Medical Rx
Î ES recommends steroidogenesis inhibitors under the following
conditions: as second-line treatment after TSS in patients with CD, either
with or without RT/radiosurgery; as primary treatment of ectopic ACTH
secretion in patients with occult or metastatic ectopic ACTH secretion;
and as adjunctive treatment to reduce cortisol levels in ACC. (1|⊕⊕⊕
)
• ES suggests pituitary-directed medical treatments in patients with CD who are not
surgical candidates or who have persistent disease after TSS. (2|⊕⊕⊕
)
• ES suggests administering a glucocorticoid antagonist in patients with diabetes or
glucose intolerance who are not surgical candidates or who have persistent disease
after TSS. (2|⊕⊕⊕
)
• ES suggests targeted therapies to treat ectopic ACTH syndrome. (2|⊕
)
Special Populations/Considerations
Î ES recommends urgent treatment (within 24-72 hours) of
hypercortisolism if life-threatening complications of CS such as infection,
pulmonary thromboembolism, cardiovascular complications and acute
psychosis are present. (1|⊕⊕⊕
).
Î The associated disorder(s) should be addressed as well
(e.g., anticoagulation, antibiotics).
Approach for Long-term Follow-up
Î ES recommends treating the specific comorbidities associated with CS
(e.g., cardiovascular risk factors, osteoporosis and psychiatric symptoms)
in all patients with CS throughout their lives until resolution. ES also
recommends testing for recurrence throughout life, except in patients
who underwent resection of an adrenal adenoma with a CT density of <10
Hounsfield units. (1|⊕⊕⊕
)
Î ES recommends educating patients and families about the clinical
features of remission. (US)
Î In patients with adrenal adenoma, ES suggests follow-up tests for the
specific comorbidities associated with CS if the adenoma density on CT
was <10 Hounsfield units. (2|⊕⊕
) For those with higher Hounsfield
unit values or pathology consistent with possible carcinoma, ES suggests
evaluating for malignancy using imaging. (2|⊕
)
Î ES recommends that patients with Carney complex have lifelong follow-
up tests for cardiac myxoma and other associated disease (testicular
tumors, acromegaly, thyroid lesions). (1|⊕⊕⊕⊕)
