Treatment
Î ES recommends that clinicians discuss and offer age-appropriate
vaccinations to CS patients—particularly influenza, H. zoster, and
pneumococcal vaccinations—due to an increased risk of infection. (US)
First-line Treatment Options
Î ES recommends initial resection of primary lesion(s) underlying Cushing's
disease (CD), ectopic and adrenal (cancer, adenoma, and bilateral disease)
etiologies, unless surgery is not possible or unlikely to significantly reduce
glucocorticoid excess. (1|⊕⊕⊕⊕)
• ES recommends unilateral resection by an experienced adrenal surgeon for all cases of
benign unilateral disease. (1|⊕⊕⊕
)
• ES recommends localizing and resecting ectopic ACTH-secreting tumors with node
dissection as appropriate. (1|⊕⊕⊕⊕)
• ES recommends transsphenoidal selective adenomectomy (TSS) by an experienced
pituitary surgeon as the optimal treatment for CD in pediatric and adult patients.
(1|⊕⊕⊕⊕)
▶ ES recommends measuring serum sodium several times during the first 5–14 days
after transsphenoidal surgery. (1|⊕⊕
)
▶ ES recommends assessing free thyroxine and prolactin within 1–2 weeks of surgery, to
evaluate for overt hypopituitarism. (1|⊕⊕
)
▶ ES recommends obtaining a postoperative pituitary MRI within 1–3 months of
successful TSS. (US)
• ES recommends surgical resection of bilateral adrenal disorders (1|⊕⊕
), and suggest
medical therapy to block aberrant hormone receptors for bilateral macronodular adrenal
hyperplasia (BMAH) (2|⊕⊕
).
Remission and Recurrence After Surgical Tumor Resection
Î ES suggests an individualized management approach based on whether
the post-operative serum cortisol values categorize the patient as
hypocortisolism, hypercortisolism, or eucortisolism. (US)
Î ES recommends additional treatments in patients with persistent overt
hypercortisolism. (1|⊕⊕⊕⊕)
Î ES recommends measuring late-night salivary or serum cortisol in patients
with eucortisolism after TSS, including those cases where eucortisolism
was established by medical treatment before surgery. (1|⊕⊕
)
Î ES recommends using tests to screen for hypercortisolism to assess for
recurrence in patients with ACTH-dependent CS. (1|⊕⊕⊕
)
Glucocorticoid Replacement and Discontinuation, and Resolution
of Other Hormonal Deficiencies
Î ES recommends that hypocortisolemic patients receive glucocorticoid
replacement and education about adrenal insufficiency after surgical
remission. (1|⊕⊕⊕⊕)
