Extragonadal germ cell tumors are well recognized in men but have rarely be
en reported in women. Reports have primarily focused on the pediatric popul
ation and have suggested a poor prognosis for extragonadal yolk sac tumors.
A 23-year-old woman with a yolk sac tumor arising in the rectum is describ
ed. A review of the English-language literature (MEDLINE 1966-1998) regardi
ng extragonadal germ cell tumors in females is provided. Treatment with fou
r courses of cisplatin, etoposide, and bleomycin as followed by surgical re
section of the involved area. No residual tumor was identified. She remains
disease free 3.5 years later. Previous reports are limited by the small nu
mber of patients, focus on the pediatric population, and treatment before t
he availability of cisplatin. Extragonadal germ cell tumors in women are ex
tremely rare but can be successfully treated with aggressive chemotherapy a
nd surgery similar to testis cancer.