3 metabolites Metanephrine Nor metanephrine VMA 2 enzymes COMT MOA
Adrenal Incidentaloma Incidentally discovered adrenal masses, also termed clinically inapparent adrenal masses or incidentalomas, are discovered through imaging performed for unrelated nonadrenal disease. Found in 4-6% on CT scans Functioning or nonfunctioning
EVALUATION Evaluation begins with history taking, with a focus on prior malignant disease, hypertension, and symptoms of glucocorticoid or sex steroid excess. Laboratory investigations to evaluate abnormalities in physiological functions. Imaging studies.
Laboratory investigations To evaluate abnormalities in HPA Axis: Baseline morning plasma cortisol 24 hrs urinary free cortisol Midnight salivary or free cortisol Morning plasma cortisol after overnight 1mg dexamethasone suppression Others: Serum testosterone DHEA Sulphate Androstenodione 11 deoxycortisol
PLASMA OR URINARY METANEPHRINES
IMAGING Characteristics suggestive of a benign lesion on CT scan include homogeneous appearance, well-defined borders, high lipid content, rapid washout of contrast material, and low degree of vascularity. Features that are concerning for malignancy include irregular or ill-defined borders, necrosis, internal calcifications or hemorrhage, and high vascularity
Adrenocortical adenoma: * <4cm * Lipid rich(<10 HU) * homogenous with smooth borders * 30% Lipid poor 10-30 HU Contrast washout >60% in 15 minutes Malignancy: * Heterogenous lesions with irregular margins * Calcifications Suspicion of malignancy: <2% for lesions < 4cm 2%-6% for lesions 4-6cm 25% for lesions >6cm
Other imaging studies: I 123 MIBG Scintigraphy FDG PET 6-FDOPA PET Somatostatin receptor based scans
Cushing’s Syndrome Screening tests *24 hour urine cortisol Values higher than 3-4 times normal are highly suggestive of autonomous cortisol secretion. *Overnight 1-mg dexamethasone suppression test Patient takes 1 mg dex pill at 11 PM, then fasts, then presents to lab at 8:00 AM for measurement of serum cortisol . Serum cortisol level > 5 mcg/dl is highly suggestive of autonomous cortisol secretion.
Primary Aldosteronism Plasma renin activity (PRA) and plasma aldosterone concentration Aldo/PRA ratio of 20 with aldo level of 15 ng /dl is positive result
Pheochromocytoma Symptoms : (in paroxysms) tachycardia, palpitations, pallor, tremor, headache, diaphoresis. May be precipitated by maneuvers that increase intra-abdominal pressure (Valsalva, lifting, pregnancy, postural), or by anxiety, or by medicines such as reglan. Signs : Hypertension, orthostatic hypotension, pallor, retinopathy, fever, tremor ,tachycardia, diaphoresis, headache, cardiac arrhythmias, left ventricular dysfunction.
MANAGEMENT Surgery is the treatment of choice for endocrine active or likely malignant and resectable ACTs and for all PCs and PGLs. A course of cortisol-lowering agents such as ketoconazole or metyrapone may be considered before surgery. A course of cortisol-lowering agents such as ketoconazole or metyrapone may be considered before surgery.
The preoperative management of PC and PGL resection includes α-blockade and volume repletion. Finally, the risk of hemodynamic instability after surgical removal of PC or PGL requires close monitoring in the postoperative period in an intensive care unit
ADRERNALECTOMY Open adrenalectomy can be broadly classified into transperitoneal and retroperitoneal approaches. Transperitoneal approaches include the anterior transabdominal and thoracoabdominal approaches, where the main advantages lie in excellent surgical exposure and better access to the hilum and great vessels. Disadvantage: intra abdominal organ injury.
Retroperitoneal approaches include the flank and posterior lumbodorsal approaches, which result in a smaller operative field but are associated with less ileus and shorter hospitalization. In addition, the retroperitoneal approach is ideal for the morbidly obese patient.