Elevated serum alpha-fetoprotein associated with Sertoli-Leydig cell tumors of the ovary

Two young girls are described who presented with amenorrhea, large ovarian masses, elevated serum alpha-fetoprotein (AFP), and signs of androgen excess. Whereas the findings of an ovarian mass and elevated serum AFP typically suggest an ominous diagnosis, ie, endodermal sinus tumor of the ovary, both of these patients were found at laparotomy to have Sertoli-Leydig cell tumors of the ovary. Unilateral oophorectomy rapidly led to undetectable AFP levels in both patients, and resumption of menses.