The World Health Organization (WHO) defines glycogen-rich clear cell carcinoma (GRCC) of the breast as a carcinoma with glycogen accumulation in more than 90% of its tumor cells. Due to the rarity of this disease, its reported survival and clinical associations have been inconsistent due to reliance on case reports and limited case series. As a result, the prognostic implication of this cancer subtype remains unclear. Using the U.S. Surveillance, Epidemiology, and End Results (SEER) program database, we compared the incidence, demographics and prognostic factors of 155 cases of GRCC of the breast to 1,251,584 cases of other (non-GRCC) breast carcinomas. We demonstrate that GRCC is more likely to be identified as high grade, advanced stage, and more likely to have triple negative receptor status. GRCC cases display a poorer prognosis than non-GRCC carcinomas of the breast irrespective of age, AJCC staging, tumor grade, joint hormone receptor/human epidermal growth factor receptor 2 (HER2) status, and treatment. Similar to non-GRCC carcinomas, older age and higher American Joint Committee on Cancer (AJCC)/TNM staging were associated with poorer prognosis for GRCC, while treatment with surgery and radiation were associated with improved survival. Radiation, specifically in the setting of breast-conserving surgery, further improved survival compared to surgery alone. Our study highlights the poorer prognosis associated with glycogen accumulation in breast cancers and hence stresses the importance of identifying this more aggressive tumor type.

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Published in Journal of Clinical Medicine, v. 8, issue 2, 246, p. 1-14.

© 2019 by the authors. Licensee MDPI, Basel, Switzerland.

This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).

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Funding Information

This study was supported by the University of Kentucky Center for Cancer and Metabolism, National Institute of General Medical Sciences COBRE program (grant ID: P20 GM121327). Additionally, R.S. is aided by grant #16-182-28 from the American Cancer Society and funding from the University of Kentucky Markey Cancer Center. C.H. is supported by the University of Kentucky Markey Cancer Center (P30CA177558). Z.Z. is supported by the NIH National Center for Advancing Translational Sciences (grant number: UL1TR001998).

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The following are available online at https://www.mdpi.com/2077-0383/8/2/246/s1, Supplemental Table S1. Demographical and clinical characteristics of GR subgroup. Supplemental Table S2. Survival duration in the GR subgroup. Supplemental Table S3. Demographical and clinical characteristics of CC subgroup. Supplemental Table S4. Survival duration in the CC subgroup. Supplemental Table S5. Multivariable analysis of overall survival for CC carcinomas. Supplemental Table S6. Comparison of demographical and clinical characteristics between GR and CC carcinomas. Supplemental Figure S1. Kaplan-Meier survival curves for (A) overall survival and (B) with or without radiation therapy after sub-mastectomy in the GR subgroup. Supplemental Figure S2. Kaplan-Meier survival curves for CC subgroup. Supplemental Figure S3. Kaplan-Meier survival curves comparing GR and CC carcinomas.

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Supplementary Materials