Vulvar Paget Disease: A Large Single-Centre Experience on Clinical Presentation, Surgical Treatment, and Long-Term Outcomes

Departments of 1Science for Woman and Child Health and 2Human Pathology and Oncology, University of Florence, Florence, Italy.
Journal of Lower Genital Tract Disease (Impact Factor: 1.99). 04/2013; 17(2):104-10. DOI: 10.1097/LGT.0b013e31826569a9
Source: PubMed


Our aim was to add information to the current literature on vulvar Paget disease by reviewing a consistent number of patients who have been all diagnosed, treated, and followed up by the same group of physicians at a single medical institution.
Clinical, surgical, histological, and follow-up data of 34 patients (mean [SD] age at diagnosis = 68.7 [10.1] years) with vulvar Paget disease were reviewed during a 27-year period.
Primary symptoms were itching (76.5%) and burning (58.8%). Clinical manifestations were present for a mean (SD) of 17.8 (7.2) months before the diagnosis was made. Multifocal lesions were observed in 17 patients (50%) and were associated with a delay in diagnosis exceeding 12 months (p = .03). Of the patients, 10 (29.4%) presented a history of malignancy in other sites. Surgery with various extent of resection was performed as primary treatment in all patients. Definitive histological examination revealed positive surgical margins in 15 cases (44.1%), stromal invasion in 4 (11.7%), and associated adenocarcinoma in 2 (5.9%). Of the patients, 6 (17.6%) underwent reconstructive technique at their primary surgery or radicalization. During a mean (SD) follow-up of 76.9 (51.3) months, 15 patients (44.1%) experienced local recurrence (1 recurrence in 29.4%, 2 recurrences in 5.9%, and 3 recurrences in 8.8%). First recurrence appeared after a mean (SD) time of 45.7 (25.1) months and was associated with multifocal lesions (p = 0.005) and surgical margins involvement (p = 0.03). One patient (2.6%) died of the disease.
Vulvar Paget disease is a chronic disease with high recurrence rate and low mortality. Early diagnosis, minimal surgery with free margins, and long-term follow-up are the cornerstones of treatment.

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    ABSTRACT: The clinicopathologic findings of 13 patients having extramammary Paget's disease of the vulva are discussed with emphasis on its histogenesis and biological behavior. For the purpose of study and assessment of prognosis, these cases were divided into two groups, those with an underlying invasive cutaneous adnexal adenocarcinoma, and those lacking an underlying invasive lesion. Four cases contained invasive cutaneous adnexal adenocarcinoma; in one of these the invasion was superficial. Three of the cases with an invasive lesion and three other cases showed in situ adenocarcinoma of sweat glands. Surgical treatment is mandatory for both groups of patients. The prognosis was excellent for the patients having Paget's disease without an underlying invasive carcinoma. From the literature, the prognosis of those with an underlying invasive carcinoma of the vulva appears to be less favorable. Multiple surgical excisions may be required to control the recurrences and metastases. A frequent association with other internal malignancy was observed. In four cases, second malignancies were found. Of special interest was the demonstration in one case of columns of neoplastic cells extending from involved sweat glands to the surface epithelium via the intradermal sweat duct. Our study leads us to support the concept that the Paget's cells, in a number of cases, are derived from an underlying carcinoma in situ of sweat gland origin.
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    ABSTRACT: Four vulvectomy specimens from patients with vulvar Paget's disease were studied by means of subserial total sectioning. In each case, the extent of histologically demonstrable disease was far greater than that of the visible lesion, the outline of the involved area was highly irregular, and multiple foci of disease were present. The implications of the findings for treatment selection, intraoperative management, and histogenesis are discussed.
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    ABSTRACT: Extramammary Paget disease is generally considered a distinct entity that can involve the genital tract skin and may be associated with underlying adenocarcinoma. Evidence is presented that vulvar Paget disease represents a heterogeneous group of epithelial neoplasms that can be similar both clinically and histopathologically. Three cases of vulvar Paget-like disease that were manifestations of urothelial carcinoma are investigated. Vulvar Paget disease can be classified based on the origin of the neoplastic Paget cells as either primary (of cutaneous origin) or secondary (of noncutaneous origin). Each classification has 3 subtypes: primary, intraepithelial cutaneous Paget disease of the usual type; intraepithelial cutaneous Paget disease with invasion, and intraepithelial cutaneous Paget disease as a manifestation of underlying skin appendage adenocarcinoma; secondary, Paget disease of anorectal origin, Paget disease of urothelial origin, and Paget disease of other origin. This subclassification is based on a review of the literature and the current study of 3 patients with Paget-like disease of urothelial neoplastic origin. The 3 subtypes of vulvar Paget disease studied here can present similarly as eczematoid skin or vulvar mucosal lesions and may appear similar on routine hematoxylin and eosin-stained slides. Immunohistochemical studies can be used to help differentiate them. The distinction between these 3 types of Paget-like lesions is essential in that the specific diagnosis has a significant influence on current treatment. The difference in surgical approach to the subtypes of vulvar Paget disease justifies classifying them into distinct lesions to avoid potential confusion and unnecessary surgery.
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