Treatment of Stage IC Poorly Differentiated Sertoli-Leydig Cell Tumor
For a patient with stage IC poorly differentiated Sertoli-Leydig cell tumor, adjuvant platinum-based chemotherapy with BEP (bleomycin, etoposide, and cisplatin) for 3-4 cycles is recommended following complete surgical staging. 1, 2
Surgical Management
Initial surgical approach should include:
- Unilateral salpingo-oophorectomy with preservation of the contralateral ovary and uterus if fertility preservation is desired 2
- Complete surgical staging including infracolic omentectomy, peritoneal biopsies (diaphragmatic peritoneum, paracolic gutters, pelvic peritoneum), and peritoneal washings 1, 2
- Systematic lymphadenectomy is not required due to the very low incidence of retroperitoneal metastases in early-stage disease 1, 2
The evidence strongly supports fertility-sparing surgery even in stage IC disease, as these tumors are typically unilateral and conservative surgery does not compromise outcomes. 1, 2
Adjuvant Chemotherapy Indication
Poor differentiation in stage IC disease is a clear indication for adjuvant chemotherapy. 1, 2 The ESMO guidelines explicitly state that "postoperative adjuvant chemotherapy should be considered for patients with stage I poorly differentiated or heterologous elements." 1
High-risk features warranting chemotherapy include:
- Poor differentiation (Grade 3) 1, 2, 3
- Stage IC (tumor rupture, surface involvement, or positive washings) 1, 2
- Presence of heterologous elements 1, 3
- Retiform pattern 2, 3
Recent data from 2024 demonstrates that among stage I tumors, those with mesenchymal heterologous elements treated with surgery alone had significantly higher recurrence risk (HR: 74.18). 3 This reinforces the need for adjuvant therapy in poorly differentiated tumors.
Chemotherapy Regimen
BEP (bleomycin, etoposide, and cisplatin) is the standard first-line regimen:
- Administer 3-4 cycles 1, 2
- BEP is the most commonly used and widely recommended regimen across all major guidelines 1, 2
- Bleomycin should be omitted in patients >40 years old or those with pre-existing pulmonary disease due to toxicity concerns 2
Alternative platinum-based regimens (if BEP is contraindicated):
The evidence consistently shows that platinum-based chemotherapy is the treatment of choice for high-risk early-stage sex cord-stromal tumors. 1
Surveillance Strategy
Long-term follow-up is mandatory as recurrences can occur up to 20 years after initial diagnosis: 2
- Clinical examination with pelvic exam and assessment for hormonal changes every 3 months for the first 2 years, then every 6 months for years 3-5 1
- Pelvic ultrasound every 6 months for patients who underwent fertility-sparing surgery 1
- CT abdomen/pelvis according to clinical indication 1
- Tumor markers (testosterone, inhibin if initially elevated) 2, 4
Critical Clinical Pitfalls to Avoid
Do not perform radical surgery (bilateral salpingo-oophorectomy with hysterectomy) in reproductive-age patients with stage IC disease - this compromises fertility without improving outcomes, as these tumors are virtually always unilateral. 2, 5
Do not omit adjuvant chemotherapy in poorly differentiated stage IC tumors - the NCCN and ESMO guidelines are clear that poor differentiation is a high-risk feature requiring systemic therapy. 1, 2 Clinical series show that poorly differentiated tumors have recurrence rates requiring adjuvant treatment. 6, 5
Do not discontinue follow-up prematurely - relapses can occur decades later, necessitating lifelong surveillance. 2, 5
Prognosis
Stage IC poorly differentiated tumors have a less favorable prognosis compared to stage IA well-differentiated tumors. 3, 5 Three-year recurrence-free survival for stage IC tumors is approximately 67% compared to 94% for stage IA disease. 3 However, with appropriate adjuvant chemotherapy, most patients achieve complete remission, and recurrences can be successfully salvaged with secondary surgery and chemotherapy. 5