Article Text

Download PDFPDF
1301 Vulvar paget disease: 12 years of experience in oporto portuguese oncology institute
  1. Catarina Costa Neves1,
  2. Mariana Vide Tavares2 and
  3. Almerinda Petiz2
  1. 1Centro Hospitalar Universitário do Algarve – Unidade de Portimão, Portimão, Portugal
  2. 2Instituto Português de Oncologia do Porto, Porto, Portugal

Abstract

Introduction/Background Vulvar Paget’s disease (VPD) is a rare cutaneous disease, representing about 1–2% of all vulvar neoplasms. It mainly affects post-menopausal women with a non-specific clinic presentation; therefore, the diagnosis is frequently delayed. VPD has a high incidence of associated synchronous or metachronous neoplasms and multiple recurrence regardless the treatment. Since there is no global consensus on its optimal management, the aim of this study was to review our experience, examining the demographic and clinic-pathological characteristics, management and outcome of women diagnosed with VPD.

Methodology We performed a retrospective review on 18 patients with pathologically confirmed diagnosis of VPD managed in Oporto Portuguese Oncology Institute, between 2011 and 2022.

Results Average age at diagnosis was 64.8years (range 38–91). The main symptom reported was itching and 50% of women had symptoms at least one year before diagnosis. The average extension of the disease was 6.1cm. All patients underwent surgery consisting in simple vulvectomy in eleven (61.1%), wide local excision in four cases, radical vulvectomy with inguinal lymphadenectomy in two and total vulvectomy with partial vaginectomy in one case. The overall complication rate was 62.5%, mainly surgical wound dehiscence. There were 62% of positive histological margins and seven cases presented signs of invasive disease. Median follow-up was 48 months (range 0–240) during which a 33.3% local recurrence rate was found. In this sample, treatment with vulvectomy versus wide local excision, positive surgical margins or signs of invasion had no apparent association with higher recurrence rates. Previous diagnosis or during follow-up, five women had a diagnosis of invasive vulvar carcinoma and three women had non-vulvar carcinomas.

Conclusion VPD has a high recurrence rate, vulvar functional impairment and negative surgical outcomes. Therefore, there is a need to carry out large series of cases with high-quality evidence for the implementation of guidelines and to consider other treatment options.

Disclosures The authors do not have any conflicts of interest to disclose.

Statistics from Altmetric.com

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.