Ovarian Sertoli-Leydig cell tumor of intermediate grade with heterologous elements of rhabdomyosarcoma. A case report and a review of the literature

Ovarian Sertoli-Leydig cell tumors (SLCTs) are rare sex cord–stromal tumors, and among them, tumors with heterologous mesenchymal elements are exceptional and mainly associated with poorly differentiated tumors and are often fatal. We present the fourth case of an ovarian SLCT of intermediate differentiation with rhabdomyosarcoma and a review of the literature. Surgical treatment was conservative with preservation of the contralateral adnexa and uterus. No adjuvant treatment was given. At 4 years control post surgery, the patient was without evidence of disease.Extensive sampling of SLCTs is important because heterologous elements may be sparse. Immature skeletal muscle cells in SLCTs often reveal only moderate pleomorphism, and as they are closely admixed with the Sertoli cells or immature gonadal stroma, they can be rather difficult to differentiate from the latter ones. Immunohistochemical analysis with a panel of antibodies including antibodies against myogenin and alpha-inhibin is very important to diagnose the rhabdomyosarcoma and to grade the SLCT accurately.