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.

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

Department of Pathology and Experimental Therapeutics School of Medicine University of Barcelona Barcelona Spain

Department of Pathology Bellvitge University Hospital Bellvitge Biomedical Research Institute Barcelona Spain

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

Department of Pathology Clinical Hospital Center Zagreb Zagreb Croatia

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

Department of Pathology East University Riga Latvia

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

Department of Pathology Instituto Nacional de Cancerologia Mexico City Mexico

Department of Pathology Medical College of Wisconsin Milwaukee WI USA

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

Department of Pathology University of Split Split Croatia

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

Ljudevit Jurak Pathology Department Clinical Hospital Center Sestre milosrdnice Zagreb Croatia

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