资源描述
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,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),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,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.,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,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.,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,.,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,).,Clinical Presentation,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,hereditary syndromes with,pheochromocytomas,or,paragangliomas,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),Catecholamine metabolism,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,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.,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 increased in patients with,pheochromocytoma,.,123,I-labeled meta-,iodobenzylguanidine,scintigraphy,(,123,I-MIBG),18,F-FDOPA PET,preoperative,preperation,Phenoxybenzamine,(,Dibenzyline,),an,-adrenoceptor,blocker,is most commonly used for preoperative control of blood pressure.The drug is initially administered orally at a dose of,1020 mg twice daily.,Alternatives to,phenoxybenzamine,for blockade of,catecholamine-induced vasoconstriction include calcium channel blockers and selective,competitive 1-adren
展开阅读全文