Cervical sarcoma botryoides and ovarian Sertoli-Leydig cell tumor

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 alpha-fetoprotein production at 27 years is presented. The sarcoma botryoides was a stage 1b, 4-cm, polypoid ectocervical tumor treated by radical hysterectomy and bilateral pelvic lymphadenectomy. The Sertoli-Leydig cell tumor was a stage 1a, 145-g mass removed piecemeal by right oophorectomy. Histologically, it was an intermediate Sertoli-Leydig cell tumor with a heterologous element composed of an endometrioid-like yolk sac tumor which was producing alpha-fetoprotein. There was no histological similarity between the two tumors. The patient is alive without evidence of disease, 16 years after diagnosis of her sarcoma botryoides and 3 years after her Sertoli-Leydig cell tumor. This is, to our knowledge, the third known association between these two rare gynecological tumors. The basis of the association remains unknown.