A Single-Center Retrospective Long-Term Analysis of 80 Cases of Ovarian Sertoli-Leydig Cell Tumors

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A Single-Center Retrospective Long-Term Analysis of 80 Cases of Ovarian Sertoli-Leydig Cell Tumors

Ovarian Sertoli-Leydig cell tumors (SLCTs) are rare sex cord-stromal neoplasms, constituting less than 0.5% of all ovarian tumors. Their low incidence and diverse clinical presentations complicate the establishment of standardized diagnostic and therapeutic guidelines. This retrospective study of 80 cases, spanning five decades (1966–2019), provides a comprehensive analysis of SLCT characteristics, treatment outcomes, and prognostic factors.

Patient Demographics and Clinical Manifestations

The cohort included patients aged 6 to 73 years, with a mean age of 33 years. A significant proportion (57.5%) were under 30 years old, while 26.6% were postmenopausal. Patients presented with three distinct clinical profiles:

  1. Androgenic Manifestations (60.0%):

    • Symptoms: Oligomenorrhea/amenorrhea (91.7%, 44/48), hirsutism (50.0%, 24/48), voice changes (41.7%, 20/48), clitoromegaly (33.3%, 16/48), and laryngeal protuberance (14.6%, 7/48).
    • Biochemical Findings: Preoperative serum testosterone was elevated in 79.2% (38/48), averaging 14.2 ± 7.1 nmol/L (range: 3.5–34.7). Postoperative normalization occurred in all retested cases (39/39).

  2. Estrogenic Manifestations (16.3%):

    • Predominantly affected older women (mean age: 61 years).
    • Postmenopausal hemorrhage (84.6%, 11/13) and abnormal endometrial pathology (72.7%, 8/11) were common, including hyperplasia (36.4%, 4/11) and polyps (18.2%, 2/11).

  3. Non-Hormonal Presentations (23.8%):

    • Large tumor size (mean: 13.4 cm), acute abdominal pain (36.8%, 7/19), and spontaneous rupture (31.6%, 6/19) characterized this group.

Pathological Characteristics

Tumor Features:

  • Location: Unilateral in 96.3% (77/80), with right-sided predominance (52.5%, 42/80).
  • Size: Mean diameter varied by presentation: 7.7 cm (androgenic), 8.6 cm (estrogenic), and 13.4 cm (non-hormonal).
  • Morphology: Solid-cystic (53.8%, 43/80) and solid (46.3%, 37/80) components were equally prevalent.

Differentiation and Staging:

  • Histologic Grade: Poorly differentiated (62.5%, 50/80), intermediately differentiated (30.0%, 24/80), and well-differentiated (6.3%, 5/80).
  • FIGO Staging:
    • Stage IA/IB: 72.5% (58/80).
    • Stage IC: 25.0% (20/80; ascites or rupture).
    • Advanced stages (IIIC/IV): 2.5% (2/80).

Prognostic Indicators:

  • Poor differentiation (62.5%), heterologous elements (7.5%, 6/80; e.g., immature striated muscle), and retiform patterns (11.3%, 9/80) correlated with aggressive behavior. Non-hormonal presentations were significantly associated with larger tumors (>10 cm), advanced stage (≥IC), and poor differentiation (p<0.05 for all).

Treatment Strategies

Surgical Management:

  • Fertility Preservation: 56.3% (45/80) underwent conservative surgery (cystectomy or unilateral salpingo-oophorectomy).
  • Radical Surgery: 31.3% (25/80) received total hysterectomy with bilateral salpingo-oophorectomy, primarily for advanced disease or postmenopausal patients.
  • Staging Procedures: Pelvic lymphadenectomy was performed in 16.3% (13/80), but no nodal metastases were identified.

Adjuvant Chemotherapy:

  • Usage: 60.0% (45/75) of intermediate/poorly differentiated cases received chemotherapy. Regimens included PEB (cisplatin + etoposide + bleomycin; 46.8%), PVB (cisplatin + vinblastine + bleomycin; 14.9%), and others (e.g., TC, PAC).
  • Efficacy: No significant difference in relapse rates between chemotherapy (17.8%, 8/45) and non-chemotherapy groups (6.7%, 2/30; p=0.17). Recurrent tumors exhibited drug resistance.

Recurrence and Survival

Recurrence Profile:

  • Incidence: 12.5% (10/80) relapsed at a median of 19 months.
  • Risk Factors: Non-hormonal presentation (p=0.011), stage ≥IC (p=0.004), tumor ≥10 cm (p=0.001), poor differentiation, and retiform/heterologous elements.
  • Sites: Pelvic/abdominal recurrence (60.0%, 6/10) dominated; one case had liver/lung metastasis.

Mortality:

  • Disease-specific mortality: 6.3% (5/80). Four deaths occurred post-recurrence; one stage IV patient died within a month of diagnosis.
  • Overall survival: 90.8% (69/76) remained disease-free after 10–383 months (mean: 86 months).

Reproductive Outcomes

Among 19 patients desiring pregnancy:

  • Success Rate: 78.9% (15/19) achieved live births, including two via IVF.
  • Menstrual Recovery: 90.0% (36/40) resumed normal menses within 1–3 months post-surgery. Chemotherapy-induced transient amenorrhea resolved in one case.

Key Conclusions

  1. Clinical-Pathologic Correlation:

    • Androgenic presentations (younger age, early stage) had favorable outcomes.
    • Non-hormonal symptoms (older age, advanced stage, large tumors) predicted higher recurrence and mortality.

  2. Surgical Considerations:

    • Fertility-sparing surgery is safe for early-stage, well-differentiated tumors.
    • Lymphadenectomy may be omitted due to negligible nodal involvement.

  3. Chemotherapy Limitations:

    • No survival benefit was observed with adjuvant chemotherapy.
    • Drug resistance underscores the need for novel therapies in recurrent disease.

  4. Prognostic Markers:

    • Tumor size ≥10 cm, retiform patterns, and heterologous elements warrant intensified surveillance.

This study highlights the heterogeneous nature of SLCTs and advocates for risk-adapted management. Long-term follow-up is critical, particularly for high-risk patients.

doi.org/10.1097/CM9.0000000000001489

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