This study explores immunohistochemical characteristics that may be of
diagnostic value in differentiating clear cell sarcoma of the kidney
(CCSK) from Wilms' tumor (WT) and may provide some insight into the hi
stogenesis of CCSK. Formalin-fixed, paraffin-embedded sections of 8 CC
SK and 9 WT were stained, using the standard avidin-biotin peroxidase
complex method, for vimentin (VIM), Factor-8 related antigen (F8A), ep
ithelial membrane antigen (EMA), desmin (DES), S-100 protein and Mac 3
87. CCSK cells consistently exhibited moderate to strong diffuse cytop
lasmic positivity for VIM and were negative for F8A, EMA, DES, S-100 a
nd Mac 387. In contrast, only patchy groups of stromal cells and primi
tive glomeruloid structures in WT exhibited VIM-positivity. Blastemal
cells were VIM-negative. Stromal cells with rhabdomyomatous differenti
ation exhibited cytoplasmic positivity for DES. Epithelial cells of ma
turing tubular structures showed EMA-positivity whereas immature tubul
ar structures were EMA-negative. Neither blastemal, stromal nor epithe
lial elements in WT were positive for F8A, S-100 or Mac 387. Podocytes
and mesangial cells of glomeruli in 3 mid-trimester human abortuses (
controls) exhibited moderate to strong VIM-positivity. The importance
of differentiating CCSK from WT has been repeatedly emphasized because
of its poorer prognosis and the necessity of adding Adriamycin to the
chemotherapeutic regime. The consistent VIM-positivity of CCSK cells
can be a useful feature in differentiating it from ''blastemal-predomi
nant'' WT, with which it is often confused. Although vimentin expressi
on by CCSK cells is consistent with a mesenchymal character, the possi
bility of a histogenetic link with glomerular podocytes or mesangial c
ells should also be considered.