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Cushing's Syndrome Treatment

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Treatment Î ES recommends follow-up morning cortisol and/or ACTH stimulation tests or insulin-induced hypoglycemia to assess the recovery of the HPA axis in patients with at least one intact adrenal gland, assuming there are no contraindications. ES also recommends discontinuing glucocorticoid when the response to these test(s) is normal. (1|⊕⊕⊕ ) Î ES recommends re-evaluating the need for treatment of other pituitary hormone deficiencies in the postoperative period. (1|⊕⊕⊕ ) Second-line Therapeutic Options Î In patients with ACTH-dependent CS who underwent a non-curative surgery or for whom surgery was not possible, ES suggests a shared decision-making approach, as there are several available second-line therapies (e.g., repeat transsphenoidal surgery, radiotherapy, medical therapy, and bilateral adrenalectomy). (2|⊕⊕ ) Î ES suggests bilateral adrenalectomy for occult or metastatic EAS or as a life-preserving emergency treatment in patients with very severe ACTH- dependent disease who cannot be promptly controlled by medical therapy. (2|⊕⊕⊕ ) Î ES recommends regularly evaluating for corticotroph tumor progression using pituitary MRIs and ACTH levels in patients with known CD who undergo bilateral adrenalectomy and in patients who undergo this procedure for presumed occult EAS (as some of the latter have a pituitary and not ectopic tumor). (1|⊕⊕⊕ ) Repeat Transsphenoidal Surgery Î ES suggests repeat transsphenoidal surgery, particularly in patients with evidence of incomplete resection or a pituitary lesion on imaging. (2|⊕⊕ ) Radiation Therapy/Radiosurgery for Cushing's Disease Î ES recommends confirming that medical therapy is effective in normalizing cortisol before administering radiation therapy (RT)/ radiosurgery for this goal, because this will be needed while awaiting the effect of radiation. (1|⊕ ) • ES suggests RT/radiosurgery in patients who have failed TSS or have recurrent CD. (2|⊕⊕ ) • ES recommends using RT where there are concerns about the mass effects or invasion associated with corticotroph adenomas. (1|⊕⊕⊕ ) • ES recommends measuring serum cortisol or UFC off-medication at 6- to 12-month intervals to assess the effect of RT and also if patients develop new adrenal insufficiency symptoms while on stable medical therapy. (1|⊕⊕⊕ )

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