American Urological Association - Sex Cord Stromal Tumors General Features
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.