Vulvar Paget disease: A national retrospective cohort study

Linden, M. van der; Oonk, Maaike H. M.; Doorn, Helena C. van; Bulten, Johan; Dorst, E.B.L. van; Fons, Guus; Lok, Christianne; Poelgeest, Mariëtte I.E. van; Slangen, Brigitte M.F.; Massuger, Leon F.A.G.; Hullu, Joanne A. de

doi:10.1016/j.jaad.2018.11.016

Cited by 49 publications

(31 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In our case study, unlike others reported in the literature, [3,4,21–23] there was only 1 case of a patient with a very aggressive disease with rapid spread and death. Furthermore, our study highlights the important role of plastic surgery.…”

Section: Discussioncontrasting

confidence: 76%

See 1 more Smart Citation

Paget disease of the vulva an analysis of 24 cases

Loiacono

Traversi²,

Deliso

et al. 2019

Medicine

View full text Add to dashboard Cite

Paget's disease can arise in the breast (mammary Paget disease) or in other locations (extramammary Paget disease) such as anogenital skin in both males and females (Paget disease of the vulva [PDV]). Underlying adenocarcinoma can be found in some cases. This study aims to report clinical aspects, surgical procedures, outcomes, and recurrences of patients with PDV.A retrospective chart review was conducted on patients with pathologically confirmed diagnosis of PDV managed at the Department of Obstetrics and Gynecology, University of Bari, and the “Giovanni Paolo II” National Cancer Institute in Bari, between 1998 and 2018.Records of 24 cases of PDV were examined. Median age of the patients at diagnosis was 69.3 (range 38–84), diagnosis of synchronous cancer was made in 2 cases and in 2 other cases of metachronous disease. Three patients had previously been diagnosed with other oncological diseases. All patients underwent surgery including wide local excision (6), simple vulvectomy (8), and extended vulvectomy (10). Lymphadenectomy was performed in 2 cases and reconstructions with advancement flaps in 7 cases. Four patients were found to have invasive disease and 1 had inguinal node involvement. Positive margins were found in 11 patients. Wound dehiscence and urethral stenosis were found in 4 and 1 case each. Eight recurrences (33.33%) were observed, regardless of positive surgical margins.PDV has a low rate of malignancy but a high rate of recurrence. It should be diagnosed early to avoid repeated surgery and to reduce symptoms and morbidity.

show abstract

Section: Discussioncontrasting

confidence: 76%

“…Our retrospective study confirms that in case of recurrence the disease rarely become invasive, so once the presence of underlying or co-existent malignancy is excluded, we could opt for the use of Topical 5% imiquimod cream, now widely considered effective for high grade vulvar squamous cell intraepithelial lesions. [20,21]…”

Section: Discussionmentioning

confidence: 99%

Paget disease of the vulva an analysis of 24 cases

Loiacono

Traversi²,

Deliso

et al. 2019

Medicine

View full text Add to dashboard Cite

show abstract

“…[1] Considering the relatively good prognosis of EMPD in those areas, with reported 5-year survival rates ranging from 50-98%, their estimated prevalence rates should be higher than our result. [11,13,20,21] Within Asian area, our result was approximated to the range of rates in Taiwan of China. [22] Therefore, we could not exclude the possibility that ethnic disparity might contribute to this discrepancy, considering the fact that skin cancers are more prevalent in whites than in Asians.…”

Section: Discussionmentioning

confidence: 86%

Prevalence of Extramammary Paget's Disease in Urban China: A Population-based Study

Yin

Wang

et al. 2020

Preprint

View full text Add to dashboard Cite

Background Extramammary Paget’s disease (EMPD) is an intraepithelial adenocarcinoma. The chronic relapsing clinical course and unbearable clinical symptoms of EMPD usually result in a markedly diminished quality of life. No national data are available on descriptive epidemiology of EMPD in China, the most populous country over the world. This population-based study aimed to estimate the prevalence and associated sex and age patterns of EMPD in China. Methods This study was conducted using data from China’s Urban Employee Basic Medical Insurance and Urban Resident Basic Medical Insurance, covering approximately 0.43 billion Chinese urban residents in 2016. Patients with EMPD were identified based on the diagnostic names and codes in claim data. Results A total of 53 males and 31 females with EMPD were found. The crude prevalence in 2016 was 0.04 per 100 000 population [95% confidence interval (CI): 0.02 to 0.06], ranging from 0.01 (95% CI: 0.00 to 0.02) in North or Northeast China to 0.08 (95% CI: 0.03 to 0.16) in Southwest China. The rates were higher in males (0.05, 95% CI: 0.03 to 0.08) compared with females (0.03, 95% CI: 0.02 to 0.05). The mean age of patients was 65.87 (standard deviation: 14.21) years, with the peak prevalence appeared in patients aged 70–79 (0.28, 95% CI: 0.16 to 0.42). Conclusions The prevalence of EMPD was markedly lower than those in the United States and Europe, and varied across regions in China. Chinese patients were much younger, with significant male predominance. Further study is warranted to examine potential pathophysiologic mechanism.

show abstract

“…Imiquimod was evaluated by 13 studies (8 cohort studies, 5 case series) in 110 patients. CR was documented in 54% of patients, and 85% achieved clinical regression of 50% or more [43][44][45][46][47][48][49][50][51][52][53][54]60]. Given the propensity of EMPD for recurrence, posttreatment histological evaluation is of great importance.…”

Section: Discussionmentioning

confidence: 99%

“…Imiquimod is an immune response modifier that stimulates innate immune pathways (via induction of inflammatory cytokines, such as IFN-α, IL-6, and TNF-α) and adaptive immune pathways, resulting in an antitumor effect [59]. Eight cohort studies (3 prospective, 5 retrospective) and 5 case series (total 110 patients) investigated the effectiveness of imiquimod for EMPD at dose schedules ranging from daily to twice weekly for a duration of 2-56 weeks [43][44][45][46][47][48][49][50][51][52][53][54]60]. Overall, 54% (95% CI, 40-67%; I 2 = 37%) of patients achieved complete response (CR) and 85% (95% CI, 74-90%; I 2 = 0%) achieved clinical regression of 50% or more.…”

Section: Imiquimodmentioning

confidence: 99%

Nonsurgical Treatments for Extramammary Paget Disease: A Systematic Review and Meta-Analysis

et al. 2020

View full text Add to dashboard Cite

Introduction: Surgery is commonly regarded as the mainstay of treatment of extramammary Paget disease (EMPD); however, nonsurgical approaches have gained popularity in recent years. Objectives: To review the published evidence for the efficacy and safety of nonsurgical modes of therapy for EMPD. Methods: A systematic review and meta-analysis of nonsurgical EMPD treatments was performed. The primary outcome was complete response (CR); secondary outcomes were clinical regression by ≥50%, adverse events, and recurrence rate. Results: The systematic review included 43 observational studies (341 patients; 7 prospective cohort studies, 19 retrospective cohort studies, and 17 cases series) evaluating 5 treatment modalities. Imiquimod (13 studies, 110 patients) administered at variable doses ranging from daily to twice weekly for 2-56 weeks demonstrated CR of 54% (95% CI, 40-67%; I 2 = 37%) and had a satisfactory safety profile. In 14 heterogeneous studies (122 patients) evaluating photodynamic therapy (PDT), only 36% (95% CI, 22-53%; I 2 = 52%) of patients achieved CR. Radiotherapy (12 studies, 67 patients) showed CR of 97%, but was associated with local and systemic side effects. Ablative lasers and topical fluorouracil and calcipotriene lacked adequate evidence of efficacy. Conclusions: Imiquimod and radiotherapy are the most appropriate nonsurgical modalities for EMPD treatment given their good efficacy and safety profile. PDT has limited efficacy but may be appropriate in selected clinical settings.

show abstract

Vulvar Paget disease: A national retrospective cohort study

Cited by 49 publications

References 17 publications

Paget disease of the vulva an analysis of 24 cases

Paget disease of the vulva an analysis of 24 cases

Prevalence of Extramammary Paget's Disease in Urban China: A Population-based Study

Nonsurgical Treatments for Extramammary Paget Disease: A Systematic Review and Meta-Analysis

Contact Info

Product

Resources

About