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