A patient with a retroperitoneal mass extending into the liver had a c
ore needle biopsy that showed a small, round, blue cell tumor of uncer
tain origin by light microscopy. The tumor cells stained with antibodi
es against vimentin, neuron specific enolase, and the S-100 protein. T
he cells did not stain with antibodies to muscle specific actin, chrom
ogranin A, cytokeratin cocktail, or leukocyte common antigen. Based up
on these histologic and immunohistochemical findings, consideration wa
s given to either an extra-osseous Ewing's sarcoma or a peripheral neu
roepithelioma, and a diagnosis of ''consistent with peripheral neuroep
ithelioma'' was rendered. The role of immunohistochemistry in resolvin
g this differential and the resulting prognostic implications are disc
ussed.