嗜铬细胞瘤0529资料.ppt

Pheochromocytoma 0.01-0.1% of HTN population Found in 0.5% of those screened M = F 3rd to 5th 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) Pheo: Signs Symptoms The five P’s: Pressure (HTN) 90% Pain (Headache) 80% Perspiration 71% Palpitation 64% Pallor 42% Paroxysms (the sixth P!) The Classical Triad: Pain (Headache), Perspiration, Palpitations Lack of all 3 virtually excluded diagnosis of pheo in a series of 21,0000 patients Pheo: ‘Rule of 10’ 10% extra-adrenal (closer to 15%) 10% occur in children 10% familial (closer to 20%) 10% bilateral or multiple (more if familial) 10% recur (more if extra-adrenal) 10% malignant 10% discovered incidentally MIBG Scan 123I or 131I labelled metaiodobenzylguanidine MIBG catecholamine precurosr taken up by the tumor Inject MIBG, scan @ 24h, 48h, 72h Lugol’s 1 gtt tid x 9d (from 2d prior until 7d after MIBG injection to protect thyroid) False negative scan: Drugs: Labetalol, reserpine, TCAs, phenothiazines Must hold these medications for 4-6 wk prior to scan Pheo Management Prior to 1951, reported mortality for excision of pheochromoyctoma 24 - 50 % HTN crisis, arrhythmia, MI, stroke Hypotensive shock Currently, mortality: 0 - 2.7 % Preoperative preperation, ?-blockade? New anesthetic techniques? Anesthetic agents Intraoperative monitoring: arterial line, EKG monitor, CVP line, Swan-Ganz Experienced Coordinated team: Endocrinologist, Anesthesiologist and Surgeon Preop Preperation Regimens Combined ? + ? blockade Phenoxybenzamine Selective ?1-blocker (ex. Prazosin) Propanolol Metyrosine Calcium Channel Blocker (CCB) Nicardipine No Randomized Clinical Trials to compare various regimens! 指南推荐 ●被确诊为嗜铬细胞瘤或副神经节瘤的患者应和医生共同决策，一齐评估是否需要进行基因检测； ●诊断流程将风险因素如年龄、家族史应当纳入考虑，以确定哪些患者进行基因或特定基因突变检测可获益最多； ●副神经节瘤患者和确诊为转移性肿瘤的患者应该进行这两种肿瘤的特定基因突变检测 ●计算机断层扫描（CT）可作为首选的影像检测手段，用于嗜铬细胞瘤或