Paget Disease of the Vulva – A Study of 56 Cases
R Shaco-Levy, SM Bean, RC Bentley, RT Vollmer, JA Papalas, MA Selim, SJ Robboy. Soroka Medical Center, Beer-Sheva, Israel; Duke University Medical Center, Durham, NC; VA Medical Center, Durham, NC
Background: Vulvar Paget Disease (VPD) is a rare locally recurrent chronic disease, accounting for < 1% of vulvar neoplasms. This study of 56 patients examines numerous clinical and pathological features as they relate to recurrence and survival rates.
Design: The medical records and pathology slides from all patients with VPD seen over a 30 year period in one institution were analyzed.
Results: The mean age at diagnosis was 69 years. The average length of follow-up was 5.6 years. Substantial delay between appearance of symptoms and diagnosis (mean 20 months), was significantly associated with larger lesions (p=0.001), leading to more radical resections. Recurrence rate after surgical management was 32%, with interval of 13-131 months from diagnosis. The two statistically significant risk factors for recurrence were disease in the perineum (p=0.044) and epidermal acantholysis (p=0.035). Patients with involved surgical margins had an increased recurrence rate, but not of statistical significance (p=0.14). Intra-operative frozen section analysis of the margins as well as radical surgery as initial treatment did not reduce recurrence rate. Stromal invasion occurred in 10 patients (18%), and was not a statistically significant adverse prognostic indicator. Radiation therapy given to five patients resulted in complete response with no further recurrences. On the last day of follow-up 24 patients (43%) had no evidence of disease, 24 patients (43%) were dead of other causes, five patients (9%) were alive with disease, two patients (3%) were lost to follow-up, and one (2%) died due to VPD with invasive adenocarcinoma.
Conclusions: Significant delay in diagnosis, leading to larger lesion size, is common in VPD patients, emphasizing the importance of awareness to this disease. Surgical treatment should aim to completely excise the lesion with the smallest possible resection. Intra-operative frozen section analysis is not useful unless invasion is suspected. Recurrences are common with disease in perineum and presence of acantholysis being adverse risk factors. Invasion is not a statistically significant poor prognostic indicator. Radiation therapy may serve as an alternative to surgery in selected patients. VPD only rarely results in patient's death, but as recurrences are common and may occur greater than a decade after initial treatment, long term follow-up is required.
Category: Gynecologic & Obstetrics
Tuesday, March 23, 2010 1:00 PM
Poster Session IV # 133, Tuesday Afternoon