From: Growth and Reproductive Outcomes in Congenital Adrenal Hyperplasia
Stage | Histological description | Testicular ultrasound | Reversibility | Treatment options |
---|---|---|---|---|
1 | Adrenal rest cells within the rete testis | Undetectable by testicular ultrasound | +++ | None |
2 | Proliferation of adrenal rest cells due to growth-promoting factors (such as Angiotensin II and ACTH) | May become visible as one or more small hypoechogenic lesions | +++ | Increase dose and intensify glucocorticoid therapy |
3 | Growth of adrenal rest cells leads to compression of the rete testis, obstruction of the seminiferous tubules, and evidence of gonadal dysfunction ( FSH, LH, sperm, inhibin B) | Detectable | ++ | Increase dose and intensify glucocorticoid therapy. Surgery can also be considered. |
4 | Further hypertrophy and hyperplasia of adrenal rest cells with progressive obstruction of rete testis with fibrosis within the tumor and focal lymphocytic infiltration | Detectable—small tumors may form a single lobulated structure | −/+ | Consider surgery but may not reverse testicular damage. |
5 | Chronic obstruction leads to destruction of surrounding testicular parenchyma | Detectable | − | None—irreversible damage. |