A 36-year-old woman presented with intermenstrual spotting and was fou
nd to have a cystic mass involving the uterine cervix on a pelvic ultr
asound examination. A necrotic and hemorragic tumor was excised by hys
terectomy and processed for light and electron microscopic investigati
on and molecular analysis. Microscopic examination revealed a small ro
und cell tumor that immunohistochemical studies (including staining fo
r the highly restricted surface antigen p30/32(MIC2)) and ultrastructu
ral studies indicated was an extraosseous Ewing's sarcoma (EES)/primit
ive neuroectodermal tumor (PNET). This diagnosis was established by de
tection of EWS/ERG fusion transcript through reverse transcription pol
ymerase chain reaction (RT-PCR) with nested primers. Full body compute
d tomography failed to detect any extrauterine tumor, and the patient
is clinically free of disease 18 months after hysterectomy. This case
represents the first report of a primary EES/PNET arising in the uteri
ne cervix.