adrenal tumor 30-9-13.ppt bc,mdbc,mdsf,sdfkhkafhz,mdcn,mdsfkjadsfmzdbbvs,jfgkjahfkew
DESCRIPTION
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Tumor adrenaldr Putra Hendra SpPDUNIBA
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Adrenal Histology
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Regulation of adrenal gland secretionACTHCortisolCortisol
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Overview of the interactions of hormones in response to stress
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Adrenal Gland Role in GAS
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Approach to the Adrenal MassFunctional versus Non-functional?
Malignant versus Benign?
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DiagnosisPreviously, histopathology was gold standard
Currently, various imaging techniques are regarded as ideal, due to ease:CT with or without contrast (83% accurate in diagnosing malignancy)MRI (94% accurate) Scintigraphy (92% accurate)
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Unilateral adrenal massesFunctional leisonsAdrenal adenoma Adrenal cortical carcinoma.Pheochromocytoma
Non Functional leisonsAdrenal adenoma Adrenal cortical carcinoma.GanglioneuromaMyelolipomaHemorrhage in adrenal gland.Metastases to the adrenal gland.
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Bilateral adrenal massesFunctional leisonsACTH dependant cushing syndromeCongenital adrenal hyperplasia.pheochromocytoma
Non Functional leisonsInfection(T.B,Fungus).Infiltrative (amyloidosis ,leukemia,lymphoma)Hemorrhage.
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DiagnosisNo. (%) of LesionsAdenoma788 (75)Myelolipoma68 (6)Hematoma47 (4)Cyst13 (1)Pheochromocytoma3 (0.3)Macronodular Hyperplasia1 (0.1)Adrenal Cortical Neoplasm of unknown malignant potential1 (0.1)Presumed benign by imaging/clinical stability128 (12)Total1049 (100)
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Functional tumors:Adrenal Medullary Tumors:PheochromocytomaChildhood tumors: ganglioneuromas, neuroblastomasAdrenal Cortical Tumors: Cortisol-secreting adenomaAldosteronomaCarcinoma
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ADRENAL GLAND DYSFUNCTIONHypercortisolism= Cushings SyndromeHypocortisolism= Addisons DiseaseConn syndromePheochromocytoma
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Hypo- & Hyper- Function of the Adrenal Cortex
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PheochromocytomaCatecholamine Physiology/PathophysiologyClinical PresentationEpidemiologySigns & SymptomsDiagnosisBiochemicalLocalizationManagementPreoperativeOperativePostoperativePregnancy
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Pheochromocytoma0.01-0.1% of HTN populationFound in 0.5% of those screenedM = F3rd to 5th decades of lifeRare, investigate only if clinically suspicion:Signs or SymptomsSevere HTN, HTN crisisRefractory HTN (> 3 drugs)HTN present @ age < 20 or > 50 ?Adrenal lesion found on imaging (ex. Incidentaloma)
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FREQUENCY OF VARIOUS DIAGNOSES IN HYPERTENSIVE PATIENTS 1980s
PRIMARY CAREREFERRAL Essential 92-95% 89% Chronic kidney dis 3-6% 5% Renovascular dis 0.2-1.0% 4% Pheochromocytoma 0.1-0.2% 0.2% Aldosteronism 0.1-0.3% 0.5% Cushings syndrome 0.1-0.2% 0.2% Coarctation 0.1-0.2% 1% Oral contraceptives 0.2-1.0%
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Catecholamine Producing TumorsNeural CrestSympathoadrenal Progenitor Cell(Neuroblasts)Sympathetic Ganglion CellIntra-adrenal Extra-adrenalPheochromocytomaGanglioneuromaNeuroblastoma
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TyrosineL-DopaDopamineNorepinephrineEpinephrineCatecholaminesNormetanephrineMetaneprinePNMTDBHCOMTCOMTMetabolitesHomovanillic acid(HVA)MAO, COMTMAOMAOTumor Secretion: Large Pheo: more metabolites
(metabolized within tumor before release) Small Pheo: more catecholamines Sporadic Pheo: Norepi > Epi Familial Pheo: Epi > Norepi Paraganglioma: Norepi Cheodectoma, glomus jugulare: Norepi Gangioneuroma: Norepi Malignant Pheo: Dopamine, HVA Neuroblastoma: Dopamine, HVA
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Clinical manifestationsIntermittent hypertension or even hypotension headache 71%, palpitations 65%, sweating 65% and dizziness 15%, sense of doomVomiting 28%, weakness 17%, nervousness 10%, pallor 16%, dyspnea 19%, substernal pain 12%, and abdominal pain 12%Glycosurianeutrophelia
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Pheo: Signs & SymptomsThe five Ps: (5P)Pressure (HTN)90%Pain (Headache)80%Perspiration71%Palpitation64%Pallor42%Paroxysms (the sixth P!)The Classical Triad: (3P)Pain (Headache), Perspiration, PalpitationsLack of all 3 virtually excluded diagnosis of pheo in a series of > 21,0000 patients
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Pheo: Paroxysms, Spells10-60 min durationFrequency: daily to monthlySpontaneousPrecipitated:Diagnostic procedures, I.A. Contrast (I.V. is OK)Drugs (opiods, unopposed -blockade, anesthesia induction, histamine, ACTH, glucagon, metoclopramide)Strenuous exercise, movement that increases intra-abdo pressure (lifting, straining)Micturition (bladder paraganlgioma)
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Pheo: Signs & SymptomsN/V, abdo pain, severe constipation (megacolon)Chest-painsAnxietyAngina/MI with normal coronaries:Catecholamine induced: myocardial oxygen consumption or coronary vasospasmCHFHTN hypertrophic cardiomyopathy diastolic dysfn.Catechols induce dilated cardiomyopathy systolic dysfn.Cardiac dysrhythmia & conduction defects
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Pheo: Signs (metabolic)HypercalcemiaMild glucose intoleranceLipolysisWeight-lossKetosis > VLDL synthesis (TG)
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Pheo: Rule of 1010% extra-adrenal (closer to 15%)10% occur in children10% familial (closer to 20%)10% bilateral or multiple (more if familial)10% recur (more if extra-adrenal)10% malignant10% discovered incidentally
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DiagnosisPlasma free metanephrines most sensitive testseen 99% of patients24 urinary catecholamines (2x normal is diagnostic)VMAClonidine suppression test
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24h Urine Collection24h urine collection:Creatinine, catecholamines, metanephrines, vanillymandelic acid (VMA), +/-dopamineHPLC with electrochemical detection or mass spectPositive results (> 2-3 fold elevation):24h Ucatechols > 2-fold elevationULN for total catechols 591-890 nmol/d 24h Utotal metanephrines > 1.2 ug/d (6.5 umol/d)24h UVMA > 3-fold elevationULN 35 umol/d for most assays
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Suppression/Stimulation TestingClonidine suppressionMay precipitate hypotensive shock!Unlike normals, pheo patients wont suppress their plasma norepi with clonidineGlucagon stimulationMay precipitate hypertensive crisis!Pheo patients, but not normals, will have a > 3x increase in plasma norepi with glucagon
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Localization: ImagingCT abdomenAdrenal pheo SEN 93-100%Extra-adrenal pheo SEN 90%MRI> SEN than CT for extra-adrenal pheo
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MIBG Scan123I or 131I labelled metaiodobenzylguanidineMIBG catecholamine precurosr taken up by the tumorInject MIBG, scan @ 24h, 48h, 72hLugols 1 gtt tid x 9d (from 2d prior until 7d after MIBG injection to protect thyroid)False negative scan:Drugs: Labetalol, reserpine, TCAs, phenothiazinesMust hold these medications for 4-6 wk prior to scan
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Pheo ManagementPrior to 1951, reported mortality for excision of pheochromoyctoma 24 - 50 %HTN crisis, arrhythmia, MI, strokeHypotensive shockCurrently, mortality: 0 - 2.7 %Preoperative preperation, -blockade?New anesthetic techniques?Anesthetic agentsIntraoperative monitoring: arterial line, EKG monitor, CVP line, Swan-GanzExperienced & Coordinated team:Endocrinologist, Anesthesiologist and Surgeon
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Pheo: Unresectable, Malignant-blockadeSelective 1-blockers (Prazosin, Terazosin, Doxazosin) 1st line as less side-effectsPhenoxybenzamine: more complete -blockade-blockerCCB, ACE-I, etc.Nuclear Medicine Rx:Hi dose 131I-MIBG or 111indium-octreotide depending on MIBG scan or octreoscan pick-upSensitize tumor with Carboplatin + 5-FU
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Cortisol-secreting Adenoma:the sweet partEpi: F > M; ages 30-50sSigns/Symptoms:May lead to clinical or sub-clinical Cushings syndromeMost commonly: obesity, HTN, glucose intolerance or DMII, hypercholesterolemiaLabwork demonstrates:Low DHEA-sulfate (increased cortisol suppresses pituitary-adrenal axis)Elevated 24 hour urinary cortisol
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Aldosteronomas:the salty partEpi: M, age 30-40Signs/Symptoms: Screen all HTN pts with incidental massLabwork reveals:Increased serum aldosteroneDecreased plasma renin activity
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Primary Hyperaldosteronism (Conn's Disease)Solitary adrenal adenomas (80-90%)Bilateral adrenal hyperplasia (10-20%)Idiopathic HyperaldosteronismAccounts for 50% of cases at some referral centersAdrenal Carcinoma (rare)Unilateral Adrenal Hyperplasia (very rare)
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TherapyIf tumor with lateralization, laparascopic adrenalectomyIf tumor without lateralization (incidentaloma), or hyperplasia, then aldosterone blockadeSpironolactoneEplerinone
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Medical Therapy of PA eplerenone 50-200 mg/day (give BID) spironolactone 25 - 100 mg / day
( amiloride allows for lower spironolactone)
FH-1: dexamethasone 0.25-1.0 mg/day
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Eplerenone
affinity 20 X > spironolactonePotency of effect 75% of spironolactoneBinding affinity for androgen and progesterone receptors 1000-fold lower than spironolactone
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