American Urological Association - Sex Cord Stromal Tumors General Features
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Sex Cord Stromal Tumors General Features

  • Neoplasms that have features of Leydig cells, Sertoli cells, granulosa cells, or rarely, theca cells. (No normal granulosa or theca cells in testis)
  • Very rarely is mixed with GCTs (Gonadoblastoma).
  • 4-6% of adult testicular neoplasm, with higher proportion (30%) of testicular tumors in infants and children.
  • No racial difference in frequency (unlike GCT which is more common in Caucasians).
  • Histogenesis is poorly understood and may relate to disruption of hypothalamic-pituitary-testicular axis and hormonal disturbance.
  • Associated with genetic syndromes such as Peutz-Jeghers syndrome (Large cell calcifying Sertoli cell tumor), Carney syndrome and testicular feminization syndrome (Sertoli cell tumor).
  • Unlike germ cell tumor, has no definitive association with cryptorchidism.
  • Presents mostly as painless mass or with hormone-related symptoms (isosexual pseudoprecocity, gynecomastia or impotence).
  • Immunohistochemistry: All SCST are inhibin+, calretinin+. (useful vs. GCT)
  • Approximately 10% of adult-type SCSTs are malignant and may metastasize.
  • Metastasis is the ONLY reliable criterion for malignancy.
  • Histologic features associated with malignancy: nuclear pleomorphism, frequent and abnormal mitosis (>4/10 HPF), necrosis, infiltrative growth, large size (>5 cm), paratesticular extension, and vascular invasion.
  • Tumors in infants and children often have benign behavior.