Elsevier

Gynecologic Oncology

Volume 104, Issue 3, March 2007, Pages 547-550
Gynecologic Oncology

The outcomes of patients with positive margins after excision for intraepithelial Paget's disease of the vulva

https://doi.org/10.1016/j.ygyno.2006.09.017Get rights and content

Abstract

Objectives.

Vulvar Paget's disease is a rare neoplasm that usually occurs in postmenopausal women. Treatment with surgical excision can be complicated by extension of microscopic disease in an irregular manner well beyond the visible margins of the lesion. The objective or our study was to analyze the outcomes of patients with primary vulvar intraepithelial Paget's disease who had positive microscopic margins after primary excision.

Methods.

We reviewed the records of all patients with Paget's disease of the vulva treated at our institution from 1/80 to 9/02. Patients whose sample showed stromal invasion or an underlying carcinoma were excluded. Data were collected regarding patient demographics, disease location, treatment, surgical margin status, additional treatment, and clinical outcome.

Results.

The medical records and histopathologic specimens of 28 women with intraepithelial Paget's disease of the vulva were evaluated. Surgical treatment consisted of radical vulvectomy in 3 patients (11%), simple vulvectomy in 18 patients (64%), and wide local excision in the other 7 patients (25%). Of the 20 patients with microscopically positive margins, 14 (70%) developed recurrent disease and the remaining 6 (30%) are disease free. Of the 8 patients with negative margins, 3 (38%) developed disease recurrence and the remaining 5 (63%) are disease free. With a median follow-up of 49 months (range, 3–186 months), there was no correlation between disease recurrence and margin status (P = 0.20). Of the 17 patients who recurred, 14 (82%) underwent additional surgical excision and 1 patient was treated with Retin-A. The remaining 2 patients refused further treatment and were lost to follow-up. In those patients who underwent surgery for recurrence, between 1 and 3 re-excisions were performed. Of the 15 evaluable patients who were treated for recurrent disease, 12 (80%) had no evidence of persistent disease and 3 (20%) had persistent disease at a median follow-up of 63.7 months (range, 18.5–186 months).

Conclusions.

Microscopically positive margins following surgical excision of vulvar intraepithelial Paget's disease is a frequent finding, and disease recurrence is common regardless of surgical margin status. Long-term monitoring of patients is recommended, and repeat surgical excision is often necessary.

Introduction

Paget's disease was first described in 1874 by Sir James Paget as a rare intraepithelial neoplasm of the areola skin [1]. He reported that it primarily affected postmenopausal white women and suggested that similar changes might also be seen in other intraepithelial sites. In 1901, Dubreuilj described the characteristic ‘cake-icing appearance’ of vulvar Paget's disease [2]. Because vulvar Paget's disease is an extremely rare clinical entity, accounting for only 1% of vulvar malignancies, our understanding of this disease is limited.

Clinically, Paget's disease presents as a pink eczematoid area with white islands of hyperkeratosis that is accompanied by pruritus in 70% of patients [3]. A palpable mass should raise concern for underlying invasive disease. Due to the non-specific clinical presentation, diagnosis and treatment are often delayed. A classification system has recently been proposed by Wilkinson and Brown that divides extramammary vulvar Paget's disease into two groups – primary and secondary disease – based on the origin of neoplastic Paget cells. Primary cutaneous Paget's disease, which is the most common type of vulvar Paget's disease, is defined as intraepithelial adenocarcinoma arising within the epidermis or underlying skin appendages. Secondary or non-cutaneous Paget's disease is thought to originate from an underlying non-cutaneous adenocarcinoma, most commonly anal or rectal adenocarcinoma [4]. This should be distinguished from primary cutaneous Paget's disease that arises in the perianal skin and extends to the vulvar area. Approximately 20% of cases of primary vulvar Paget's arises in the perianal area [5] and immunohistochemical studies may be helpful in distinguishing primary and secondary lesions [6].

Standard treatment of vulvar Paget's disease is surgical excision; however, recurrence is common, with a reported range of 21% to 61% [7], [8], [9]. In an attempt to decrease the risk of recurrence, some authors have advocated for the use of intraoperative frozen-section analysis of biopsies from the perimeter of the planned area of resection to better identify the true negative pathologic margins. Unfortunately, frozen-section analysis can be misleading, appearing negative intraoperatively but proving to be positive on later permanent analysis [10], [11], [12]. In addition, the clinical importance of microscopically positive margins remains unclear.

The objective of our study was to analyze the outcomes of patients who had microscopically positive margins after surgical excision of primary vulvar intraepithelial Paget’s disease at our institution from 1980 to 2002.

Section snippets

Methods

After obtaining approval from our Institutional Review Board (IRB), we queried our Department of Pathology database to identify all women with vulvar Paget's disease who were treated by the Gynecology Service at our institution from January 1980 to September 2002. As a requirement for further analysis in this study, all histologic preparations were reassessed by a gynecologic pathologist and the diagnosis of vulvar Paget's disease confirmed for each patient.

Intraepithelial Paget's disease is

Results

Fifty-six patients with Paget's disease of the vulva were treated at our institution during the study period. There were 35 patients for whom complete medical records and histopathologic specimens were available for review. Of these 35 patients, 28 (80%) were found to have primary vulvar intraepithelial Paget's disease; these patients comprised our study group. The median age at diagnosis was 68 years (range, 48–86), and all patients were Caucasian. The 2 most common presenting complaints were

Discussion

Surgical excision is accepted as the standard modality of treatment for vulvar Paget's disease. Historically, surgery for Paget's disease consisted of a radical vulvectomy because of the risk of recurrence and the risk of an underlying adenocarcinoma. However, this type of extensive surgery is associated with significant disfigurement and a persistently significant local recurrence rate. Therefore, many authors believe that there is little advantage to such surgery and advocate a more

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