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      Clear Cell Carcinoma of the Breast: A Rare Breast Cancer Subtype - Case Report and Literature Review

      case-report
      * ,
      Case Reports in Oncology
      S. Karger AG
      Breast cancer, Glycogen-rich clear cell carcinoma

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          Abstract

          Background: Glycogen-rich clear cell breast carcinoma is a rare histological breast cancer subtype. Its prognosis may vary depending on specific clinical and pathological characteristics such as low grade, strong positivity of estrogen receptor (ER) expression and early diagnosis. Case Presentation: We present the case of a 53-year-old woman with a bleeding 10-cm-diameter mass in the left breast. The histological examination showed a poorly differentiated tumor with malignant cells characterized by abundant clear cytoplasm. The diagnosis of clear cell carcinoma was based on the histological characteristics of the tumor, and a nonmammary origin was initially ruled out. The tumor was triple negative [i.e. ER, progesterone receptor (PR) and HER2 negative]. Four months after the initial locoregional treatment, the patient developed lung and distant lymph node metastases. Conclusions: Glycogen-rich clear cell carcinoma of the breast is a rare tumor. Early diagnosis, absence of lymph node metastases and ER/PR positivity are associated with a better prognosis, as in other common breast cancer subtypes.

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          Most cited references17

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          Breast carcinoma--rare types: review of the literature.

          Invasive breast cancer is a heterogeneous disease in its presentation, pathological classification and clinical course. However, there are more than a dozen variants which are less common but still very well defined by the World Health Organization (WHO) classification. The rarity of many of these neoplasms does not allow large or randomized studies to define the optimal treatment. Many of the descriptions of these cancers are from case reports and small series. Our review brings updated information on 16 epithelial subtypes as classified by the WHO system with a very concise histopathology description and parameters helpful in the clinic. The aim of our review is to provide a tool for breast cancer caregivers which will enable a better understanding of the disease and its optimal approach to therapy. This may also stand as a clinical framework for a future understanding of these rarer breast cancers when gene analysis work is reported.
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            Clinical and pathological features of glycogen-rich clear cell carcinoma of the breast.

            Twenty cases of invasive ductal carcinoma of the breast with a pure or partial glycogen-rich clear cell carcinoma(GRCC)component are reported. GRCC of the breast is composed almost entirely of polygonal cells with clear cytoplasm. These contain large amounts of partly water-soluble glycogen. The cases were analyzed using various parameters, including age at presentation, tumor size, tumor grade, axillary lymph node and Her2/neu status. Between 1990 and 2004, 723 patients with primary breast carcinomas were treated and clinicopathologic analysis was performed. 20 cases were identified as GRCC among the 723 cases. The patients' age at presentation ranged from 33 to 68 years (mean, 52 years). Tumor size ranged from 1 to 6.5 cm (mean, 2.6 cm); 35% (7 of 20) of cases that underwent axillary dissection had positive lymph nodes. Among 15 of 20 cases who were followed for 1-72 months, 5 cases died from their breast carcinoma within 5 years following the diagnosis. Our series included more small size carcinomas than did previous series. Lymph node status does not appear to be markedly different from that of the usual invasive ductal carcinomas. Her2/neu expression was similar to that found in common breast carcinomas.
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              Glycogen-rich clear cell carcinoma of the breast. A clinicopathologic study of 21 cases.

              Twenty-one glycogen-rich clear cell carcinomas of the breast were studied. There were 13 invasive carcinomas and 8 intraductal carcinomas. Ten of the invasive carcinomas contained an intraductal component. Three intraductal carcinomas were papillary carcinomas ("intracystic" papillary carcinoma), and the others showed a mixture of solid, micropapillary, and cribriform patterns of growth. Most of the invasive carcinomas had nested, corded, or sheet-like growth patterns, and all were grade 2 or 3 neoplasms. Among patients with invasive carcinoma, two had metastases to axillary lymph nodes and three died of tumor. One patient with intraductal papillary carcinoma experienced two local recurrences, with invasion in the second recurrence. Thirteen cases (62%) exhibited focal apocrine features. Based on this small series, the clear cell morphology did not appear to influence the clinical outcome once stage and grade were taken into account. Glycogen-rich clear cell carcinoma of the breast may be a variant of apocrine carcinoma.
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                Author and article information

                Journal
                CRO
                CRO
                10.1159/issn.1662-6575
                Case Reports in Oncology
                S. Karger AG
                1662-6575
                2015
                September – December 2015
                04 November 2015
                : 8
                : 3
                : 472-477
                Affiliations
                Department of Medical Oncology, Oncology Institute of Southern Switzerland, Bellinzona, Switzerland
                Author notes
                *Vilma Ratti, Oncology Institute of Southern Switzerland (IOSI), Ospedale Beata Vergine, Via Alfonso Turconi 23, CH-6850 Mendrisio (Switzerland), E-Mail vilma.ratti@eoc.ch
                Article
                441838 PMC4649731 Case Rep Oncol 2015;8:472-477
                10.1159/000441838
                PMC4649731
                26600782
                d2b507a6-83ec-4b24-a2e8-d272f4b49035
                © 2015 S. Karger AG, Basel

                This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC). Usage and distribution for commercial purposes requires written permission. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                Page count
                Figures: 3, References: 15, Pages: 6
                Categories
                Published: November 2015

                Oncology & Radiotherapy,Pathology,Surgery,Obstetrics & Gynecology,Pharmacology & Pharmaceutical medicine,Hematology
                Breast cancer,Glycogen-rich clear cell carcinoma

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