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      OncoTargets and Therapy (submit here)

      This international, peer-reviewed Open Access journal by Dove Medical Press focuses on the pathological basis of cancers, potential targets for therapy and treatment protocols to improve the management of cancer patients. Publishing high-quality, original research on molecular aspects of cancer, including the molecular diagnosis, since 2008. Sign up for email alerts here. 50,877 Monthly downloads/views I 4.345 Impact Factor I 7.0 CiteScore I 0.81 Source Normalized Impact per Paper (SNIP) I 0.811 Scimago Journal & Country Rank (SJR)

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      Prognostic significance of mammary Paget’s disease in Chinese women: a 10-year, population-based, matched cohort study

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          Abstract

          Purpose

          To explore the prognostic significance of mammary Paget’s disease (PD) in breast cancer (BC) patients and to investigate the association between clinical manifestation and outcome in invasive ductal carcinoma patients with PD (PD-IDC).

          Patients and methods

          Eighty-five patients diagnosed with mammary PD with underlying BC from 2006 to 2012 at Zhejiang Cancer Hospital were recruited. A matched group comprised 85 patients diagnosed with BC without PD. Patients were matched according to four variables: stage (0–IV), age at diagnosis (within 5 years), histologic subtype, and the year of surgery. The 74 patients diagnosed with PD-IDC were divided into three groups based on their clinical presentation.

          Results

          Compared with the matched group, the PD group had more HER2 positivity ( P<0.01) and hormone receptor negativity ( P<0.01), and a worse outcome (Kaplan–Meier analysis, P<0.001 for disease-free survival and P=0.002 for overall survival). Multivariate Cox regression analyses showed that PD was an independent prognostic predictor for BC patients with PD. In addition, the 22 PD-IDC patients who presented with skin lesions in the nipple/areola and a mass in the breast or axilla had a higher risk of disease relapse than patients who presented with a mass in the breast without skin lesions or patients who presented with skin changes without a palpable mass (adjusted hazards ratio, 0.24; 95% CI, 0.08–0.73; P=0.012 and adjusted hazard ratio, 0.30; 95% CI, 0.06–1.40; P=0.124, respectively).

          Conclusion

          PD is an independent prognostic indicator of outcome in BC patients with PD. Furthermore, the primary symptoms at presentation may be an available indicator of prognosis in PD-IDC.

          Most cited references22

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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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            Paget disease of the nipple: a multifocal manifestation of higher-risk disease.

            The treatment of Paget disease by mastectomy has been challenged recently in favor of breast-conserving techniques. A large series of patients treated with mastectomy has been reviewed to assess the feasibility of less radical surgery. The cases of 70 women with a clinical diagnosis of Paget disease were reviewed. The type, grade, receptor and node status, and the mammographic and pathologic extent of the underlying breast malignancy were determined. The survival of patients with invasive disease was compared with matched controls without Paget disease. The underlying malignancy was invasive in 58% of cases. Despite the fact that only one third of women presented with a palpable mass, the malignancy was frequently extensive, being confined to the retroareolar region in only 25% of cases. The true extent of the disease was underestimated by mammography in 43% of cases. Of the patients with ductal in situ carcinoma, 96.5% had high-grade carcinomas and 100% had invasive carcinomas of high cytonuclear grade. Overexpression of the c-erb-B2 oncogene was detectable in 83% of cases. Patients with Paget disease had a significantly worse survival than matched controls, but this difference was eliminated if they were also matched for c-erb-B2 status. Paget disease is often associated with extensive underlying malignancy, which is difficult to assess accurately either clinically or mammographically. As a consequence, cone excision of the nipple would have resulted in incomplete excision in 75% of cases. The underlying disease is of high grade and is frequently c-erb-B2 positive with a resulting poor prognosis. Aggressive local and systemic treatment would seem to be merited. Copyright 2002 American Cancer Society.
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              Paget disease of the breast: mammographic, US, and MR imaging findings with pathologic correlation.

              Paget disease is a rare malignancy of the breast characterized by infiltration of the nipple epidermis by adenocarcinoma cells. The clinical features of Paget disease are characteristic and should increase the likelihood of the diagnosis being made. An important point is that more than 90% of cases of Paget disease are associated with an additional underlying breast malignancy. Paget disease is frequently associated with ductal carcinoma in situ (DCIS) in the underlying lactiferous ducts of the nipple-areolar complex; it may even be associated with DCIS or invasive breast cancer elsewhere in the breast, at least 2 cm from the nipple-areolar complex. Nevertheless, mammographic findings may be negative in up to 50% of cases. Magnetic resonance (MR) imaging can be useful in patients with Paget disease for evaluation of the nipple-areolar complex and identification of an additional underlying malignancy in the breast. The appropriate surgical treatment must be carefully selected and individualized on the basis of radiologic findings, especially those obtained with breast MR imaging.
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                Author and article information

                Journal
                Onco Targets Ther
                Onco Targets Ther
                OncoTargets and Therapy
                OncoTargets and therapy
                Dove Medical Press
                1178-6930
                2018
                23 November 2018
                : 11
                : 8319-8326
                Affiliations
                [1 ]Department of Medical Oncology, Zhejiang Cancer Hospital, Hangzhou, Zhejiang, People’s Republic of China, wangxj@ 123456zjcc.org.cn ; czred@ 123456sina.com
                [2 ]Department of Medical Oncology, Shaoxing Second Hospital, Shaoxing, Zhejiang, People’s Republic of China
                [3 ]State Key Laboratory for Diagnosis and Treatment of Infectious Diseases, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, People’s Republic of China
                Author notes
                Correspondence: Xiaojia Wang; Zhanhong Chen, Department of Medical Oncology, Zhejiang Cancer Hospital, Hangzhou, Zhejiang, People’s Republic of China, Tel +86 139 0650 0190; +86 136 0650 5124, Email wangxj@ 123456zjcc.org.cn ; czred@ 123456sina.com
                Article
                ott-11-8319
                10.2147/OTT.S171710
                6260180
                30538501
                e60fe1a9-edb6-463d-98f0-a562b5fe23d5
                © 2018 Zhou et al. This work is published and licensed by Dove Medical Press Limited

                The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License ( http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.

                History
                Categories
                Original Research

                Oncology & Radiotherapy
                paget’s disease,invasive ductal carcinoma,prognosis,clinical presentation

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