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      Extramammary Paget's disease with the appearance of a nodule: a case report

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      Author(s): 1 , 1 , , 1
      Publication date (Electronic):
      Journal: BMC Cancer
      Publisher: BioMed Central

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          Abstract

          Background

          Extramammary Paget's disease (EMPD) remains a rare condition with only a limited number of cases reported in the literature. EMPD is mainly composed of intraepidermal Paget cells, and possesses variable clinical behaviors and histological appearances, leading to difficulty in the diagnosis of this disease.

          Case presentation

          We here report a case of primary EMPD with the appearance of a nodule on the background of erythema. Histological assessment showed Paget cell infiltration throughout the epidermis with dermal spread. Using immunohistochemistry, the expressions of CK7, CK19, CK20, GCDFP-15, CEA, S-100 protein and bcl-2 were examined to elucidate the cellular differentiation of the carcinoma.

          Conclusion

          According to the histological assessment, this case was diagnosed as primary EMPD with carcinoma cells invading into the dermis, but without lymph node infiltration.

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          Most cited references14

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          Mammary and extramammary Paget's disease.

          Farrell J. Lloyd, Lisa Flanagan (2000)
          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.

            M. Yamada, R Morita, N Hatta … (2008)
            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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              The Roswell Park Cancer Institute experience with extramammary Paget's disease.

              N Zeitouni, J Zollo (1999)
              Extramammary Paget's disease (EMPD) is a rare intraepithelial neoplasm. Common sites of occurrence include the vulva, perianal region, perineum and scrotum. Despite frequent recurrences, surgery is the standard treatment. This study examines the recurrence rate for EMPD treated by conventional surgical management. Alternative and multimodal therapeutic approaches are reviewed. This retrospective analysis included all 30 patients treated for EMPD at Roswell Park Cancer Institute (RPCI) between 1970 and 1998. Following conventional surgical treatment, 44% of our patients developed recurrence. Vulvectomy provided the lowest recurrence rate, but involved extensive tissue loss and functional debility. Multimodal treatment using Mohs' micrographic surgery and photodynamic therapy has been used at RPCI to manage EMPD with minimal tissue loss and no functional impairment. Surgical treatment offers a moderate chance of EMPD cure. Long-term multimodal approaches require close follow-up, but may conserve both tissue and function.
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                Author and article information

                Journal
                Journal ID (nlm-ta): BMC Cancer
                Title: BMC Cancer
                Publisher: BioMed Central
                ISSN (Electronic): 1471-2407
                Publication date Collection: 2010
                Publication date (Electronic): 4 August 2010
                Volume: 10
                Page: 405
                Affiliations
                [1 ]Department of Dermatology, the Second Affiliated Hospital of Guangzhou Medical University, Guangzhou 510260, P. R. China
                Article
                Publisher ID: 1471-2407-10-405
                DOI: 10.1186/1471-2407-10-405
                PMC ID: 2921398
                PubMed ID: 20684770
                SO-VID: c2512860-3d8e-4b32-acc9-7cd16f4461af
                Copyright © Copyright ©2010 Wang et al; licensee BioMed Central Ltd.
                License:

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 20 March 2010
                Date accepted : 4 August 2010
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Oncology & Radiotherapy
                Data availability:
                ScienceOpen disciplines: Oncology & Radiotherapy

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