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      The Effectiveness of Mapping Biopsy in Patients with Extramammary Paget's Disease

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          Abstract

          Background

          Extramammary Paget's disease (EMPD) is an intraepithelial carcinoma usually occurring on the skin or mucosa of the perineum. Clinically, it resembles eczema or dermatitis, and misdiagnosis and treatment delays are common. The treatment of choice for EMPD is a wide excision with adequate margins. Wide excision with intraoperative frozen biopsy and Mohs micrographic surgery are common methods; however, these are associated with a high recurrence rate and long operation time, respectively.

          Methods

          Between January 2010 and June 2013, 21 patients diagnosed with EMPD underwent mapping biopsy. Biopsy specimens were collected from at least 10 areas, 2 cm from the tumor margin. When the specimens were positive for malignancy, additional mapping biopsy was performed around the biopsy site of the positive result, and continued until no cancer cells were found. Based on the results, excision margins and reconstruction plans were established preoperatively.

          Results

          The patients (18 male, 3 female) had a mean age of 66.5 years (range, 50-82 years). Almost all cases involved in the perineal area, except one case of axillary involvement. Permanent biopsy revealed one case (4.8%) of positive cancer cells on the resection margin, in which additional mapping biopsy and re-operation was performed. At the latest follow-up (mean, 27.4 months; range, 12-53 months), recurrence had not occurred.

          Conclusions

          Preoperative mapping biopsy enables accurate resection margins and a preoperative reconstructing plan. Additionally, it reduces the operation time and risk of recurrence. Accordingly, it represents an effective alternative to Mohs micrographic surgery and wide excision with intraoperative frozen biopsy.

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          Most cited references22

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          Mammary and extramammary Paget's disease.

          Mammary and extramammary Paget's disease are uncommon intraepithelial adenocarcinomas. Both conditions have similar clinical features, which mimic inflammatory and infective diseases. Histological diagnostic confusion can arise between Paget's disease and other neoplastic conditions affecting the skin, with the most common differential diagnoses being malignant melanoma and atypical squamous disease. The glandular differentiation of both mammary Paget's disease and extramammary Paget's disease is indicated by morphological appearances, the presence of intracellular mucin in many cases, and positive immunohistochemical staining for glandular cytokeratins, epithelial membrane antigen, and carcinoembryonic antigen. This article provides an overview of mammary and extramammary Paget's disease and discusses recent evidence regarding the cell of origin. The concepts of primary and secondary Paget's disease are presented and the differential diagnosis is discussed with reference to immunohistochemical markers that might be of diagnostic value.
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            Extramammary Paget's disease: treatment, prognostic factors and outcome in 76 patients.

            Extramammary Paget's disease (EMPD) is a rare cutaneous carcinoma usually presenting as a genital erythematous lesion in the elderly. Although most EMPD tumours are in situ, invasive EMPD has a poor prognosis. To evaluate the clinical and pathological features of EMPD and determine prognostic factors for survival. The medical records of 76 patients with EMPD were retrospectively reviewed. Of the 66 patients who underwent curative surgical excision, five (8%) developed local recurrence, but surgical margin ( 2 cm) was not correlated with local recurrence. Thirteen of the 76 patients (17%) developed systemic metastases and 10 of these died of disease. On univariate analysis, the presence of nodules in the primary tumour, clinical lymph node swelling, elevated serum carcinoembryonic antigen (CEA) levels, tumour invasion level and lymph node metastasis were significant prognostic factors. On multivariate analysis, invasion level and elevated serum CEA were the only factors that were significantly associated with reduced survival. Invasion level and lymph node metastasis are important prognostic factors in EMPD. In patients with in situ tumour, local tumour control is the major aim of treatment; however, wide surgical margins are not associated with a lower risk of local recurrence.
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              Extramammary Paget's disease: prognosis and relationship to internal malignancy.

              Extramammary Paget's disease is a rare cutaneous adenocarcinoma, usually of epidermal origin and glandular differentiation and frequently associated with an underlying adnexal carcinoma and perhaps with underlying internal malignancy. One hundred ninety-seven cases of extramammary Paget's disease (196 cases reported in the English literature from 1962 to 1982 and one case of my own) are reviewed. It remains a rare cutaneous malignancy that occurs primarily in elderly people. It is seen more frequently in women than in men and occurs predominantly in vulvar and perianal locations. Twenty-six percent of patients with this disease will ultimately die of it or an associated internal malignancy. Twenty-four percent of patients with the disease have an associated underlying cutaneous adnexal adenocarcinoma. These patients have a higher mortality rate--46%--than patients with extramammary Paget's disease without underlying cutaneous adnexal adenocarcinoma. Twelve percent of patients with extramammary Paget's disease have an associated concurrent underlying internal malignancy. The location of the underlying internal malignancy appears to be closely related to the location of the extramammary Paget's disease--i.e., a perianal location is associated with adenocarcinoma of the digestive system, a penile-scrotal-groin location with genitourinary malignancy, etc. A directed internal malignancy search may be of benefit in patients who are diagnosed as having extramammary Paget's disease.
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                Author and article information

                Journal
                Arch Plast Surg
                Arch Plast Surg
                APS
                Archives of Plastic Surgery
                The Korean Society of Plastic and Reconstructive Surgeons
                2234-6163
                2234-6171
                November 2014
                03 November 2014
                : 41
                : 6
                : 753-758
                Affiliations
                Department of Plastic and Reconstructive Surgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
                Author notes
                Correspondence: Hak Chang. Department of Plastic and Reconstructive Surgery, Seoul National University Hospital, Seoul National University College of Medicine, 103 Daehak-ro, Jongno-gu, Seoul 110-744, Korea. Tel: +82-2-2072-3086, Fax: +82-2-747-5130, hchang@ 123456snu.ac.kr
                Article
                10.5999/aps.2014.41.6.753
                4228221
                25396191
                ae73b4a9-0a85-4639-ba81-cbcbfb508ef8
                Copyright © 2014 The Korean Society of Plastic and Reconstructive Surgeons

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 10 June 2014
                : 03 July 2014
                : 14 July 2014
                Funding
                Funded by: National Research Foundation of Korea
                Award ID: R11-2005-065
                Categories
                Original Article

                Surgery
                paget disease, extramammary,mohs surgery,frozen sections
                Surgery
                paget disease, extramammary, mohs surgery, frozen sections

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