Small cell variant of chromophobe renal cell carcinoma: Clinicopathologic and molecular-genetic analysis of 10 cases.

Authors

  • Joanna Rogala Department of Pathology, Charles University in Prague, Faculty of Medicine in Plzeň, Pilsen, Czech Republic https://orcid.org/0000-0001-5368-5021
  • Fumiyoshi Kojima Department of Human Pathology, Wakayama Medical University, Wakayama, Japan https://orcid.org/0000-0002-9478-9692
  • Reza Alaghehbandan Department of Pathology, Faculty of Medicine, University of British Columbia, Royal Columbian Hospital, Vancouver, BC, Canada https://orcid.org/0000-0002-6708-5032
  • Nikola Ptakova Department of Biology and Medical Genetics, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, Czech Republic https://orcid.org/0000-0002-2561-4717
  • Ana Bravc Department of Pathology, General Hospital, Slovenj Gradec, Slovenia https://orcid.org/0000-0001-7365-4521
  • Stela Bulimbasic Department of Pathology, University Hospital Centre Zagreb, Zagreb, Croatia
  • Delia Perez Montiel Department of Pathology, Institute Nacional de Cancerologia, Mexico City, Mexico https://orcid.org/0000-0002-9338-2284
  • Maryna Slisarenko Department of Pathology, Charles University in Prague, Faculty of Medicine in Plzeň, Pilsen, Czech Republic https://orcid.org/0000-0002-5221-4735
  • Leila Ali Department of Pathology, 'Carol Davila' University of Medicine and Pharmacy, Bucharest, Romania https://orcid.org/0000-0002-7857-6208
  • Levente Kuthi Department of Pathology, University of Szeged, Szeged, Hungary https://orcid.org/0000-0001-9247-6679
  • Kristyna Pivovarcikova Department of Pathology, Charles University in Prague, Faculty of Medicine in Plzeň, Pilsen, Czech Republic https://orcid.org/0000-0002-9553-0105
  • Kvetoslava Michalova Department of Pathology, Charles University in Prague, Faculty of Medicine in Plzeň, Pilsen, Czech Republic https://orcid.org/0000-0003-3231-6870
  • Boris Bartovic Departmnet of Pathology, Cytopathos, Bratislava, Slovakia https://orcid.org/0000-0003-3488-6586
  • Adriena Bartos Vesela Department of Urology, Charles University in Prague, Faculty of Medicine in Plzeň, Pilsen, Czech Republic https://orcid.org/0000-0003-3770-2439
  • Olga Dolejsova Department of Urology, Charles University in Prague, Faculty of Medicine in Plzeň, Pilsen, Czech Republic https://orcid.org/0000-0002-5936-4955
  • Michal Michal Department of Pathology, Charles University in Prague, Faculty of Medicine in Plzeň, Pilsen, Czech Republic
  • Ondrej Hes Department of Pathology, Charles University in Prague, Faculty of Medicine in Plzeň, Pilsen, Czech Republic https://orcid.org/0000-0002-3236-3127

DOI:

https://doi.org/10.17305/bjbms.2021.6935

Keywords:

kidney, chromophobe renal cell carcinoma, small cell variant

Abstract

The morphologic diversity of chromophobe renal cell carcinoma (ChRCC) is well-known. Aside from typical morphology, pigmented adenomatoid, multicystic and papillary patterns have been described. Ten cases of CHRCC composed of small cell population in various percentages were analysed, using morphologic parameters, immunohistochemistry and next-generation sequencing (NGS) testing. Patients were five males and five females, with age ranging from 40 to 78years. The size of tumors ranged from 2.2 cm to 11 cm (mean 5.17 cm). Small cell component comprised 10 to 80% of the tumor volume, while the remaining was formed by cells with classic ChRCC morphology. The immunohistochemical profile of the small cell component was consistent with typical ChRCC immunophenotype, with CD117 and CK7 positivity. Neuroendocrine markers were negative. Mutations of 13 genes were found: DCIER1, FGFR3, JAK3, SUFO, FAM46C, FANCG, MET, PLCG2, APC, POLE, EPICAM, MUTYH and AR. However, only the PLCG2 mutation is considered pathogenic.The small cell variant of ChRCC further highlights and expand upon existing morphologic heterogeneity spectrum. Recognition of small cell variant of CHRCC is not problematic in tumors, where the “classic” CHRCC component is present. However, in limited material (i.e., core biopsy), this may present a diagnostic challenge. Based on the limited follow-up data available, it appears that the small cell tumor component had no impact on prognosis, since there was no aggressive behavior documented.   Awareness of this unusual pattern and applying additional sections to find classic morphology of ChRCC, as well as excluding neuroendocrine nature by immunohistochemistry, may help resolve difficult cases.

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Small cell variant of chromophobe renal cell carcinoma: Clinicopathologic, and molecular-genetic analysis of 10 cases.

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Published

2022-03-10

How to Cite

1.
Rogala J, Kojima F, Alaghehbandan R, Ptakova N, Bravc A, Bulimbasic S, Perez Montiel D, Slisarenko M, Ali L, Kuthi L, Pivovarcikova K, Michalova K, Bartovic B, Bartos Vesela A, Dolejsova O, Michal M, Hes O. Small cell variant of chromophobe renal cell carcinoma: Clinicopathologic and molecular-genetic analysis of 10 cases. Bosn J of Basic Med Sci [Internet]. 2022Mar.10 [cited 2022Jul.4];. Available from: https://bjbms.org/ojs/index.php/bjbms/article/view/6935

Issue

Section

Pathology