Vulvar Paget's disease: the need to exclude an invasive lesion

Nineteen patients with intraepithelial Paget's disease of the vulva were treated surgically. Of these patients, five also had invasive Paget's disease of the vulva, two with only minimally invasive lesions. The latter two patients and one patient with a grossly invasive lesion progressed from intraepithelial Paget's disease. One patient had an associated carcinoma of the periurethral glands and an invasive Paget's lesion of the vagina. Since 5 of 19 patients (26%) had an associated noncontiguous carcinoma, the recommended initial workup included sigmoidoscopy, cystoscopy, mammogram, and colposcopy. Koilocytosis was found in 67% of patients. Exclusion of an invasive lesion from intra-epithelial Paget's disease was determined to be necessary because (1) the incidence of minimally invasive Paget's lesions may be significant; (2) intraepithelial Paget's disease has the capacity to become invasive; (3) the rate of associated invasive Paget's disease and/or underlying adnexal malignancies may exceed 20%; (4) grossly invasive lesions may be occult; and (5) the survival rate for grossly invasive lesions is low. Simple vulvectomy at a depth of at least 0.5 cm of subcutaneous tissue is recommended as optimal treatment for intraepithelial Paget's disease.