Prognostic factors for recurrence and survival among patients with invasive vulvar Paget disease included in the VULCAN study

Objective To identify prognostic factors associated with recurrence and overall survival among patients with invasive vulvar Paget disease. Methods An analysis was conducted of patients with invasive vulvar Paget disease included in VULCAN, an international multicenter retrospective study of patients diagnosed with vulvar cancer between January 1, 2001, and December 31, 2005. Data regarding diagnosis, treatment, and follow-up were obtained from the patients' medical records. Univariate and multivariate analyses were performed. Results Among 1727 patients registered in the VULCAN database, 38 patients had invasive vulvar Paget disease. The mean follow-up time for these patients was 44.1 ± 35.7 months, the overall recurrence rate (local lesions and distant metastases) was 58%, and the mean overall survival time was 58.5 ± 0.5 months. Case load at the treating center inversely correlated with local recurrence (P = 0.01). Tumor size and FIGO stage were associated with the presence of distant metastases (P < 0.001 for both). Adjuvant therapy (radiotherapy or chemotherapy) was associated with a reduced risk of distant metastases and increased overall survival (P < 0.001 for both). Conclusion Local recurrences of invasive vulvar Paget disease were associated with the case load at the treating center. Distant recurrences were associated with tumor size and FIGO stage. It is possible that adjuvant radiotherapy or chemotherapy could offer patients benefits by increasing overall survival rates.