Paget's Disease of the Vulva: Pathology, Pattern of Involvement, and Prognosis

Baylor College of Medicine, Houston, Texas, United States
Gynecologic Oncology (Impact Factor: 3.77). 04/2000; 77(1):183-9. DOI: 10.1006/gyno.2000.5741
Source: PubMed


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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    • "Some studies found that positive margins correlate with increased recurrence rate [19] [20]. However, other studies have found no correlation between margin status and local recurrence [21] [22] [23] [24]. The high frequency of recurrence, usually outside the area of previous resection, remains the most challenging feature in the management of vulvar intraepithelial Paget's disease. "
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    ABSTRACT: Paget's disease of the vulva remains a rare condition with only a limited number of cases reported in the literature. It is an uncommon neoplasm usually of postmenopausal white women characterized by controversies in its prevalence, clinical features, treatment strategies, and prognostic. We here report a case of a primary Paget's disease of the vulva in premenopausal woman treated by only surgery with a favorable issue.
    Full-text · Article · Sep 2012 · Case Reports in Oncological Medicine
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    • "In the majority of the cases, the disease is limited to the epidermis. However, it is well known that EMPD has the potential of dermal invasion [4]. Moreover, its association with underlying internal malignancies remained one of the most interesting characteristics of the disease. "
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    ABSTRACT: Background. To examine the results of treatment of Extramammary Paget's disease (EMPD) in ethnic Chinese. Method. Between 1990 and 2010, patients treated for EMPD were reviewed. Data were analyzed retrospectively. Results. Forty-eight patients were treated by surgical resection. Local recurrence rate was 14.6%. The postresection defects were repaired by primary closure (8.3%), partial thickness skin graft (72.9%), or local/regional flaps (18.8%). Dermal invasion was found in 9 patients (18.8%). Seven patients (14.6%) developed regional lymph node metastasis (concurrent with surgery, n = 1; subsequent to surgery, n = 6), and 3 patients (6.3%) had systemic metastasis after surgery. The presence of dermal invasion was associated with significantly higher incidence of regional lymph nodes and systemic metastasis. The incidence of associated internal malignancy was 8.3%. Conclusion. The mainstay of treatment for EMPD is surgery. Pathological dermal invasion increases the chance of regional lymph node as well as systemic metastasis. The association with internal malignancy warrants preoperative endoscopic examination in all patients.
    Full-text · Article · Feb 2012 · International Journal of Surgical Oncology
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    ABSTRACT: Objective: To review our experience in diagnosis, pathology, treatment, and prognosis of extramammary Paget’s disease of the vulva. Methods: Seven patients with vulvar Paget’s disease who were treated at the Cancer Hospital cAMS were retrospectively reviewed for the period from 1960 to 2002. The symptoms, location of disease, pathology, mode of treatment and current status of the patients were evaluated. Results: A total of seven women with Paget’s disease of the vulva were admitted to the Department of Gynecologic of Oncology. The mean age was 67.3 years (range 54–81 years). Vulvar lesion and pruritus were the common symptoms and it took average 3.4 years to confirm the diagnosis. All patients underwent surgery as initial treatment, vulvectomy was performed for 2 patients and radical vulvectomy plus groin node dissection for 5 patients. Two patients had invasive disease and four were associated with underlying adenocarcinoma. Five patients experienced recurrence, on average, 16.2 months after the surgery. Four of them underwent radiotherapy or chemotherapy and the other received no further treatment. The mean follow-up time was 41.1 months. Three patients, with invasive lesion or underlying adenocarcinoma were dead of the disease. Conclusion: There is a delay in the diagnosis of vulvar Paget’s disease. The major treatment is surgery. Recurrence is relatively common. Patients with invasive disease or underlying carcinoma have a poor prognosis.
    No preview · Article · Sep 2004 · Chinese Journal of Cancer Research
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