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      CLINICOPATHOLOGIC EVALUATION OF MAMMARY PAGET'S DISEASE

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          Abstract

          Mammary and extramammary Paget's diseases are rare neoplasms of epidermis and mucosal epithelium. Due to their nonspecific and variable clinical view, they have differential diagnosis with eczema, melanoma, Bowen's disease, etc. To the best of our knowledge, no such study has been performed in Iran regarding the prevalence, clinical aspects, underlying disease and pathological characteristics of these two diseases. In this study, we have evaluated the clinical and histopathological aspects of this disorder.

          Materials and Methods:

          In this retrospective study, all Paget's biopsied samples referred to the Pathology Department of Imam-Reza hospital, Mashhad, since 1984 till 2004 were evaluated. Collected data were analyzed by descriptive statistical methods.

          Results:

          Among 98925 specimens, there were 29 cases of Paget's disease. All cases were married women suffering from mammary Paget. The mean age was 53 ± 11 years. Left and right breast involvement was observed in 17 and 12 cases, all unilateral. The most common clinical view was ulcerated (27%) and then erythematosus exudative plaques. More than 50% of patients were symptomatic. Most common symptoms were itching, pain and burning. The exclusive underlying pathological diagnosis was ductal carcinoma (55%).

          Discussion:

          In most cases, the clinical view of mammary Paget's disease was helpful. Unilateral ulcerated plaque was the most common clinical sign. Majority of the accompanying pathology was ductal carcinoma. We had no cases of extramammary Paget's disease in our study.

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          Most cited references18

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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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            Mammary and extramammary Paget's disease.

            Paget's disease is an intra-epidermal adenocarcinoma seen over the nipple/areola (mammary Paget's disease) or in extramammary body zones, such as the anogenital and perineal skin and the axilla. Mammary and extramammary Paget's disease share many common clinicopathological features but also show several differences, namely, with regard to pathogenesis and association with underlying malignancies. Indeed, mammary Paget's disease is as a rule associated with an underlying breast carcinoma whereas association of extramammary Paget's disease with underlying (skin or visceral) malignancies occurs much less frequently. We review here the main clinicopathological and therapeutic features of mammary and extramammary Paget's disease.
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              Mammary and extramammary Paget's disease: an immunohistochemical study of 83 cases.

              Mammary Paget's disease (MPD) and extramammary Paget's disease (EMPD) are rare neoplasms. The aim of this study was, by the use of immunohistochemistry, to derive further information about the cell(s) of origin, find a diagnostically useful immunohistochemical panel and investigate candidates for possible targeted therapy. Sixty MPD and 23 EMPD cases were studied using antibodies to cytokeratin (CK) 34betaE12, CK8/18, CK7, CK5/6, CK20, gross cyctic disease fluid protein (GCDFP)-15, MUC1-8, epidermal growth factor receptor (EGFR) (HER1), HER3 and HER4. In all MPD cases CK7 and MUC1 were positive. CK8/18 was positive in 59/60 cases. GCDFP-15, MUC2, MUC3, MUC4, MUC7, MUC8 were positive in 29/60, 3/60, 35/47, 4/40, 3/43 and 2/45 cases, respectively. In all EMPD cases CK8/18 and CK7 were positive. MUC1, GCDFP-15, MUC5AC, MUC3, MUC8 and CK20 were positive in 22/23, 19/23, 8/19, 3/19, 1/19 and 3/23 cases, respectively. With the remaining antibodies no immunoreactivity was observed. MUC1 and low-molecular-weight CKs in conjunction with immunonegativity for high-molecular-weight CKs are the most diagnostically useful markers. MPD is caused by the epidermotropic spread of underlying tumour cells, whereas EMPD probably arises from intraepithelial cells of sweat gland origin. Targeted therapy with antibodies against EGFR (HER1), HER3 or HER4 is unlikely to prove of clinical value.
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                Author and article information

                Journal
                Indian J Dermatol
                IJD
                Indian Journal of Dermatology
                Medknow Publications (India )
                0019-5154
                1998-3611
                Jan-Mar 2008
                : 53
                : 1
                : 21-23
                Affiliations
                From the Pathology Department Imam-Reza Educational Hospital, Mashhad University of Medical Sciences, Mashhad, Iran
                [1 ] From the Dermatology, Department Imam-Reza Educational Hospital, Mashhad University of Medical Sciences, Mashhad, Iran
                Author notes
                Address correspondence to: Dr. Naser Tayyebi Meibodi, Pathology Department, Imam-Reza educational hospital, Mashhad University of Medical Sciences (MUMS), Mashhad, Iran. E-mail: naser_tayyebi@ 123456yahoo.com
                Article
                IJD-53-21
                10.4103/0019-5154.39736
                2784580
                19967013
                5c6acec6-c285-4cdd-8716-773b84ca4ab1
                © Indian Journal of Dermatology

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : June 2007
                : July 2007
                Categories
                Clinicopathological Colloquium

                Dermatology
                intraepidermal neoplasia,extramammary,mammary,paget's disease
                Dermatology
                intraepidermal neoplasia, extramammary, mammary, paget's disease

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