Paget's disease of the vulva: pathology, pattern of involvement, and prognosis.
ABSTRACT The aim of this study was to determine prognostic factors and risk factors for recurrence in patients with Paget's disease of the vulva.
The medical records of 76 patients with a diagnosis of Paget's disease 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), invasive Paget's disease (IP) (n = 9), intraepithelial Paget's disease with underlying adenocarcinoma (IEPUA) (n = 13), and intraepithelial Paget's disease with a coexisting cancer (CCA) (n = 8). Comorbid conditions, location of disease, pathologic diagnosis, method of treatment, margin status, and current status of the patient were evaluated. Descriptive statistical data and univariate analysis were generated using the Statview statistical package.
A diagnosis of IEPUA, IP, 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 disease 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 more radical treatments (P = 0.02). Risk factors for recurrence included treatment with WLE (P = 0.004).
Patients with IP, IEPUA, or CCA have a poorer prognosis than patients with IEP. Location of Paget's disease is important for prognosis; and patients with clitoral Paget's disease may require more aggressive treatment. WLE is associated with a higher risk of recurrence, but overall patients with WLE tend to survive longer than patients treated more radically.
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ABSTRACT: Seven cases of patients with Paget's disease of the anus are presented. The clinical picture is variable. Treatment and prognosis depend on the presence of an underlying invasive carcinoma. If present, treatment is directed toward the underlying carcinoma. If invasive carcinoma is not present, local excision with clear margins is sufficient. Patients who had Paget's disease of the anus without underlying invasive malignancy were cured, although multiple excisions were required. Patients who had perianal Paget's with invasive carcinoma that had already metastasized to the inguinal nodes did poorly, and all died despite treatment. When extramammary Paget's disease was associated with early localized invasive carcinoma the patients did well. The case for “cancer proneness” in extramammary Paget's disease is supported by this study. Seventy-one per cent of patients who had perianal Paget's disease had associated malignancies, and one patient had three separate carcinomas.Diseases of the Colon & Rectum 12/1975; 19(1):30-40. · 3.34 Impact Factor
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ABSTRACT: A case of locally extensive perianal Paget's disease is presented. Initial wide local excision, guided by frozen sections, was inadequate. Multiple punch biopsies subsequently revealed extensive circumferential involvement of the anoderm by Paget's disease, making wide local excision difficult. Therefore, the patient was treated with combined chemoradiotherapy (5000 cGy, 5-fluorouracil, and mitomycin C). Fourteen months after treatment, the patient had a complete response.Diseases of the Colon & Rectum 01/1990; 33(2):150-152. · 3.34 Impact Factor
Article: Paget's disease of the vulva[show abstract] [hide abstract]
ABSTRACT: Thirty-six patients with Paget's disease of the vulva were reviewed. Median age of the patients at diagnosis was 64 years (range 41–84 years). Five patients (14%) had an associated invasive adenocarcinoma of the vulva at the time of diagnosis. Of 31 patients with superficial noninvasive Paget's disease, 28 were available for follow-up. Treatment of patients with superficial Paget's disease was surgical and based on the extent of disease. Procedures performed included total vulvectomy (25), wide local excision (4), and skinning vulvectomy with skin graft (1). The median follow-up was 108 months (range 6–266 months). Twenty-two of twenty-eight patients remained free of disease. Six patients have required multiple procedures for recurrent superficial Paget's disease. Treatment of Paget's disease of the vulva is surgical. Radical surgery is the preferred treatment of patients with an associated invasive adenocarcinoma; three of five patients with an invasive adenocarcinoma were long-term survivors. Patients with superficial Paget's disease of the vulva should be treated by local excision utilizing frozen-section margin evaluation as a guide to extent of excision. Only one patient with an initial diagnosis of superficial Paget's disease of the vulva developed invasive adenocarcinoma.Gynecologic Oncology 01/1991; · 3.93 Impact Factor