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Poorly Differentiated Ovarian Sertoli-Leydig Cell Tumor with Heterologous Rhabdomyosarcoma and Glandular Elements: Diagnosis and Management of a Rare Neoplasm.

POSTED BY: Florida Hospital

Summary

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.

Highlights

  • 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.