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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 – – – – – – – – – 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 • • • • • • • • 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 • http://www.scielo.br/img/revistas/abem/v51n8/17f2.gif • Up To Date • Dr. O’Connell and Dr. DeCherney, UNC Endocrinology