Management of Sertoli-Leydig Cell Tumor in a 16-Year-Old
For a 16-year-old with Sertoli-Leydig cell tumor, perform fertility-sparing surgery with unilateral salpingo-oophorectomy, preserving the contralateral ovary and uterus, followed by careful long-term surveillance without adjuvant chemotherapy if the tumor is stage IA and well- to moderately-differentiated. 1
Surgical Management
Primary Surgical Approach
Perform unilateral salpingo-oophorectomy with preservation of the contralateral ovary and uterus, as this is the standard fertility-sparing approach for young patients with apparently localized disease 1
Surgical staging procedures should include careful examination of the abdominal cavity, infracolic omentectomy, biopsy of diaphragmatic peritoneum, paracolic gutters, pelvic peritoneum, and peritoneal washings 1
Systematic lymphadenectomy is NOT required for Sertoli-Leydig cell tumors due to the very low incidence of retroperitoneal metastases in early-stage disease 1
Laparoscopic approach is acceptable in selected cases, as it is minimally invasive with faster recovery, though care must be taken to avoid tumor rupture 1, 2
Special Surgical Considerations
If the tumor is very small and difficult to identify which ovary is affected, selective sampling by laparoscopy or Seldinger technique of the left and right ovarian veins may indicate the tumor site 1
The contralateral ovary should NOT be biopsied if it appears macroscopically normal 1
Adjuvant Therapy Decision-Making
Stage IA Disease (Most Common Scenario)
Most Sertoli-Leydig cell tumors are stage IA and confined to the ovary; there is no firm evidence to support adjuvant chemotherapy in this setting 1
Indications for Adjuvant Chemotherapy
Adjuvant chemotherapy should be considered ONLY for: 1, 3, 4
- Poorly differentiated tumors (Grade 3)
- Presence of heterologous elements
- Stage IB or higher (beyond stage IA)
- Retiform pattern (poor prognostic factor)
Chemotherapy Regimen When Indicated
BEP (bleomycin, etoposide, and cisplatin) is the standard first-line regimen for 3-4 cycles 1, 5
Important caveat: Bleomycin should NOT be given to patients >40 years old or those with pre-existing pulmonary disease due to toxicity concerns 1
Alternative regimens include carboplatin/paclitaxel or etoposide/cisplatin (EP) 1
Follow-Up Strategy
Long-Term Surveillance Requirements
Careful long-term follow-up is mandatory as there is risk of relapse as late as 20 years after initial diagnosis 1
The median time to relapse in one series was 28 months (range 9-70 months), with most relapses occurring as peritoneal carcinomatosis 4
Monitoring Parameters
Clinical examination for signs of recurrence or hormonal changes 1
Imaging surveillance (though specific intervals are not definitively established in guidelines) 1
Tumor markers have limited utility but may include inhibin, testosterone levels if initially elevated 1
Prognostic Factors
Stage at diagnosis remains the most important prognostic factor for Sertoli-Leydig cell tumors 1
Additional poor prognostic factors include: 3, 4
- Poor differentiation (Grade 3)
- Presence of heterologous elements
- Retiform pattern
- Tumor rupture or higher stage
Clinical Pitfalls to Avoid
Do NOT perform radical surgery (bilateral salpingo-oophorectomy with hysterectomy) in reproductive-age patients with stage IA disease, as this compromises fertility without improving outcomes 1
Do NOT routinely administer adjuvant chemotherapy for stage IA well- to moderately-differentiated tumors without high-risk features, as there is no evidence of benefit 1
Do NOT perform systematic lymphadenectomy as it does not affect prognosis and adds morbidity 1
Do NOT discontinue follow-up prematurely, as relapses can occur up to 20 years later 1