Glycogen-Rich Clear Cell Carcinoma of the Breast: Report of Two New Cases and an Updated Literature Review
DOI:
https://doi.org/10.5644/ama2006-124.487Keywords:
Breast Cancer, Special Patterns, Glycogen-Rich Pattern, Biomarkers, OutcomeAbstract
Objective. To report two additional cases of glycogen-rich clear cell carcinoma (GRCC) of the breast – detailing their clinicopathologic features, immunophenotypes, and follow-up – and to provide an updated literature review since 2020.
Case Reports. Two patients (66 and 52 years old) had GRCC confirmed morphologically and histochemically. Case 1 was ER-positive/HER2- positive (luminal B/HER2-positive) and was managed with surgery, followed by adjuvant chemotherapy, endocrine therapy, and anti-HER2 therapy (trastuzumab). Case 2 was triple-negative and received neoadjuvant chemoimmunotherapy (pembro- lizumab-based) with marked pathologic tumor regression at resection. Both patients were disease-free at one and 12 months, respectively.
Conclusions. GRCC is heterogeneous and should not be regarded as a single clinicopathologic entity within invasive breast carcinoma of no special type or assumed to have a uniform prognosis. Management should be biomarker-guided, as illustrated by these cases. The role of targeted and immune therapies in GRCC warrants multi-institutional studies.
References
Siegel RL, Giaquinto AN, Jemal A. Cancer statistics, 2024. CA Cancer J Clin. 2024;74(1):12-49.
WHO Classification of Tumours: Breast Tumours. 5th edition ed. Lyon: International Agency for Research on Cancer; 2019.
Wu Y, Shin SJ, Sahin AA. Special Histologic Types and Special Morphologic Patterns of Invasive Ductal Carcinoma of No Special Type: Mucinous, Micropapillary, Mu- cinous Cystadenocarcinoma, Neuroendocrine Neoplasm, Cystic Hypersecretory, Glycogen-Rich Clear Cell, Carcinoma with Osteoclast-Like Giant Cells. A Comprehensive Guide to Core Needle Biopsies of the Breast, Second Edition 2022. p. 575-614.
Vranic S, Skenderi F, Beslagic V, Gatalica Z. Glycogen-rich Clear Cell Carcinoma of the Breast: A Comprehensive Review. Appl Immunohistochem Mol Morphol. 2020;28(9):655-60.
Vranic S, Schmitt F, Sapino A, Costa JL, Reddy S, Castro M, et al. Apocrine carcinoma of the breast: a comprehensive review. Histol Histopathol. 2013;28(11):1393-409.
Vranic S, Gatalica Z. An Update on the Molecular and Clinical Characteristics of Apocrine Carcinoma of the Breast. Clin Breast Cancer. 2022;22(4):e576-e85.
Hull MT, Warfel KA. Glycogen-rich clear cell carcino- mas of the breast. A clinicopathologic and ultrastructural study. Am J Surg Pathol. 1986;10(8):553-9.
Hull MT, Priest JB, Broadie TA, Ransburg RC, McCarthy LJ. Glycogen-rich clear cell carcinoma of the breast: a light and electron microscopic study. Cancer. 1981;48(9):2003-9.
Zhou Z, Kinslow CJ, Hibshoosh H, Guo H, Cheng SK, He C, et al. Clinical Features, Survival and Prognostic Factors of Glycogen-Rich Clear Cell Carcinoma (GRCC) of the Breast in the U.S. Population. J Clin Med. 2019;8(2).
Skenderi F, Palazzo J, Swensen J, Feldman R, Contreras E, Florento E, et al. Novel targetable biomarkers in clear cell carcinoma of the breast uncovered by molecular profiling: A study of nine cases. Breast J. 2020;26(9):1781-3.
Solanki MH, Derylo AF, Jorns JM. Invasive Mammary Car- cinoma With Mixed Invasive Papillary and Glycogen Rich Clear Cell Features. Int J Surg Pathol. 2018;26(6):569-72.
Toikkanen S, Joensuu H. Glycogen-rich clear-cell carcinoma of the breast: a clinicopathologic and flow cytometric study. Hum Pathol. 1991;22(1):81-3.
Demyashkin GA, Ryzhov AO, Sidorin AV, Gevandova MG, Shchekin VI, Kolyada AP. Immunophenotype characteristics of glycogen-rich clear cell breast carcinoma. Medical News of North Caucasus. 2021;16(3):303-5.
Wolff AC, Somerfield MR, Dowsett M, Hammond MEH, Hayes DF, McShane LM, et al. Human Epidermal Growth Factor Receptor 2 Testing in Breast Cancer. Arch Pathol Lab Med. 2023;147(9):993-1000.
Allison KH, Hammond MEH, Dowsett M, McKernin SE, Carey LA, Fitzgibbons PL, et al. Estrogen and Progesterone Receptor Testing in Breast Cancer: Ameri- can Society of Clinical Oncology/College of American Pathologists Guideline Update. Arch Pathol Lab Med. 2020;144(5):545-63.
Salgado R, Denkert C, Demaria S, Sirtaine N, Klauschen F, Pruneri G, et al. The evaluation of tumor-infiltrating lymphocytes (TILs) in breast cancer: recommendations by an International TILs Working Group 2014. Ann On- col. 2015;26(2):259-71.
Bossuyt V, Provenzano E, Symmans WF, Boughey JC, Coles C, Curigliano G, et al. Recommendations for standardized pathological characterization of residual disease for neoadjuvant clinical trials of breast cancer by the BIG- NABCG collaboration. Ann Oncol. 2015;26(7):1280-91.
Provenzano E. Neoadjuvant Chemotherapy for Breast Cancer: Moving Beyond Pathological Complete Response in the Molecular Age. Acta Med Acad. 2021;50(1):88-109.
Sanjeeviah RC, Bandimegal M, Ramaswamy V, Telkar KG, Patil D. Excellent long term oncological outcome in a patient with rare glycogen rich clear cell carcinoma of breast following breast conservation surgery. Int J Surg Case Rep. 2022;99:107640.
Georgescu TA, Munteanu O, Lisievici AC, Tebeica T, Cre- toiu D, Toader O, et al. Glycogen-rich clear cell carcinoma of the breast with solid papillary pattern: Two cases with heterogeneous clinicopathological features. Exp Ther Med. 2021;21(5):524.
Singh GR, Kumari M, Sunny K, Haldar D, Kumar M, Prasad R. Interesting Breast Tumours: A Tripod of Cases. Niger Med J. 2024;65(2):222-30.
De la Sancha C, Ruiz-Cordero R, Popnikolov N. Genetic Alterations in Invasive Breast Carcinoma with a Glycogen-Rich Clear Cell Pattern: A Case Report. Case Rep Oncol. 2021;14(1):500-5.
Lee N, Tran Y, Farshid G. Invasive breast carcinoma of no special type with glycogen-rich clear cell pattern. Pathol- ogy. 2022;54:S50-S1.
Liu Y, Niu S. Analysis of clinicopathologic features of glycogen-rich clear cell carcinoma (GRCC) of the breast. Virchows Archiv. 2020;477:S227-S.
Braganza JS, Mohamed B, Presendieu C, Pierre NL, Thieme H. Management of Glycogen-Rich Clear Cell Carcinoma of the Breast: A Case Report. Cureus. 2025;17(9):e93429.
Fisher ER, Tavares J, Bulatao IS, Sass R, Fisher B. Glycogen-rich, clear cell breast cancer: with comments concerning other clear cell variants. Hum Pathol. 1985;16(11):1085-90.
Hayes MM, Seidman JD, Ashton MA. Glycogen-rich clear cell carcinoma of the breast. A clinicopathologic study of 21 cases. Am J Surg Pathol. 1995;19(8):904-11.
Kuroda H, Sakamoto G, Ohnisi K, Itoyama S. Clinical and pathological features of glycogen-rich clear cell carcino- ma of the breast. Breast Cancer. 2005;12(3):189-95.
Di Tommaso L, Pasquinelli G, Portincasa G, Santini D. [Glycogen-rich clear-cell breast carcinoma with neuroendocrine differentiation features]. Pathologica. 2001;93(6):676-80.
Eun NL, Cha YJ, Son EJ, Gweon HM, Kim JA, Youk JH. Clinical Imaging of Glycogen-rich Clear Cell Carcinoma of the Breast: A Case Series with Literature Review. Magn Reson Med Sci. 2019;18(3):238-42.
Ratti V, Pagani O. Clear Cell Carcinoma of the Breast: A Rare Breast Cancer Subtype - Case Report and Literature Review. Case Rep Oncol. 2015;8(3):472-7.
Ma X, Han Y, Fan Y, Cao X, Wang X. Clinicopathologic characteristics and prognosis of glycogen-rich clear cell carcinoma of the breast. Breast J. 2014;20(2):166-73.
Akbulut M, Zekioglu O, Kapkac M, Erhan Y, Ozdemir N. Fine needle aspiration cytology of glycogen-rich clear cell carcinoma of the breast: review of 37 cases with histologic correlation. Acta Cytol. 2008;52(1):65-71.
Chen X, Yang N, Wang Y, Yang S, Peng Y. PCK1-mediated glycogenolysis facilitates ROS clearance and chemo- therapy resistance in cervical cancer stem cells. Sci Rep. 2024;14(1):13670.
Schmid P, Cortes J, Dent R, Pusztai L, McArthur H, Kum- mel S, et al. Event-free Survival with Pembrolizumab in Early Triple-Negative Breast Cancer. N Engl J Med. 2022;386(6):556-67.
Kurtovic-Kozaric A, Vranic S, Kurtovic S, Hasic A, Kozaric M, Granov N, et al. Lack of Access to Targeted Cancer Treatment Modalities in the Developing World in the Era of Precision Medicine: Real-Life Lessons From Bosnia. J Glob Oncol. 2018;4:1-5.
Paric A, Karan-Krizanac D, Saric I, Vranic S. A Chromo- some 9p24.1 Amplification in Colorectal Cancer with Metastases to the Kidney and Adrenal Gland: A Case Report. Case Rep Oncol. 2023;16(1):803-10.
Tomic K, Karan Krizanac D, Skenderi F, Krpina K, Carapina Bilic A, Galic K, et al. Comprehensive genomic profiling of a metastatic small cell lung carcinoma with a complete and long-term response to atezolizumab: A case report. Respir Med Case Rep. 2023;45:101920.
Li M, Ka D, Chen Q. Disparities in availability of new cancer drugs worldwide: 1990-2022. BMJ Glob Health. 2024;9(9).
Tomic K, Begagic E, Voloder E, Naletilic MP, Jozic GB, Cale S, et al. Outcomes of extensive-stage small cell lung cancer treatment in a real-world clinical setting: a single-center experience. Contemp Oncol (Pozn). 2025;29(3):271-80.
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