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Morphological, immunohistochemical, and chromosomal analysis of multicystic chromophobe renal cell carcinoma, an architecturally unusual challenging variant

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Abstract

Chromophobe renal cell carcinoma (ChRCC) is typically composed of large leaf-like cells and smaller eosinophilic cells arranged in a solid-alveolar pattern. Eosinophilic, adenomatoid/pigmented, or neuroendocrine variants have also been described. We collected 10 cases of ChRCC with a distinct multicystic pattern out of 733 ChRCCs from our registry, and subsequently analyzed these by morphology, immunohistochemistry, and array comparative genomic hybridization. Of the 10 patients, 6 were males with an age range of 50–89 years (mean 68, median 69). Tumor size ranged between 1.2 and 20 cm (mean 5.32, median 3). Clinical follow-up was available for seven patients, ranging 1–19 years (mean 7.2, median 2.5). No aggressive behavior was documented. We observed two growth patterns, which were similar in all tumors: (1) variable-sized cysts, resembling multilocular cystic neoplasm of low malignant potential and (2) compressed cystic and tubular pattern with slit-like spaces. Raisinoid nuclei were consistently present while necrosis was absent in all cases. Half of the cases showed eosinophilic/oncocytic cytology, deposits of pigment (lipochrome) and microcalcifications. The other half was composed of pale or mixed cell populations. Immunostains for epithelial membrane antigen (EMA), CK7, OSCAR, CD117, parvalbumin, MIA, and Pax 8 were positive in all tumors while negative for vimentin, TFE3, CANH 9, HMB45, cathepsin K, and AMACR. Ki67 immunostain was positive in up to 1 % of neoplastic cells. Molecular genetic examination revealed multiple chromosomal losses in two fifths analyzable tumors, while three cases showed no chromosomal numerical aberrations. ChRCC are rarely arranged in a prominent multicystic pattern, which is probably an extreme form of the microcystic adenomatoid pigmented variant of ChRCC. The spectrum of tumors entering the differential diagnosis of ChRCC is quite different from that of conventional ChRCC. The immunophenotype of ChRCC is identical with that of conventional ChRCC. Chromosomal numerical aberration pattern was variable; no chromosomal numerical aberrations were found in three cases. All the cases in this series have shown an indolent and non-aggressive behavior.

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Authors and Affiliations

  1. Department of Pathology, Bellvitge University Hospital, Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain

    Maria Pané Foix & Enric Condom Mundó

  2. Department of Pathology and Experimental Therapeutics, School of Medicine, University of Barcelona, Barcelona, Spain

    Maria Pané Foix & Enric Condom Mundó

  3. Department of Pathology, University of Split, Split, Croatia

    Ana Dunatov

  4. Department of Pathology, , Medical Faculty and Charles University Hospital Plzen, Charles University, Alej Svobody 80, 304 60, Pilsen, Czech Republic

    Petr Martinek, Kvetoslava Peckova, Krystina Pivovarcikova, Daum Ondrej, Pavla Rotterova, Martin Dusek, Michal Michal & Ondrej Hes

  5. Department of Pathology, Medical College of Wisconsin, Milwaukee, WI, USA

    Saul Suster

  6. Department of Pathology, East University, Riga, Latvia

    Maris Sperga

  7. Department of Pathology, Cruces University Hospital, Biocruces Research Institute, University of the Basque Country, Barakaldo, Spain

    Jose I. Lopez

  8. “Ljudevit Jurak” Pathology Department, Clinical Hospital Center “Sestre milosrdnice”, Zagreb, Croatia

    Monika Ulamec

  9. Department of Pathology, Clinical Hospital Center Zagreb, Zagreb, Croatia

    Stela Bulimbasic

  10. Department of Pathology, Instituto Nacional de Cancerologia, Mexico City, Mexico

    Delia Perez Montiel

  11. Department of Pathology, Faculty of Medicine, Royal Columbian Hospital, University of British Columbia, Vancouver, BC, Canada

    Reza Alaghehbandan

  12. Department of Pathology, Clinical Center of the University of Srajevo, Sarajevo, Bosnia and Herzegovina

    Faruk Skenderi

  13. Department of Urology, Medical Faculty and Charles University Hospital, Charles University, Plzen, Czech Republic

    Kristyna Prochazkova & Milan Hora

  14. Biomedical Centre, Faculty of Medicine in Lzen, Charles University in Prague, Plzen, Czech Republic

    Ondrej Hes

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  1. Maria Pané Foix
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Correspondence to Ondrej Hes.

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Study design has been approved by local ethical committee (Charles University, Medical School Plzen) LEK FN Plzeň.

Funding

The study was supported by the Charles University Research Fund (project number P36), by the project FN 00669806, and by SVV 260283.

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All authors declare no conflict of interest

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Foix, M.P., Dunatov, A., Martinek, P. et al. Morphological, immunohistochemical, and chromosomal analysis of multicystic chromophobe renal cell carcinoma, an architecturally unusual challenging variant. Virchows Arch 469, 669–678 (2016). https://doi.org/10.1007/s00428-016-2022-x

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