Objective: To investigate the clinical history of patients with clinical stage II sex cord stromal tumors who underwent retroperitoneal lymph node dissection (RPLND) at our institution. Methods: Our prospectively maintained testicular cancer database was queried to identify patients who presented with or developed clinical stage II sex cord stromal tumors and underwent RPLND at our institution between 1980 and 2018. Demographic, clinical, and pathologic characteristics were reviewed. Kaplan-Meier curves were graphed to assess recurrence-free and overall survival. Results: Fourteen patients were included in the study with a median age of 44.2years. Four patients presented with clinical stage II disease and 10 patients developed metastatic disease during follow-up of initial clinical stage I disease with a median time to metastasis of 2.7years (range: 0.4-19.5 years). Of the 10 patients with orchiectomy pathology data available, all patients had at least 1 risk factor on testis pathology (mean: 2.9 risk factors). Nine patients received treatment prior to referral to our institution. All patients recurred post-RPLND at Indiana University. Median recurrence-free survival was 9.8 months. Twelve patients died of disease with a median overall survival of 14.4 months. Conclusion: Metastatic sex cord stromal tumors are rare and are more resistant to standard treatment modalities than metastatic germ cell tumors. Patients presenting with sex cord stromal tumors should consider prophylactic primary RPLND in the setting of 1 or more pathologic predictor of malignancy.
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