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      Surgical Treatment of Brain Metastasis of Extramammary Paget’s Disease: A Case Report

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          Abstract

          Extramammary Paget’s disease (EMPD) is a rare form of neoplasm. Metastasis of EMPD to locations other than lymph nodes and intra-epithelial regions is rare; there are a limited number of case reports of metastases to the liver, lung, bone, and brain. We present a rare case of EMPD that metastasized to the brain and was treated with surgical resection. A 66-year-old man presented with a small palpable mass in the scrotum. After 5 years of observation, he was diagnosed with EMPD that metastasized to the lymph nodes and lung. Tumor resection and postoperative chemotherapy were performed. Six months after the last chemotherapy treatment, he presented with a right temporal lobe tumor and underwent surgical resection. Histopathological analysis revealed brain metastasis of EMPD. Three months after surgery, magnetic resonance imaging (MRI) showed local tumor recurrence, and intensity modulated radiation therapy (IMRT) (45 Gy/15 Fr) was performed. Although the metastatic brain tumor was well controlled, the primary tumor progressed. He was provided best supportive care and died 5 months after brain tumor resection. In this report, we present a rare case of brain metastasis of EMPD, treated with surgical resection, and histopathologically confirmed to be metastatic EMPD.

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          Extramammary Paget's disease: Summary of current knowledge.

          Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy accounting for approximately 1% of vulvar cancers. The rarity of this disease has caused difficulties in its characterization. Controversies exist in the literature regarding many aspects of this condition including the prevalence of concurrent vulvar adenocarcinoma or invasive EMPD, association with regional and distant cancers, and recurrence rates following surgical excision. This extensive review takes a closer look at what is known about EMPD and the conclusions that have been drawn from this information. This article also provides a practical approach to patients with EMPD. Copyright © 2010 Elsevier Inc. All rights reserved.
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            Extramammary Paget's disease: a review of the literature.

            Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy. The disease typically affects older individuals aged 60-80 years and is seen most frequently in postmenopausal Caucasian women and Asian men. EMPD exhibits a predilection for the genital and perianal regions and may be associated with an underlying carcinoma in adjacent organs. EMPD presents a challenge in both diagnosis and management. Often treated empirically as various dermatitides, the correct diagnosis is frequently delayed by many years. Following diagnosis, an extensive search for an associated malignancy should be initiated. If invasive disease is present on biopsy, a sentinel lymph node biopsy may guide further treatment. Mohs micrographic surgery appears to be superior to wide local excision when considering tissue sparing ability and disease recurrence. Nonsurgical interventions have also been investigated with varied results. Regardless of treatment method, long-term follow-up is recommended to monitor for local disease recurrence, development of internal malignancy, regional lymphadenopathy, or distant metastasis.
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              Extramammary Paget's disease.

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                Author and article information

                Journal
                NMC Case Rep J
                NMC Case Rep J
                NMCCRJ
                NMC Case Report Journal
                The Japan Neurosurgical Society
                2188-4226
                September 2020
                17 September 2020
                : 7
                : 4
                : 189-193
                Affiliations
                [1 ] Department of Neurosurgery, Osaka National Hospital, National Hospital Organization, Osaka, Osaka, Japan
                [2 ] Department of Neurosurgery, Osaka University, Graduate School of Medicine, Suita, Osaka, Japan
                [3 ] Department of Dermatology, Osaka National Hospital, National Hospital Organization, Osaka, Osaka, Japan
                [4 ] Department of Central Laboratory and Surgical Pathology, Osaka National Hospital, National Hospital Organization, Osaka, Osaka, Japan
                Author notes
                Corresponding author: Noriyuki Kijima, MD, PhD, Department of Neurosurgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan. n-kijima@ 123456nsurg.med.osaka-u.ac.jp
                Article
                nmccrj-7-189
                10.2176/nmccrj.cr.2019-0292
                7538455
                6e81d880-f8ca-4066-a3c3-3b7b17d6cfd1
                © 2020 The Japan Neurosurgical Society

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by-nc-nd/4.0/

                History
                : 07 January 2020
                : 04 March 2020
                Categories
                Case Report

                extramammary paget’s disease,metastasis
                extramammary paget’s disease, metastasis

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