肾上腺嗜铬细胞瘤课件

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,Pheochromocytoma and Paraganglioma,中南大学湘雅二医院代谢内分泌研究所 盛志峰,Pheochromocytoma and Paragangl,1,WHO definition(2004),Intra-adrenal paraganglioma,Pheochromocytoma(NE,EP),Extra-adrenal paragangliomas,extra-adrenal sympathetic(abdominal,pelvis)or parasympathetic paraganglia(head,neck)(NE,DA),Zuckerkandl organ(NE,EP),WHO definition(2004)Intra-adr,2,Significance of diagnosis,(1)curable hypertension:surgical removal,(2)A risk of lethal paroxysm exists,(3)10%of the tumors are malignant,(4)between 1020%of these tumors are familial,and their detection in the proband may result in early diagnosis in other familymembers,Significance of diagnosis(1)c,3,Clinical Presentation,1 Hypertension/cardiovascular disease (paroxysmal/sustained/resistant),lability in blood pressure and orthostatic hypotension,episodic release of catecholamines,chronic volume depletion,impaired sympathetic reflexes.,Clinical Presentation1 Hyper,4,In addition to volume depletion,altered sympathetic vascular regulation may have a role in orthostasis,which is frequently observed in patients with pheochromocytoma,Symptoms of orthostatic hypotension(e.g.,lightheadedness,presyncope,syncope)may dominate the presentation,especially in patients with epinephrine-or dopamine-predominant tumors,hypotenison/shock,/,Heart disease,In addition to volume depleti,5,2 Metabolic disturbances(glucose/lipid/electrolytes),Fasting hyperglycemia and diabetes mellitus are caused in part by the-adrenergic inhibition of insulin release.,Although hypercalcemia may be a sign of MEN2,it is usually isolated and resolves with resection of the catecholamine-secreting tumor.,Calcitonin secretion is in part a catecholamine dependent process;serum calcitonin concentrations are frequently mildly elevated in patients with pheochromocytoma,usually unrelated to MEN2.,2 Metabolic disturbances(g,6,3.Additional clinical signs of pheochromocytoma,Constipation/Raynauds phenomenon,livedo reticularis,erythrocytosis,mass effects from the tumor,Painless hematuria and paroxysmal attacks induced by micturition and defication are associated with urinary bladder paragangliomas.,3.Additional clinical signs o,7,Some of the cosecreted hormones,ACTH(Cushings syndrome),parathyroid hormone-related peptide(hypercalcemia),vasopressin(syndrome of inappropriate antidiuretic,hormone secretion),vasoactive intestinal peptide(watery diarrhea),growth hormonereleasing hormone(acromegaly).,Some of the cosecreted hormone,8,Clinical Presentation,Clinical Presentation,9,肾上腺嗜铬细胞瘤课件,10,A“rule of 10”,10%extra-adrenal,10%occur in children,10%multiple or bilateral,10%recur after surgical removal,10%malignant,10%are familial(nowadays 20%),10%of benign sporadic adrenal pheochromocytomas are found as adrenal incidentalomas,A“rule of 10”10%extra-adrena,11,hereditary syndromes with pheochromocytomas or paragangliomas,hereditary syndromes with pheo,12,Case Detection,Hyperadrenergic spells(e.g.,self-limited episodes of palpitations,diaphoresis,headache,tremor,or pallor),Resistant hypertension,A familial syndrome that predisposes to catecholamine secreting,tumors(e.g.,MEN2,NF1,VHL),A family history of pheochromocytoma,An incidentally discovered adrenal mass,Hypertension and diabetes,Pressor response during anesthesia,surgery,or angiography,Onset of hypertension at a young age(20 years),Idiopathic dilated cardiomyopathy,A history of gastrointestinal stromal tumor or pulmonary chondromas(Carney triad),Case DetectionHyperadrenergic,13,肾上腺嗜铬细胞瘤课件,14,Catecholamine metabolism,Catecholamine metabolism,15,Sensitivity and specificity of biochemical tests for the detection of pheochromocytoma or paraganglioma,Biochemical test Sensitivity,Sensitivity(%),Specificity(%),Children,Adults,Children,Adults,Plasma normetanephrine and metanephrine,100,99,94,89,Plasma norepinephrine and epinephrine,92,84,91,81,Urinary normetanephrine and metanephrine,100,97,95,69,Urinary norepinephrine and epinephrine,100,86,83,88,Urinary vanillylmandelic acid,-,64,-,95,Sensitivity and specificity of,16,Blood sampling should be performed at a supine position after about 1520 mins of i.v.catheter insertion.,Food,caffeinated beverages,strenuous physical activity,or smoking are not permitted at least about 812 hours before the testing.The elevation of plasma metanephrines of more than 4-fold above the upper reference limit is associated with close to 100%probability of the tumor.,Blood sampling should be perfo,17,肾上腺嗜铬细胞瘤课件,18,Clonidine Suppression Test,Clonidine is a centrally acting 2-adrenergic,receptor agonist that normally suppresses the release of,catecholamines from neurons but does not affect the catecholamine,secretion from a pheochromocytoma.,Clonidine(0.3 mg)is administered orally,and plasma fractionated,catecholamines or metanephrines are measured before and,3 hours after the dose.,In patients with essential hypertension,plasma catecholamine/,normetanephrine,concentrations decrease.,However,these concentrations remain increa