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

Choose the health content that's right for you, and get it delivered right in your inbox

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 Call407-303-5909.

Recent Blogs

Chris Nikic running
Blog
Mission Possible: Our Partnership with Chris Nikic
Two Ladies Laugh and Talk as They Walk Through a Park at Sunrise
Blog
Celebrating Non-Scale Victories After Bariatric Surgery
Blog
When to Consider Knee Replacement Surgery
A couple dancing in the kitchen.
Blog
What You Need to Know About Hip Replacement
Blog
How to React Like a Pro Athlete
View More Articles