Paget's disease of the vulva: Pathology, pattern of involvement, and prognosis

Objective. The aim of this study was to determine prognostic factors and ri sk factors for recurrence in patients with Paget's disease of the vulva, Methods, The medical records of 76 patients with a diagnosis of Paget's dis ease of the vulva were retrospectively reviewed. The diagnosis in each case was confirmed by reviewing the pathology. Patients were then divided into four groups by diagnosis: intraepithelial Paget's disease (IEP) (n = 46), i nvasive Paget's disease (IP) (n = 9), intraepithelial Paget's disease with underlying adenocarcinoma (IEPUA) (n = 13), and intraepithelial Paget's dis ease with a coexisting cancer (CCA) (n = 8). Comorbid conditions, location of disease, pathologic diagnosis, method of treatment, margin status, and c urrent status of the patient were evaluated. Descriptive statistical data a nd univariate analysis were generated using the Statview statistical packag e. Results. A diagnosis of IEPUA, IF, or CCA predicted a poor survival (P = 0. 0017), Patients who had received chemotherapy or radiation as treatment had a poor survival (P < 0.0001 and 0.0002), Patients with clitoral Paget's di sease had a higher incidence of death from disease (P = 0.026), When death from all causes was considered, patients treated with wide local excision ( WLE) had a significantly longer survival than patients treated with other m ore radical treatments (P = 0.02), Risk factors for recurrence included tre atment with WLE (P = 0.004). Conclusions. Patients with IF, IEPUA, or CCA have a poorer prognosis than p atients with IEP, Location of Paget's disease is important for prognosis; a nd patients with clitoral Paget's disease may require more aggressive treat ment. WLE is associated with a higher risk of recurrence, but overall patie nts with WLE tend to survive longer than patients treated more radically. ( C) 2000 Academic Press.