| Abstract: |
The diagnosis of sex cord-stromal tumors is challenging. They show a wide spectrum of behaviors and associations with clinically important pathogenic germline variants. In view of recent advances in molecular subtyping and risk factors, we wished to investigate the differences in diagnosis for a range of these tumors using expert genitourinary pathologists with an interest in this area. Forty-four tumors were selected, and 18 pathologists (members of TEsticular Sex cord-Stromal Tumor group) were invited to view the cases online and give a diagnosis. Consensus was taken as 70% agreement. Consensus was achieved in 59% (26/44) cases. However, there were many areas of disagreement, which included variability in the diagnosis of Sertoli cell and Leydig cell tumors, particularly in malignant lesions, and difficulty in the assignation of fibrothecomas or myoid gonadal stromal tumors, as well as variability in the diagnosis of granulosa cell tumors and in the diagnosis of rarer pediatric tumors. Pathologists placed different weights on positivity with some markers, particularly beta-catenin, S100, and SMA. Some pathologists diagnosed novel diagnostic entities, such as inflammatory and nested testicular sex cord tumors, not currently in the World Health Organization classification. Recommendations to assist in the construction of a new classification to achieve more concordance and better treatment of these rare tumors are included. © 2025 The Authors |
