Management
The management of Cushing’s syndrome depends on the aetiology. Management in each case is summarised below:
| Aetiology | Management | Comments |
|---|---|---|
| Cushing’s disease (ACTH-secreting pituitary adenoma) | Transsphenoidal pituitary adenomectomy | Performed endoscopically or microscopically. |
| Somatostatin analogues e.g. pasireotide, steroidogenesis inhibitors e.g. osilodrostat, glucocorticoid receptor antagonists e.g. mifepristone | These are used to address short-term severe hypercortisolism whilst awaiting definitive treatment, or if the patient is not fit for surgery. | |
| Hormonal replacement | Due to pituitary resection, replacement of thyroxine, gonadal hormones, somatropin and desmopressin may be needed. | |
| Pituitary radiotherapy | Indicated if high cortisol persists post-tumour resection. | |
| Ectopic ACTH | Surgical resection of tumour/metastases | Resection of ectopic tumours responsible for ACTH (or CRH) production. |
| Steroidogenesis inhibitors, glucocorticoid receptor antagonists | As above. | |
| Chemotherapy and radiotherapy | As above. | |
| Adrenal carcinoma or adenoma | Laparoscopic adrenalectomy or tumour resection | Unilateral resection is often curative without subsequent adrenal insufficiency. |
| Steroidogenesis inhibitors, glucocorticoid receptor antagonists | As above. | |
| Chemotherapy and radiotherapy | Adrenal carcinoma will often require this, as it often will have progressed at time of diagnosis. | |
| Bilateral adrenal hyperplasia or adenoma | Bilateral adrenalectomy | Resection is often curative without subsequent adrenal insufficiency. In some cases mineralocorticoid and corticosteroid replacement may be indicated. |
| Steroidogenesis inhibitors, glucocorticoid receptor antagonists | As above. | |
| Exogenous glucocorticoids | Gradual withdrawal of glucocorticoids | Aiming to discontinue or reduce maintenance dose. |
Table 2: Management of Cushing’s syndrome dependent on aetiology.
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