We report 3 cases of primary renal cell
tumor simulating atrophic kidney with distinct gross, morphologic, immunohistochemical, and molecular genetic features. The
tumors were retrieved out of more than 17 000 renal
tumors from the Plzen
Tumor Registry. Tissues for light microscopy had been fixed, embedded, and stained with
hematoxylin and
eosin using routine procedures. The
tumors were further analyzed using immunohistochemistry, array comparative genomic hybridization, and human
androgen receptor. Analyses of VHL gene and loss of heterozygosity (LOH) 3p were also performed. The patients were 2 women and 1 man, with ages ranging from 29 to 35 years (mean, 31.3 years). Grossly, the
neoplasms were encapsulated and round with largest diameter of 3.5 cm (mean, 3.2 cm). Follow-up available for all patients ranged from 2 to 14 years (mean, 8 years). No aggressive behavior was noted. Histologically, akin to atrophic (postpyelonephritic) kidney parenchyma, the
tumors were composed of follicles of varying sizes that were filled by eosinophilic secretion. Rare areas contained collapsed follicles. Each follicle was endowed with a small capillary. The stroma was loose, inconspicuous, and focally fibrotic. Two types of calcifications were noted: typical psammoma bodies and amorphous dark-blue stained calcified deposits. Immunohistochemically,
tumors were strongly positive for cytokeratins (OSCAR), CD10, and
vimentin, with weak immunopositivity for
CAM5.2 and AE1-AE3. WT1 and
cathepsin K were weakly to moderately focally to diffusely positive.
Tumors were negative for
cytokeratin 20,
carbonic anhydrase IX,
parvalbumin, HMB45, TTF1, TFE3,
chromogranin A,
thyroglobulin, PAX8, and ALK. Only 1 case was suitable for molecular genetic analyses. No mutations were found in the VHL gene; no methylation of VHL promoter was noted. No numerical aberrations were found by array comparative genomic hybridization analysis. LOH for chromosome 3p was not detected. Analysis of clonality (human
androgen receptor) revealed the monoclonal nature of the
tumor. We describe an unknown
tumor of the kidney that (1) resembles renal atrophic kidney or
nodular goiter of thyroidal gland; (2) contains a leiomyomatous
capsule and 2 types of calcifications; (3) lacks mitoses, atypias, necroses, and
hemorrhages and nearly lack Ki-67 positivity; and (4) so far showed benign biological behavior.