Pheochromocytoma

Pheochromocytoma - Pheochromocytoma Pheochromocytoma...

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Pheochromocytoma Pheochromocytoma William Harper, MD, FRCPC Assistant Professor of Medicine McMaster University
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Pheochromocytoma Pheochromocytoma 1. Catecholamine Physiology/Pathophysiology 2. Clinical Presentation 1. Epidemiology 2. 3. Diagnosis 1. Biochemical 2. Localization 4. Management 1. Preoperative 2. Operative 3. Postoperative 4. Pregnancy
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Catecholamine Producing Tumors Catecholamine Producing Tumors Neural Crest Sympathoadrenal Progenitor Cell (Neuroblasts) Chromaffin Cell Sympathetic Ganglion Cell Intra-adrenal Extra-adrenal Pheochromocytoma Ganglioneuroma Neuroblastoma
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Pheochromocytoma Paraganglioma (extra-adrenal pheo) Originate in extra-adrenal sympathetic chain/chromaffin tissue Ganglioneuroma Behave like paraganglioma biochemically Neuroblastoma Common malignancy in children, adrenal or sympathetic chain Catecholamine humoral effects usually minor Some differentiate and spontaneously regress Rx complex (surgery, XRT, chemotherapy) Catecholamine Producing Tumors Catecholamine Producing Tumors
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Cheodectoma Carotid body, behave like paraganglioma biochemically Glomus jugulare tumor Intracranial branch of CN IX and X Behave like paragangliomoa biochemically Catecholamine Producing Tumors Catecholamine Producing Tumors
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Tyrosine L-Dopa Dopamine Norepinephrine Epinephrine Catecholamines Normetanephrine Metaneprine PNMT DBH COMT COMT Metabolites Homovanillic acid (HVA) MAO, COMT Vanillymandelic Acid (VMA) MAO MAO Tumor 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 TH
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Adrenergic Receptors Adrenergic Receptors Alpha-Adrenergic Receptors α 1 : vasoconstriction , intestinal relaxation, uterine contraction, pupillary dilation α 2 : presynaptic NE (clonidine), platelet aggregation, vasoconstriction, insulin secretion Beta-Adrenergic Receptors β 1 : HR/contractility, lipolysis, renin secretion β 2 : vasodilation , bronchodilation, glycogenolysis β 3 : lipolysis, brown fat thermogenesis
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Pheochromocytoma Pheochromocytoma 1. Catecholamine Physiology/Pathophysiology 2. Clinical Presentation 1. Epidemiology 2. 3. Diagnosis 1. Biochemical 2. Localization 4. Management 1. Preoperative 2. Operative 3. Postoperative 4. Pregnancy
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Pheochromocytoma 0.01-0.1% of HTN population Found in 0.5% of those screened M = F 3 rd to 5 th decades of life Rare, investigate only if clinically suspicion: Signs or Symptoms Severe HTN, HTN crisis Refractory HTN (> 3 drugs) HTN present @ age < 20 or > 50 ? Adrenal lesion found on imaging (ex. Incidentaloma)
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This note was uploaded on 12/21/2011 for the course STEP 1 taught by Professor Dr.aslam during the Fall '11 term at Montgomery College.

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Pheochromocytoma - Pheochromocytoma Pheochromocytoma...

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