Sex Cord-Stromal Tumors of the Testis

Sex cord-stromal tumors (SCST) are very rare, representing approximately 4 % of all testis tumors and about 8 % of testis tumors in prepubertal males. Most resemble or recapitulate normal testicular components such as Leydig cells, Sertoli cells, and nonspecific stromal cells. SCST usually present as a painless testicular mass, but some may be associated with endocrine manifestations such as isosexual precocity, or gynecomastia, or erectile dysfunction. Most SCST are sporadic, but some show particular clinical associations with Peutz-Jeghers syndrome, Carney complex, and androgen insensitivity syndrome. In certain cases, the diagnosis of SCST may be the presenting feature for one of these syndromes.