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|⊕⊕⊕
)
