Abstract
AIMS
Leydig cell tumors (LCTs) are rare but the most common non-germ cell testicular tumors. Only limited evidence exists for reliably differentiating between benign and malignant LCTs and for optimally managing the different types and stages of this rare disease. This review aims to synthesize the available evidence regarding the clinical presentation and clinicopathological characteristics associated with LCT malignancy and management.
METHODS
We analyzed published case series with LCTs patients. The association between clinicopathological variables and the presence of metastatic disease was assessed using regression analyses.
RESULTS
We included 357 reports, reviewing available data from 1,375 patients (median age: 34y). Testis-sparing surgery (TSS) was performed in 463 patients. Local recurrence after TSS occurred in 8 of 121 (7%) patients with available follow-up information. Metastases were found in 101 patients and were most often located in the retroperitoneal lymph nodes (60%), lungs (38%), and/or liver (29%). The multivariable models with or without multiple imputation predicting metastatic disease included older age, larger tumor size, the presence of any adverse factor (larger tumor diameter, necrosis, angiolymphatic invasion, pleomorphism, high mitotic index, atypia), and any protective factor (Reinke crystals, lipofuscin pigments, gynecomastia) with model AUCs of 0.93. Durable remission after resection of metastases or platinum-based chemotherapy was rarely seen.
CONCLUSION
Our risk tables using clinicopathological parameters can help identify patients harboring malignant tumors. These patients should undergo staging and either be followed or receive further treatment. In metastatic disease surgical and systemic treatment might result in disease control in in some patients.
Fankhauser, Christian D