The case of a woman who developed a cervical sarcoma botryoides tumor
at age 14 years and a right ovarian Sertoli-Leydig cell tumor with alp
ha-fetoprotein production at 27 years is presented. The sarcoma botryo
ides was a stage Ib, 4-cm, polypoid ectocervical tumor treated by radi
cal hysterectomy and bilateral pelvic lymphadenectomy, The Sertoli-Ley
dig cell tumor was a stage la, 145-g mass removed piecemeal by right o
ophorectomy. Histologically, it was an intermediate Sertoli-Leydig cel
l tumor with a heterologous element composed of an endometrioid-like y
olk sac tumor which was producing alpha-fetoprotein. There was no hist
ological similarity between the two tumors, The patient is alive witho
ut evidence of disease, 16 years after diagnosis of her sarcoma botryo
ides and 3 years after her Sertoli-Leydig cell tumor, This is, to our
knowledge, the third known association between these two rare gynecolo
gical tumors. The basis of the association remains unknown. (C) 1997 A
cademic Press.