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K. Poppe Endocrinologie
Adrenal masses K. Poppe Endocrinologie
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Incidentaloma
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Serendipitiously discovered by radiologic examinations (CT / MRI)
Definition Mass lesion > 1 cm Serendipitiously discovered by radiologic examinations (CT / MRI)
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Prevalence
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Incidentaloma’s are bilateral in 10-15%
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Work-up
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Q1: Benign vs Malign?
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W. Young NEJM 2007
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Evaluate for malignancy
Size and imaging characteristics (imaging phenotype) The maximum diameter is predictive of malignancy Adenocortical carcinomas - significantly association with mass size 90% > 4 cm - sensitivity 93 % - specificity 76 % The National Italian Study Groups
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Benign adenoma Round & homogenous density < 4 cm, unilateral
Low unenhanced CT attenuate values (<10HU) Rapid contrast washout (10 min) Absolute contrast washout >50% Isointensity with liver on both T-1 & T-2 (MRI) Chemical shift : lipid on MRI
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Adrenocortical carcinoma
Irregular shape Inhomogenous density (central necrosis) > 4 cm, unilateral, calcify High unenhanced CT (>20HU) Delayed contrast washout (10 min) Absolute contrast washout < 50 % Hypointensity compared with liver T-1 and high to intermidiate intensity T-2 MRI Evidence of local invasion or Mets
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MRI has advantages in certain situations:
1- Conventional spin-echo MRI - T1 and T2 - distinguish benign from malignancy and pheochromocytoma 2- Gadolinium-DPTA-enhanced MRI - adenoma : mild enhancement and rapid washout of contrast - malignancy : marked enhancement and a slower washout 3- Chemical shift imaging (CSI) - in-phase : water & lipid are aligned: signal intensity high - out of phase : opposite from each other: signal intensity low
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Others Adrenal cysts Adrenal hemorrhage Myelolipoma
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Others PET (Positron Emission Tomography)
- fluoro-2-deoxy-D-glucose (FDG) - high sensitivity for detecting malignancy - however : 16% benign cortical lesions may have FDG-PET uptake - metomidate (MTO) : lack of MTO – specific to non-adrenal cortical origin (metastasis & pheochromocytoma) FDG-PET and MTO-PET are not routinely recommend ---> lack of data
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Fine-needle aspiration biopsy
Cannot distinguish a benign adrenal mass from adrenal carcinoma, but between an adrenal and a metastatic tumor. sensitivity of 81–96 % and a specificity of 99–100 % inconclusive biopsies were reported in 6–50 % Complications of FNAB is ranging from % (pneumothorax, bleeding, infection, and pancreatitis). FNAB is useful in selected cases only: in patients with a history of an underlying extra-adrenal malignancy In case of inconclusive results of imaging tests if there is suspicion of a rare tumor (infiltrative, infection) ! It is mandatory to biochemically exclude a pheochromocytoma before FNAB is performed !
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Q2: Functional ?
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24-25 % = secreting
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Hormonal hypersecretion is most likely if mass is ≥ 3 cm in diameter
Occurs mostly within the first 3 years after diagnosis
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Cushing’s disease 3 major causes: Cushing dsease 68 %
Adrenal origin 20 % Para Neoplastic 12 %
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Cushing
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Cushing's syndrome. Howlett TA et al. Clin Endo Metab 1985
Symptoms Cushing's syndrome. Howlett TA et al. Clin Endo Metab 1985
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Cushing’s syndrome 5-20 % of pt with adrenal incidentaloma are reported to have subclinical Cushing syndrome Subclinical Cushing's syndrome mild hypercortisolism without clinical manifestations of Cushing's syndrome
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Hormonal evaluation Cushing's syndrome
Lack of suppressibility of cortisol after 1 mg overnight dexamethasone intake (NL: cortisol < 1.8 mcg/dL ) Supranormal 24-hour urinary cortisol excretion Disturbed cortisol circadian rhythm (midnight salivary cortisol) Low baseline secretion of ACTH Confirmatory (second line) tests: Blunted plasma ACTH responses to CRH Overnight 8 mg dexamethasone
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Diagnosis - Cushing
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Pheochromocytoma Screening for pheochromocytoma is mandatory in all case because high rate morbidity and mortality It is symptomatic up to 15% of case
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Pheo: Signs & Symptoms PHEO is easily suspected in someone with paroxysmal hypertension, resistant hypertension & in the presence of the so-called “Spells” the “5 P’s” Pressure (HTN) 90% Pain (Headache) 80% Perspiration 71% Palpitation 64% Pallor 42% Paroxysms (the sixth P!)
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Plasma free metanephrines
Screening test is measurement of plasma free or 24 hrs urines metanephrines Plasma free metanephrines sensitive 99 % specific %
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Confirmatory test
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Primary Hyperaldosteronism (Conn’s syndrome)
% of adrenal incidentalomas Pts with hypertension should be evaluated Hypokalemia suggests hyperaldosteronism (~ 40 %), this means that a normal K+ doesn’t exclude it ! The best screening test is the ratio of the plasma aldosterone : renin activity (> 20)
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Confirmatory test Saline infusion test —
IV 2l NaCl 0,9 % / 4h (from 8 AM to noon) the PAC will fall below 5 ng/dL (139 pmol/L) in normal subjects, whereas values above 10 ng/dL (277 pmol/L) are consistent with primary aldosteronism. Ahmed AH et al. Seated saline suppression testing for the diagnosis of primary aldosteronism: JCEM 2014
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Management of adrenal incidentaloma
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Natural evolution Some adrenal incidentalomas followed for an average of 4 years can: increase (range 0–26%) decrease (~ 4%) and/or can develop hyperfunction (range 0–11%) EJE (2015) 173, 275–282
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W.Young NEJM 2007;356:601-10
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Bilateral adrenal masses
The management of bilateral adrenal masses is different from that for unilateral masses!
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SUMMARY
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Benign versus Malign ? Functional ? Bilateral ≠ Unilateral
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Merci de votre attention
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