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Adrenal mass
Cushing’s Syndrome
Taylor Wofford
September 18, 2009
Diagnosis
• Excess glucocorticoid
• Timing of symptoms
• Severity of symptoms
• Cause of symptoms
Frequency of causes of
Cushing’s syndrome
ACTH-dependent
Percentage ACTHindependent
Percentage
Cushing’s disease
(ACTH-secreting
pituitary tumor)
68
Adrenal adenoma
10
Ectopic ACTH
syndrome
12
Adrenal carcinoma 8
Ectopic CRH
syndrome
<<1
Micronodular
hyperplasia
1
Macronodular
hyperplasia
<<1
Hypothalamic-Pituitary-Adrenal
Axis
Cushing’s Syndrome
Blood clots?
 IOP, cataracts
Comparing Obesity vs. Cushing’s
Physical
exam
Striae
Obesity
Cushing’s
Pink
Purple
Weakness
General
Proximal (squat)
Distribution
General
Hirsutism
Mild
Supraclavicular,
Buffalo hump
Male pattern
Bruising
Normal
Very easily
Cataracts
Anterior
Posterior
Cortisol
Mildly elevated
>> ULN
Diagnostic testing algorithm
UpToDate
Indeterminate ACTH Testing
• Cortocotropin Releasing Hormone
stimulation test
– When CRH is given, ACTH-dependent
disease should produce an increase in ACTH
and cortisol
– Draw labs  dose of CRH  measure
change 45 min post.
ACTH-dependent workup
• MRI-pituitary
• CRH stimulation test AND
• High dose dexamethasone suppression
test
– Nonpituitary tumors associated with ectopic
ACTH are completely resistant to feedback
inhibition
– 8mg dex po at 2300. measure serum cortisol
at 0800 next day. <5 μg/dL=pituitary source.
ACTH-dependent workup-2
• Inferior petrosal sinus sampling
– If ACTH secretion is coming from a pituitary source, inferior
petrosal sinus ACTH levels will be greater than peripheral ACTH
levels
– Central-to-peripheral ratio of ≥2.0 pre-CRH or ≥3.0 post-CRH =
pituitary source
ACTH-independent workup
• Adrenal CT or MR imaging
– Thin cuts
– Masses often difficult to differentiate. ?PET.
Favor adenoma
Favor carcinoma
Size <5 cm
Size >5 cm
Homogeneous
Heterogeneous
Density <20 HUs
Density >20 HUs
Necrosis, hemorrhage,
calcifications
Adrenal mass
Adrenal histology
• No one characteristic feature
• Weiss score 0-9
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–
–
–
–
–
–
–
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Nuclear grade
Mitotic rate
Atypical mitosis
Character of cytoplasm
Architecture of tumor cells
Necrosis
Invasion of venous structures
Invasion of sinusoid structures
Invasion of the capsule of the tumor
Steroid synthesis pathway
Infectious complications of
Cushing’s syndrome
•
•
•
•
•
•
•
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Cryptococcosis
Aspergillosis
Nocardiosis
Pneumocystis carinii
Staph aureus
Candida albicans
Alternariosis
Tinea
Bibliography
•
Graham BS, Tucker WS, Opportunistic infections in endogenous Cushing’s syndrome. Ann Intern Med 1984 Sep;101
(3):334-8.
•
http://images.google.com/imgres?imgurl=https://courses.stu.qmul.ac.uk/smd/kb/resources/endocrinologyresource/2136.JPG&imgrefurl=http://flipper.diff.org/app/items/info/418&usg
•
http://models.cellml.org/workspace/bingzheng_zhenye_liansong_1990/@@rawfile/f475e016ed033c4b3dda595794addb2c8a6bcdc9/bingz
heng_1990.png
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http://www.scielo.br/img/revistas/abem/v51n8/17f2.gif
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Up To Date
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Dr. O’Connell and Dr. DeCherney, UNC Endocrinology