Ovarian Sertoli-Leydig cell tumors (SLCT) represent < 2% of primary ovarian tumors, which range from benign to malignant; the majority of the latter are low-grade. We present the case of a 12-year-old pre-pubertal female with poorly differentiated SLCT and heterologous rhabdomyosarcoma (FIGO stage-IA). She presented with worsening abdominal pain, pelvic mass, and elevated pre-operative serum Alpha-fetoprotein (AFP, 77.1 ng/mL). She underwent right salpingo-oophorectomy, omentectomy, and lymphadenectomy. The high-risk histology warranted 4 cycles of adjuvant BEP (bleomycin, etoposide, platinum/cisplatin) chemotherapy. There was no clinical evidence of recurrence at the 20-month follow-up. High-grade sex cordstromal tumors are rare, present with low-stage disease, and have good progression-free survival following chemotherapy. Rarity of these tumors poses challenges in their diagnosis and treatment. Review of literature suggests that the presented case is the youngest patient with dedifferentiated SLCT.
- SLCTs are uncommon; mostly well-differentiated; dedifferentiation is rare.
- AFP can be elevated; caveat is not to misdiagnose as yolk-sac tumor.
- Presented case is youngest reported, had low-stage disease, high-risk histology.
- High-risk histology, rupture and high-stage need treatment and confer poor prognosis.
For more information or to refer a patient, call GYN Oncology Care Coordinator Denise Cochran, MSN, ARNP, BC, at (407) 303-5909.