We describe a rare case of an extragonadal retroperitoneal endodermal sinus
(yolk sac) tumor in the minor pelvis. Radiologic investigation, which incl
uded abdominal ultrasound and computed tomography (CT), showed a large soft
tissue mass occupying the pelvic cavity. Radionuclide bone scans demonstra
ted bone metastases. The serum alpha fetoprotein was elevated. Pathologic e
xamination of the surgical specimen revealed extragonadal yolk sac tumor. I
mmunohistochemically, the tumor was positive for a-fetoprotein and cytokera
tins. After postoperative combination therapy, follow-up CT showed decreasi
ng tumoral disease, while serum alpha fetoprotein returned to normal.