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Invasive Paget Disease of the Vulva

Chiara Borghi, Giorgio Bogani, Antonino Ditto

    International Journal of Gynecological Cancer : Official Journal of the International Gynecological Cancer Society
    |October 5, 2017
    PubMed
    Abstract

    Insights

    Invasive extramammary Paget disease of the vulva (EPDV) is rare but aggressive. This study found a high mortality (40%) and recurrence rate (60%) in patients with invasive EPDV.

    Area of Science:

    • Gynecologic Oncology
    • Dermatopathology

    Background:

    • Extramammary Paget disease of the vulva (EPDV) is a rare condition with a typically indolent and relapsing course.
    • Progression to invasive disease occurs in a significant minority of cases (4-19%).

    Purpose of the Study:

    • To report the clinical-pathological features and outcomes of patients treated for invasive EPDV.
    • To highlight the challenges in managing this rare but aggressive gynecological neoplasm.

    Main Methods:

    • Retrospective review of consecutive patients treated for invasive EPDV between 2000 and 2017.
    • Analysis of clinical data, treatment modalities, and patient outcomes.

    Main Results:

    • Out of 79 EPDV patients, 10 (12.7%) had invasive disease at diagnosis or follow-up.
    • The mortality rate was 40%, and the recurrence rate after treatment was 60%, with a median time to recurrence of 20 months.
    • All patients underwent radical surgery, and 70% received adjuvant radiotherapy, chemotherapy, or both.

    Conclusions:

    • Invasive EPDV is a rare gynecological neoplasm with a poor prognosis.
    • Multicenter trials and prospective data collection are needed to improve understanding and management strategies for invasive EPDV.

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