Background. Primitive neuroectodermal tissue in teratomas of testis ha
s been reported in the literature. A mixed germ cell tumor of testis w
ith a prominent neuroblastoma component dictating the clinical behavio
r was found to be unique. Methods. Tissue sections were stained with h
ematoxylin and eosin, and immunohistochemical, ultrastructural, cytoge
netic, and flow cytometric analyses were performed on the primitive ne
uroectodermal component of the testicular mass. Follow-up results at 2
.5 years are included. Results. The microscopic findings on hematoxyli
n and eosin slides showed cells composing the majority of the neoplasm
to have features of neuroblastoma. The immunohistochemical stains sho
wed positivity for neuron-specific enolase in the cells comprising the
neuroblastoma, and transmission electron microscopic study corroborat
ed these findings by demonstrating microtubules and rare membrane-limi
ted, dense-core granules in the cytoplasm. Flow cytometry showed a hyp
ertetraploid population with a large aneuploid DNA content. Cytogeneti
cs revealed a hypertriploid modal number of 74 chromosomes. The clinic
al features were dictated by the neuroblastoma component in a fashion
similar to that of adult neuroblastomas and responded to the chemother
apeutic regimen designed for treating neuroblastoma. Conclusions. The
neuroblastoma component proved to be more aggressive than the other el
ements of this neoplasm. This finding suggests that mixed germ cell tu
mors showing a large neuroblastoma component should be treated promptl
y and aggressively with chemotherapy.