Brief introduction of diagnosis and treatment of Cushing syndrome from Cmabell urology.

1. Cushing syndrome

2. Cause of Cushing syndrome
1. Exogenous -> most common
a. itragenic corticosteroid
b. even low dose steroid can
cause cushing
2. ACTH dependent
a. abnormal corticotropin
b. 85% of Endogenous cushing
3.ACTH independent -> rare

3. Symptom and Sign of Cushing syndrome

4. Common occurrence of hypogonadal hypogonadism in men with
Cushing
Up to 50% of patients with Cushing syndrome exhibit urolithiasis
Symptom and Sign of Cushing syndrome

5. ACTH dependent Cushing
Cushing Disease
excessive ACTH -> hypercortisolsim
corticotropin-producing adenoma of Pituitary
Ectopic adrenocorticotropic hormone syndrome
nearly always malignant
Bronchial carcinoid & SCLC
difficult to dx from subclinical pituitary adenoma
adrenal gland markly hyperplasitc than Cushing

6. ACTH independent Cushing
Adrenal tumors
Cortiso-secreting adenoma
dominate unilateral hyperplasia
10% of endogenous Cushing syndrome
adrenal corticocarcinoma :8% of Cushing
ACTH independent bilateral macronodular adrenal hyperplasia (AIMAH)
< 1% Cushing
miltiple large (up to 4cm) nodule

7. Primary Pigmented Nodular Adrenocortical Disease (PPNAD)
adrenal in normal size w/ black or white cortico nodule
50% autosomal dominate
ACTH independent Cushing

8. Subclinical Cushing syndrome
● Hypercortisolemia without cushingoid phenotype
● 10% of adrenal incidentalomas
● Surgery as treatment is still under debate
● Higher risk for post-adrenalectomy adrenal insufficiency

9. Diagnostic test - confirm
● 24-hr urinary free cortisol (UFC)
○ avoid circadian rythm of corticosteroid
○ NOT indicative for adrenal incedentaloma
screening
● Overnight low dose dexamethasone suppression
test (LD-DST)
○ probes pt’s glucocorticoid negative feedback
○ “Low dose” but 4 times physical steroid level
○ Indicative of cushing syndrome but dose not
suggest any cause
● Late-night salivary cortisol
○ loss of diurnal rythm in cushing syndrome
○ inability to supress cortisol at night
○ popular diagnostic test for hypercorticolism

10. Identify the cause of Cushing
ACTH - dependent vs independent
a. measure serum ACTH
b. Low ACTH -> look for adrenal mass
i. 10% bil. adrenal lesion -> AVS for diagnosis
ii. No adrenal mass -> exogenous or PPNAD
c. High ACTH -> ectopic ACTH vs cushing disease
i. Difficult to image pituitary or ACTH tumor
ii. pituitary vein sampling after CRH -> GOLD standard

11. Treatment
Cushing disease
Trans-sphenoidal surgical resection
Cure rate 60-80% and 25% relapse
Bilateral adrenectomy
Primary treatment failed
after Ax-> 8-29% enlarged pituitary -> Nelson-Salassa syn.
warn patient about residual funtional adrenal tissue
life threatening hypercortisolism

12. Treatment
ACTH independent disease
unilateral adrenal secreting mass
unilateral adrenectomy
AIMAH or PPNAD
bilateral adrenectomy + life replacement -> Standard
Medication
Block enzyme for steroid synthesis
for briding to surgery or surgery is not possible