Prognostic Factors in Paget’s Disease of the Vulva: A Study of 21 Cases

A review of the surgical pathology files of the British Columbia Cancer Agency and the Vancouver Hospital and Health Sciences Center found 21 cases of Paget’s disease over the last 31 years. Possible prognostic factors, including presence and depth of invasion, status of resection margins, tumor DNA cell content, and immunoreactivity for p53 and estrogen receptor proteins were identified. Additional testing and immunostaining were performed using paraffin block samples, which were available for 20 of the 21 cases. The median age of the patients at diagnosis was 67 years (range, 45–82 years). Noninvasive vulvar Paget’s disease was seen in 11 patients; minimally invasive vulvar Paget’s disease (invasion of ≤1 mm), in 7 patients; and invasive disease with invasion of 2, 3, and 8 mm, in 3 patients. All three patients with invasive disease underwent radical vulvectomy and inguinal node dissection. Noninvasive disease and minimally invasive disease were treated with wide local excision or some form of vulvectomy. The three patients with invasive disease all had positive nodes but negative specimen margins. Positive margins were seen in 5 of the 11 patients with noninvasive disease and in 6 of the 7 patients with minimally invasive disease. After 1.8 months to 31 years of follow-up, recurrences of in situ Paget’s disease have been seen in five patients with noninvasive disease (mean time to recurrence, 106 months; range, 1–28 years). One patient had negative margins at her initial treatment. Three patients with minimally invasive disease, all with positive margins, had local recurrence of in situ Paget’s disease 15 to 106 months (mean, 72 months) after treatment. One of the three patients with tumor invasion died of metastatic Paget’s disease 4.4 years after treatment. At the close of the study period, the second patient with invasive tumor was alive and well at 25 months, and the third had died of unknown causes. Five of seven patients with minimal invasion were alive with no evidence of disease, and one died of myocardial infarction. The other woman was alive with in situ recurrence over most of the vulva after declining additional surgery. Seven of the 11 patients with noninvasive Paget’s disease were alive with no evidence of disease, one had recurrence of in situ disease 17.6 years after her initial diagnosis and was preparing for treatment, and three died of other causes. Immunohistochemical analysis showed strong immunoreactivity for CK7, which facilitated identification of Paget’s disease in the dermis. The immunohistochemical profiles were GCDFP+/CK7+/CK20− in 14 specimens, GCDFP+/CK7+/CK20+ in 4, and GCDFP−/CK7+/CK20− in 2. All lesions were estrogen receptor positive. Sixteen tumors were positive for p53 on immunostaining, and four were negative. Diploid tumors did not recur more often than aneuploid tumors (3 of 5 and 4 of 10, respectively). Int J Gynecol Pathol 1999;18;351–359

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