1 Laser vulvectomy Margin status: NR | NR | 0 | - | - | 0 | - | - | 3: topical imiquimod 5% cream |
Jeon et al. 2016 [68] | 1 | Minimal excision with a 1 cm safety margin Margin status: R0 | NR | 1 | 1.8 Gy per day, 5 times per week, up to 50.4 Gy over 6 weeks to perineum, both inguinal lymph nodes, followed by 19.8 Gy to perineum for 2 weeks | Minimal excision with a 1-cm safety margin | 0 | - | - | 0 |
Dogan et al. 2017 [69] | 1 | HV → WLE Margin status: R+ → R+ | NR | 0 | - | - | 0 | - | - | 1 (topical imiquimod) |
Vicentini et al. 2017 [70] | 0 | - | - | 0 | - | - | 0 | - | - | 1 (topical imiquimod, laser, photodynamic therapy |
Parashurama et al. 2017 [71] | 18 | WLE (88.9%) Radical excision (11.1%) Margin status: R1 in 13 cases (72.2%) | NR | 3 | 45 Gy in 25 fractions was delivered to the pelvis over 5 weeks with a boost to the vulva of 9 Gy in 5 fractions | NR | 0 | - | - | 0 |
Konstantinova et al. 2017 [72] | 102 | WLE, local resections, or vulvectomies Margin status: NR | NR | 0 | - | - | 0 | - | - | 0 |
Mota et al. 2017 [73] | 1 | SV Margin status: NR | NR | 0 | - | - | 0 | - | - | 0 |
Long et al. 2017 [74] | 90 | WLE (96.7%) Abdominoperineal resection (3.3%) Margin status: R1 in 39.2% | NR | 0 | - | - | 0 | - | - | 0 |
Kato et al. 2018 [75] | 2 | 1: wide local excision of the primary site and biopsies of both inguinal lymph nodes 1: wide local excision and left groin lymph node dissection | NR | 2 | Aim: adjuvant at ilioinguinal areas and iliac region | NR | 2 | Aim: palliative Scheme: 1° line CT: docetaxel (60 mg/m2) 2° line CT: TS-1 (100 mg/body) | NR | 0 |
Borghi et al. 2018 [76] | 79 | Vulvar resection, simple vulvectomy, radical vulvectomy. Locoregional (inguinal or pelvic) lymphadenectomy | NR | 7 | Aim: adjuvant | NR | 7 | Aim: palliative Scheme: 5-FU, cisplatin, carboplatin, mitomycin C, vincristine, etoposide, and docetaxel, either as single agents or in combined regimes | NR | 0 |
Sawada et al. 2018 [77] | 4 | Simple vulvectomy | NR | 0 | - | - | 0 | - | - | 9 (topical imiquimod) |
Hiratsuka et al. 2018 [78] | 0 | - | - | 1 | Aim: radical Total dose/Fr: 25 Gy-Eq Technique: BNCT Volume: NR | Acute moderate skin erosion and dysuria | 0 | - | - | 0 |
Hsieh et al. 2018 [79] | 6 | WLE Margin status: NR | NR | NR | Aim: rescue RT Total dose/Fr and technique: external beam vulvar radiation 56 Gy + interstitial brachytherapy to 30 Gy. Volume: perineum and the left-side inguinal nodes + interstitial brachytherapy | NR | NR | Aim: palliative Scheme: 1° line CT: 6 cycles of carboplatin AUC 5 + paclitaxel 175 mg/m2 q21, TZB from cycles 2 to 6 + TZB 5 cycles maintenance 2° line CT: T-DM1 3.6 mg/kg q21 for 6 cycles | 2° line CT: G1 fatigue and bleeding, (intermittent vaginal bleeding, hematuria, epistaxis, and gum bleeding, which started after cycle 2) | 0 |
Nitecki et al. 2018 [80] | 42 | WLE (19%) Partial SV (26.2%) RV (47.7%) Vulvar mapping (7.1%) Margin status: R+ in 3 cases (7.1%) | NR | 0 | - | - | 0 | - | - | 0 |
Rioli et al. 2018 [81] | 0 | - | - | 0 | - | - | 0 | - | - | 13 |
Kato et al. 2018 [82] | 0 | - | 0 | - | - | 8 | Aim: palliative Scheme: 5-fluorouracil (500 mg/body, 7 days/week) and cisplatin (5 mg/body 5 days/week) | - | 0 | |
Cai et al. 2018 [83] | 0 | - | - | 0 | - | - | 8 | Aim: palliative Scheme: docetaxel 60 mg/m day 1; cisplatin 25 mg/m day 1–3 | - | 0 |
Bouceiro-Mendes et al. 2019 [84] | 1 | Local surgical excision followed by WLE ➝ total Vu with fasciocutaneous flap reconstruction. Margin status: R1 (both excision); R0 (Vu) | NR | 1 | Aim: rescue RT at 4 years after surgery Total dose/Fr: 59.4 Gy Technique: NR Volume: NR | None | 0 | - | - | 0 |
Loiacono et al. 2019 [85] | 24 | WLE (25%), SV (33.3%), extended Vu (41.7%) Margin status: R1 in 12 patients | Wound dehiscence (4) and 1 case urethral stenosis (1) | 0 | - | - | 0 | - | - | 0 |
Mujukian et al. 2019 [86] | 1 | Wide local excision and then radical excision with a split skin graft reconstruction 6 years after the original diagnosis | NR | 0 | - | - | 0 | - | - | 1 (topical imiquimod) |
Molina et al. 2019 [87] | 3 | Local excision | NR | 0 | - | - | 0 | - | - | 3 (topical imiquimod 5% in all, photodynamic therapy in case 1) |
Hirai et al. 2019 [88] | 0 | - | - | 0 | - | - | 5 | CDDP (30 mg/m2), EPI (50 mg/m2), and TXL (120 mg/m2) per cycle | Ematotoxicity (100%) | 0 |
van der Linden et al. 2019 [89] | 74 | ni-VPD (51): 64.7% WLE, 9.8% HV, 23.5% (skinning) Vu mi-VPD (10): NR i-VPD (13): 37.5% skinning Vu, 12.5% HV, 31.3% WLE, 29.2% groin surgery. Margin status: ni-VPD: R+ in 82.4% mi-VPD: R+ i-VPD: R+ in 76.9% | NR | 3 (1 in mi-VPD group, 2 in i-VPD group) | NR | NR | 1 | In i-VPD group Aim: palliative Scheme: NR | NR | 21 |
Panoskaltsis et al. 2019 [90] | 1 | WLE | NR | 1 | Aim: adjuvant | - | 0 | - | - | 0 |
Nasioudis et al. 2020 [91] | 2412 | WLE (46.9%) PV (34.1%) RV (4.2%) Total vulvectomy (6.4%) NR (6.8%) Margin status: R+ in 52.7% | NR | 35 | NR | NR | 0 | - | - | 121 (immunotherapy) |
Bartoletti et al. 2020 [92] | 4 | Case 1: vulvo-perianal WLE with left colostomy ➝ ileo-inguinal LA and enlargement of previous perineal resection Case 2: SV ➝ vulvar resection ➝ urethral relapse excision ➝ new resection of the urethral meatus Case 3: SV with positive margins ➝ RV with the excision of the perineum, perianus, and part of the gluteal muscle ➝ bulky inguinofemoral LNs excision Case 4: laser vaporization of the anal and perianal mucosa and a SV with skin grafts, with two large triangular flaps bilaterally (V-Y plastic) ➝ skinning Vu of the anterior hemi-vulva with plastic reconstruction and perianal/anal excision Margin Status: NR | NR | 2 | Aim: palliative RT after surgery Total dose/Fr: Case 2: 30 Gy Case 3: 30 Gy/10 fr Technique: Case 2: NR Case 3: whole brain radiation Volume: Case 2: pelvis Case 3: brain | NR | 4 | Aim: palliative after surgery Scheme: Case 1: w-paclitaxel + w-TZB for 9 months, then maintenance with 3-w-TZB for 1 year ➝ re-challenge with w-paclitaxel and TZB for 7 months, then maintenance with 3-w-TZB for 7 months ➝ TZB emtansine for 2 months, ongoing Case 2: 3-w-cisplatin for 2 cycles➝ w-paclitaxel + w-TZB for 5 months Case 3: w-paclitaxel for 4 total cycles + w-TZB for 4 months, then 3-w-TZB for 11 months ➝ 3-w-TZB and carboplatin for 1 cycle Case 4: 3-w-TZB ➝ w-paclitaxel and 3-w-TZB | Case 1: G3: neutropenia Case 2: G2: anemia, onychopathyCase 3: G1: fatigue, constipation, nausea, anemia and alopecia, G2: neurotoxicityCase 4: G2: neurotoxicity | 3 |
Bruce et al. 2020 [93] | 1 | Left RV ➝ bilateral sentinel LN biopsy ➝ left inguinal LA. Margin status: R0 in adenocarcinoma area, R+ in EMPD area | NR | 1 | Aim: adjuvant Total dose/fr: 5940 cGy/33 fr Technique: NR, but in combination with CT Volume: pelvis | NR | 1 | Aim: adjuvant Scheme: 2 cycles of carboplatin + paclitaxel ➝ delivery ➝ CT-RT with w- cisplatin ➝ TZB 1 year | NR | 0 |
Noel et al. 2020 [94] | 1 | Left RV with left groin dissection, laparoscopic left inguinal LN dissection, and perineal V-Y plasty repair Margin status: NR | NR | 1 | Aim: adjuvant Total dose/Fr: NR Technique: NR Volume: NR | NR | 0 | - | - | 0 |
Kilts et al. 2020 [95] | 1034 | Surgery | NR | 0 | - | - | 0 | - | - | 0 |
Rathore et al. 2020 [96] | 0 | - | - | 0 | - | - | 0 | - | - | 1 |
Sarkar et al. 2020 [97] | 1 | WLE Margin status: R0 inferior, R+ superior and lateral | NR | 1 | Aim: adjuvant Total dose/fr: 50 Gy/28 fr Technique: NR Volume: groin, bilateral inguino-femoral and pelvic LNs | NR | 0 | - | - | 0 |
Sopracordevole et al. 2020 [98] | 7 | WLE (57%), superficial total Vu (29%), skinning Vu (14%) Margin status: R+ in 3 cases (42.9%) | NR | 3 | Aim: prior treatment Total dose/Fr: NR Technique: NR Volume: NR | NR | 0 | - | - | 0 |
Stasenko et al. 2020 [99] | 19 | NR | NR | 0 | - | - | 1 | Aim: palliative Scheme: PIK3CA inhibitor in clinical trial | NR | 7 |
Liang et al. 2021 [100] | 38 | ni-VPD (29): PV (48%), SV (28%), RV (14%), WLE (10%) mi-VPD (8): PV (88%), SV (12%) i-VPD (1): NR Margin status: ni-VPD: R+ in 51.7% mi-VPD: R+ in 12.5% i-VPD: NR | NR | 1 | Aim: adjuvant, concurrent CT-RT Total dose/Fr: NR Technique: NR Volume: NR | NR | 1 | Aim: adjuvant, concurrent CT-RT Scheme: NR | NR | 0 |
Hirata et al. 2021 [101] | 1 | Surgical resection of vulvar and perineal tumors Margin status: NR | NR | 0 | - | - | 1 | Aim: palliative Scheme: docetaxel, for more than 16 months | NR | 0 |
Kosmidis et al. 2021 [102] | 2 | WLE Margin status: R0 | None | 0 | - | - | 0 | - | - | 0 |
Mazzilli et al. 2021 [103] | 1 | NR | NR | - | - | - | 0 | - | - | 0 |
Preti et al. 2021 [104] | 95 | WLE (41–44%) HV (26–27%) TV (13–16%) Margin status: R+ in 91.7% | NR | 0 | - | - | 0 | – | - | 27 |
Liu et al. 2021 [105] | 54 | 25 extended resection (8 experienced unilateral or bilateral inguinal lymph node metastasis and underwent inguinal lymph node dissection), 29 palliative resection Margin status: NR | NR | 0 | - | - | 0 | - | - | 0 |
Ferrara et al. 2021 [106] | 0 | - | - | 0 | - | - | 0 | - | - | 10 |
Bajracharya et al. 2022 [107] | 1 | RV + bilateral groin dissection + bilateral gracilis pedicles flap + urethral and introitus reconstruction Margin status: R0 | NR | 1 | Aim: adjuvant Total dose/Fr: NR Technique: NR Volume: NR | NR | 0 | - | - | 0 |
Wang et al. 2022 [108] | 0 | - | - | 0 | - | - | 0 | - | - | 2 |
Borella et al. 2022 [109] | 0 | - | - | 0 | - | - | 0 | - | - | 55 |
Van der Linden et al. 2022 [110] | 0 | - | - | 0 | - | - | 0 | - | - | 24 |
Reference | Median FU | DCR/Recurrence Rate | PFS | OS | Recurrence Treatment | |
---|---|---|---|---|---|---|
Systemic PD | Local Recurrence | |||||
Kodama et al. 1995 [16] | NR | NR | ni-VPD + mi-VPD: 65.7% i-VPD: 27.7% | NR | i-VPD: CT, RT | ni-VPD: CT, RT, surgery mi-VPD: surgery i-VPD: CT, RT, surgery |
Fishman et al. 1995 [17] | NR | Mean time to recurrence:
| NR | NR | - | Surgery |
Yoshitatsu et al. 1997 [18] | 6 years | CR | 6 years | 6 years | - | Surgery |
Goldblum et al. 1997 [19] | 9 years | ni-VPD: 4 pts with local recurrence from 25 months to 12 years i-VPD: 1 died of the disease | NR | NR | - | Surgery |
Fanning et al. 1999 [20] | 7 years (3–23) | Recurrence rate: 34% at a median of 3 (0.6–15) years | 3 years | NR | - | - |
Henta et al. 1999 [21] | NR | Nearly local CR | NR | NR | - | - |
Murata et al. 1999 [22] | NR | PR to RT → died of the disease | NR | 13 months | - | - |
Louis-Sylvestre et al. 2001 [23] | 5.1 years (1–12.1) | Mean time to recurrence:
| NR | NR | - | - |
Wilkinson et al. 2002 [6] | NR | NR | Case 1: NR Case 2: no PD at 9 months Case 3: NR | Case 1: NR Case 2: still alive Case 3: NR | - | - |
Tebes et al. 2002 [24] | 13.5 months (1–216) | Mean time to recurrence: 30 months (3–89) | NR | NR | - | Surgery |
Luk et al. 2003 [25] | NR | NR | 10 months | 15 months | - | - |
Wang et al. 2003 [26] | NR | CR | NR | NR | - | Imiquimod |
Chin et al. 2004 [27] | NR | - | 8 years | - | - | - |
Zawislak et al. 2004 [28] | 3 months | CR | - | - | - | - |
Bhattacharya et al. 2005 [29] | 8 years | NR | NR | NR | - | Surgery |
Raspagliesi et al. 2006 [30] | 1–5 months | DCR: 100% (CR 57.1%, PR 42.9%) | NR | NR | - | - |
Yanagi et al. 2007 [31] | 5 months (case 1) and 2 years (case 2) | CR | NR | NR | - | - |
Hatch et al. 2008 [32] | 12 months 6 months | CR | NR | NR | - | - |
Karam et al. 2008 [33] | NR | NR | NR | NR | - | Imiquimod, TZB |
Challenor et al. 2009 [34] | 4 months 3 months | CR | NR | 3.5 months | - | - |
Sendagorta et al. 2010 [35] | 22 months (20–26) | CR | NR | NR | - | - |
Shaco-Levy et al. 2010 [36] | NR | Recurrence rate: 32% | NR | 43% NED, 43% DID, 3% LFU, 2% DOD with invasive adenocarcinoma, 9% AWD | - | - |
Roh et al. 2010 [37] | 36.6 | RR: 56% | NR | NR | NR | NR |
Anton et al. 2011 [38] | NR | CR | NR | NR | - | - |
Feldmeyer et al. 2011 [39] | 12 months | CR | 12 months | 12 months | - | - |
Hanawa et al. 2011 [40] | NR | PR after 4 courses of CT, then PD | NR | NR | RT in inguinal LNs and primary lesion | RT |
Jones et al. 2011 [41] | NR | NR | NR | NR | NR | NR |
Tonguc et al. 2011 [42] | 24 months | CR | 24 months | 24 months | - | Surgery, imiquimod |
Mendivil et al. 2012 [43] | 53 months | RR: 56.2% | NR | NR | NR | NR |
Al Yousef et al. 2012 [44] | NR | Persistence of disease: 100% | NR | NR | NR | NR |
Baiocchi et al. 2012 [45] | 30.5 months (21–40) | DCR: 100% (PR: 25%; CR: 75%) Recurrence rate: 25% | NR | 30.5 months | - | - |
Wakabayashi et al. 2012 [46] | NR | Recurrence | 5 years after 1° surgery, 3 years after 2° surgery | NR | 3-w-TZB | - |
Cai et al. 2013 [47] | NR | Recurrence rate: 34.3% | NR |
| Surgery (12) RT (3) | - |
Choi et al. 2013 [48] | 38 months (34–46) | CR: 100% | 38 months | 38 months | - | - |
Gavriilidis et al. 2013 [49] | 7 months | NR | NED | NED | - | - |
Sanderson et al. 2013 [50] | 18 months (12–24) | CR: 50% PD: 33.3% Recurrence rate: 16.6% | NR | 18 months | - | - |
De Magnis et al. 2013 [51] | 76.9 months | Recurrence rate: 44.1% | 45.7 months | NR | - | WLE |
Treglia et al. 2013 [52] | NR | DCR: 3 years | 3 years | 3 years | Chemotherapy | Chemotherapy |
Magnano et al. 2013 [53] | NR | DCR: 2 months | NR | NR | MAL-PDT (3 treatments) + topical tretinoin 0.05% and vitamin E with CR at time of writing (6 months) | |
Marchitelli et al. 2014 [54] | 18 months | CR: 90% | NR | 14.4 months | - | - |
Liu et al. 2014 [55] | 43.6 months | Recurrence rate: 43.5% | 12.7 months | NR | - | - |
Luyten et al. 2014 [56] | 14.4 months | NR | NR | 14.4 months | - | - |
Carrozzo et al. 2014 [57] | 34 months | All patients showed complete remission of the disease without histological evidence of tumor at the end of the treatments * | 34 months | 34 months | * Second course of brachytherapy in persistent/recurrent disease | - |
Frances et al. 2014 [58] | NR | CR at 2 weeks after the end of the treatments | NR | NR | - | - |
Hata et al. 2015 [59] | 38 months (range, 2–109 months) | 3- and 5-year overall local control: 100% | 3-year distant metastasis-free rates: 66% 5-year distant metastasis-free rates: 55% | 3-year OS: 92%; 5-year OS: 62% 3-year cause-specific survival rates: 92% 5-year cause-specific survival rates: 71% | - | - |
Asaka et al. 2015 [60] | 32 months | CR | NR | Still alive | - | - |
Sopracordevole et al. 2016 [61] | 79.5 months | Recurrence rate: 29.6% | NR | NR | - | - |
Cowan et al. 2016 [62] | 35 months | DCR: 75% (CR) Of the 6 pts for whom a CR was achieved, VPD recurred in 4 (67%) | NR | NR | - | - |
Fan et al. 2016 [63] | 70 months | Recurrence rate 11% | NR | NR | - | - |
Nagai et al. 2016 [64] | Recurrence rate: 50% | 8 months | 25 months | - | - | |
Onaiwu et al. 2016 [65] | 73.9 months | Recurrence rate: 58.4% | NR | 73.2 months | - | Excision + local imiquimod |
Hillmann et al. 2016 [66] | NR | NR | NR | NR | - | - |
Liau et al. 2016 [67] | NR | NR | NR | NR | - | Imiquimod treatment Total duration of the treatment: 22-100-16 months |
Jeon et al. 2016 [68] | NR | NR | NR | NR | - | |
Dogan et al. 2017 [69] | 6 months | CR | 6 months | 6 months | - | - |
Vicentini et al. 2017 [70] | NR | NR | NR | NR | - | - |
Parashurama et al. 2017 [71] | NR | Recurrence rate: 67% | NR | 8 years | - | - |
Konstantinova et al. 2017 [72] | NR | NR | NR | NR | - | - |
Mota et al. 2017 [73] | NR | NR | NR | NR | - | - |
Long et al. 2017 [74] | 5 years | Recurrence rate: 37.7% | 56.1% at 5 years | NR | - | - |
Kato et al. 2018 [75] | 5.7 months 11 months | 18 weeks after surgery 10 months after surgery | NR | 5.7 months 11 months | - | - |
Borghi et al. 2018 [76] | 57 months | Recurrence rate: 60%, with a mean time to first recurrence of 20 (range, 5–36) months | NR | Invasive group: 70% at 48 months | - | - |
Sawada et al. 2018 [77] | 46 months | DCR: 100% (56% CR, 44% PR) | NR | NR | - | - |
Hiratsuka et al. 2018 [78] | NR | DCR: CR in 6 months | 3.2 years | 3.2 years (DID) | - | - |
Hsieh et al. 2018 [79] | NR | DCR: CR after 1° line CT, CR after 6 T-DM1 cycles | 7.0 months with 1° line CT followed by TZB maintenance; 6.0 months with T-DM1 | NR | TDM1 | 6 excisional procedures, 3 laser ablations, several courses of topical imiquimod treatment, RT |
Nitecki et al. 2018 [80] | 45.8 months | Recurrence rate: 2 months (1–6) | 28.7 months (0–169) | NR | - | Weekly cisplatin + palliative radiation |
Rioli et al. 2018 [81] | 38 months | Recurrence rate: 7% | NR | NR | - | - |
Kato et al. 2018 [82] | - | DCR: 75% (50% PR, 25% SD) PD: 25% | 25.0 weeks | 77.4 weeks | - | - |
Cai et al. 2018 [83] | 53 months | DCR: 100% (50% PR, 50% SD) | 9.9 months | 28.9 months | - | - |
Bouceiro-Mendes et al. 2019 [84] | 4 years | Disease control: 4 years post-Vu; 3 months post-RT | 4 years | 4 years | - | RT 4 years after surgery; imiquimod after 3 months post-RT |
Loiacono et al. 2019 [85] | NR | Recurrence rate: 33% | NR | NR | - | Surgery |
Mujukian et al. 2019 [86] | NR | NR | NR | NR | - | Surgery |
Molina et al. 2019 [87] | NR | NR | NR | NR | - | - |
Hirai et al. 2019 [88] | NR | - | 20.1 months | 8 months | - | - |
van der Linden et al. 2019 [89] | 38 months | Recurrence rate: 36.4% | ni-VPD: 69.3 months i-VPD: 26.5 months | NR | CT | - |
Panoskaltsis et al. 2019 [90] | 18 months | CR | NR | 18 months | - | - |
Nasioudis et al. 2020 [91] | 66.5 months | NR | NR | NR | - | - |
Bartoletti et al. 2020 [92] | NR | NR | NR | NR | CT | Surgery |
Bruce et al. 2020 [93] | NR | NR | NR | NR | - | - |
Noel et al. 2020 [94] | NR | NR | NR | NR | - | - |
Kilts et al. 2020 [95] | NR | NR | NR | Five-year cancer specific survival (CSS) was 95.5% and was associated with the stage. Compared to patients with localized disease, patients with distant metastases had dramatically worse CSS (HR: 85.8 (31.8–248) p < 0.0001). Vital status (120 months): alive, 569 (94.7%); died, 32 (5.3%) | - | - |
Rathore et al. 2020 [96] | NR | NR | 1 month | 1 month | - | - |
Sarkar et al. 2020 [97] | NR | DCR: 18 months | 18 months | Still alive | - | - |
Sopracordevole et al. 2020 [98] | NR | DCR: 24 months | NR | NR | - | Surgery (9 pts, 90%); third treatment for recurrence (4 pts, 40%), a quarter treatment (2 pts, 20%), fifth treatment for recurrence of disease (1 pt, 10%) |
Stasenko et al. 2020 [99] | 8 months | Recurrence rate: 62% | NR | NR | PIK3CAi (1 pt 6%) | Surgery (10 pts, 63%), imiquimod (4 pts, 25%), topical 5-FU (1 pt 6%). |
Liang et al. 2021 [100] | NR | NR | NR | NR | - | Surgical excision |
Hirata et al. 2021 [101] | NR | NR | NR | NR | - | - |
Kosmidis et al. 2021 [102] | NR | NR | NR | NR | - | - |
Mazzilli et al. 2021 [103] | NR | NR | NR | NR | - | - |
Preti et al. 2021 [104] | 94.6 months | Recurrence rate: 73% | NR | 39 months | - | - |
Liu et al. 2021 [105] | 2 years and at most 10 years | NR | NR | NR | - | - |
Ferrara et al. 2021 [106] | 12 months | Recurrence rate: 60% | NR | 12 months | - | - |
Bajracharya et al. 2022 [107] | 24 months | - | 24 months | 24 months | - | - |
Wang et al. 2022 [108] | 17.4 months | Recurrence rate: 36.4% DCR: Case 1: relapse at 24 month (FU 27 months). Case 2: no relapse (FU 14 months) | 24 months | NR | - | - |
Borella et al. 2022 [109] | 66 months | NR | NR | 31 months | - | - |
Van der Linden et al. 2022 [110] | 31 months | Recurrence rate: 34.8% | NR | 31 months | - | - |
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Share and Cite
Caruso, G.; Barcellini, A.; Mazzeo, R.; Gallo, R.; Vitale, M.G.; Passarelli, A.; Mangili, G.; Pignata, S.; Palaia, I. Vulvar Paget’s Disease: A Systematic Review of the MITO Rare Cancer Group. Cancers 2023, 15, 1803. https://doi.org/10.3390/cancers15061803
Caruso G, Barcellini A, Mazzeo R, Gallo R, Vitale MG, Passarelli A, Mangili G, Pignata S, Palaia I. Vulvar Paget’s Disease: A Systematic Review of the MITO Rare Cancer Group. Cancers. 2023; 15(6):1803. https://doi.org/10.3390/cancers15061803
Chicago/Turabian StyleCaruso, Giuseppe, Amelia Barcellini, Roberta Mazzeo, Roberta Gallo, Maria Giuseppa Vitale, Anna Passarelli, Giorgia Mangili, Sandro Pignata, and Innocenza Palaia. 2023. "Vulvar Paget’s Disease: A Systematic Review of the MITO Rare Cancer Group" Cancers 15, no. 6: 1803. https://doi.org/10.3390/cancers15061803