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Recurrence patterns and prognostic factors in vulvar extramammary Paget's disease: a 10-year single institution
Nicolò Clemente1, Marco Bernardi2, Fabiola Giudici3
1Centro di Riferimento Oncologico di Aviano (CRO), IRCCS, National Cancer Institute, Gynecological Oncology Unit, Aviano (PN), Italy.
Objective:
Extramammary Paget's disease of the vulva is a rare intra-epithelial malignancy with a high propensity for multiple local recurrences. Surgical excision remains the treatment of choice in most of the cases. However, due to its rarity, treatment modalities and prognostic indicators are still debated. We aimed to evaluate the recurrence patterns and to identify clinical factors associated with risk of recurrence in women with this disease.
Methods:
Retrospective analysis of women treated for vulvar Paget's disease at a single institution from January 2014 to December 2024. Clinical-pathological features, treatment modalities, and factors potentially related to disease recurrence were analyzed. An additional analysis was conducted to identify factors potentially associated with the development of multiple subsequent recurrences over time.
Results:
A total of 69 patients fulfilling the study inclusion criteria were considered. A wide vulvo-perineal-peri-anal involvement was observed in 39.1% of cases, with multifocal disease observed in 37.7% of cases. In total, 34 women (49.3%) developed recurrent disease, with a median disease-free survival of 59.6 months (95% CI 33.3 to 95.9). Multifocal disease at initial diagnosis was the only factor significantly associated with an increased risk of recurrence (OR 2.89, 95% CI 1.05 to 7.96, p = .04). In 32.4% of cases, the recurrence occurred in a vulvo-perineal or peri-anal area distinct from the original site. Multiple recurrences over time were observed in 33.3% of patients.
Conclusions:
Vulvar Paget's disease is associated with a substantial risk of recurrence, particularly in patients with multifocal disease. Recurrences often arise at new anatomical sites, which may be more appropriately defined as "second field" Paget's disease. Although surgery remains a feasible and safe approach even in the setting of recurrent disease, individualized therapeutic strategies are advisable to improve long-term outcomes in this challenging patient population.
