嗜铬细胞瘤诊断与定位的进展

血浆儿茶酚胺的测定对嗜铬细胞瘤诊断和定位是一种最敏感和最可靠的方法。其诊断标准：静息卧位血浆去甲肾上腺素＞１１．８２ｐｍｏｌ／ｍｌ及／或肾上腺素＞５．４６ｐｍｏｌ／ｍｌ。肿瘤主要以持续弥散方式释放儿茶酚胺，根据其释放量，可分为多分泌型与少或不分泌型。血浆儿茶酚胺浓度取决于肿瘤分泌会，而与肿瘤大小和儿茶酚胺总含量无关。腔静脉分段取血定位肿瘤阳性率为９７％。分别测定血浆去甲肾上腺素和肾上腺素有助于肿瘤位置的判断。对极少数病例，血浆儿茶酚胺正常不能完全排除嗜铬细胞瘤。因生理病理应激使血浆儿茶酚胺升高时，也不要误诊为嗜铬细胞瘤。我国在这方面研究已达到国际先进水平。     

嗜铬细胞瘤分子生物学研究进展

嗜铬细胞瘤是引起继发性高血压的原因之一，死亡率高，因此，寻找嗜铬细胞瘤特异性早期诊断方法及区别嗜铬细胞瘤良恶性。是目前亟待解决的难题。综合运用分子生物学诊断技术，对早期诊断治疗嗜铬细胞瘤及降低死亡率有重要意义。本文综述嗜铬细胞瘤分子生物学的有关进展。     

嗜铬细胞瘤的诊疗进展

肾上腺疾病是继发性高血压的重要病因,临床上最常见引起高血压的肾上腺性疾病是原发性醛固酮增多症和嗜铬细胞瘤。尤其是嗜铬细胞瘤,以前认为是一种罕见病,但随着诊断技术的提高和临床医生对此病的认识增强,发现其在高血压人群中相当多见,近年国外报道高血压人群患病率约为1.9%     

母女同患嗜铬细胞瘤

1临床资料例1,女,56岁,因头痛、头昏、胸闷1年加重10天于1995年8月10日入院。患者1年来经常头痛、头昏、胸闷、心悸、出汗、恶心、呕吐,发作时面色苍白、出汗、四肢湿冷,每次持续几分钟至1小时,血压波动在150～220/80～130mmHg。一直诊断为高血压病3级、冠心病,服用多种降压药,效果不佳。入院前10天,因上述症状加重,以高血压病3级、冠心病入院。查体:中年女性,面色苍白。T36℃,P110次/分,BP200/110mmHg。甲状腺不大,颈软。双肺(-),心界不大,心率110次/分,律齐,A2>P2,各瓣膜区无杂音。腹部(-),尿常规、血常规、电介质、肾功均正常。心电…     

嗜铬细胞瘤150例临床分析和诊断探讨

对150例嗜铬细胞瘤的临床分析中,肿瘤位于肾上腺者120例、肾上腺外30例、恶性16例。全部病例均有高血压,表现类型多样。全部病例于高血压状态下尿儿茶酚胺或其主要代谢产物VMA均增高。作者对肿瘤定位检查方法的选择也作了探讨。     

散发性嗜铬细胞瘤基础研究进展

嗜铬细胞瘤可分为遗传性和散发性两大类.基因突变在嗜铬细胞瘤的发生中起重要作用.对散发性嗜铬细胞瘤患者进行基因筛查,可以早期发现遗传性疾病,早期治疗.本文阐述了目前对散发性嗜铬细胞瘤基因筛查的临床进展及其意义.     

腔静脉血浆儿茶酚胺对嗜铬细胞瘤的定位

腔静脉血浆儿茶酚胺对嗜铬细胞瘤的定位金律，刘力生，王蕾礼，郑德裕应用高效液相色谱电化学检测法（ＨＰＬＣ－ＥＣ）测定腔静脉血浆儿茶酚胺（ＣＡ）对嗜铬细胞瘤定位，并探索此法对肿瘤定位的标准。一、对象和方法：经手术、病理与肿瘤ＣＡ检查证实的嗜铬细胞瘤患者９...     

嗜铬细胞瘤诊治进展

嗜铬细胞瘤是一种功能性肿瘤,临床症状多样。如能及时诊断及恰当治疗,绝大部分患者预后良好,否则可出现严重并发症甚至死亡。1　定性诊断1.1　生化检验　在现有生化检验中,血及尿儿茶酚胺测定和尿甲氧基肾上腺素测定具有较高的敏感性和特异性,而尿3-甲氧基-4羟基苦杏仁酸(VMA)的敏感性及特异性较低[1]。24小时尿甲氧基肾上腺素对嗜铬细胞瘤诊断的敏感性为98%。高压液相(HPLC)测定去甲肾上腺>80μg/24h、肾上腺素>20μg/24h,多巴胺>400μg/24h对本病诊断极敏感,但特异性差。将24小时尿去甲肾上腺素>170μg、肾上腺素>35μg确定为阳性标准,…     

血浆儿茶酚胺对嗜铬细胞瘤诊断标准的探讨

根据37例嗜铬细胞瘤患者血浆和肿瘤组织几茶酚胺值,提出了诊断标准:静息卧位时血浆去甲肾上腺素>2ng/ml及(或)肾上腺素>1ng/ml。其阳性率为94.6％。并对一些传统概念进行了探讨。     

The role of combined imaging in metastatic medullary thyroid carcinoma: <Superscript>111</Superscript>In-DTPA-octreotide and <Superscript>131</Superscript>I/<Superscript>123</Superscript>I-MIBG as predictors for radionuclide therapy

Purpose The medical treatment of metastatic medullary thyroid carcinoma (MTC) is still questionable. The aim of this study was to evaluate a combined imaging protocol using ¹¹¹In-DTPA-octreotide and ¹³¹I/¹²³I-MIBG to decide whether targeted radiotherapy would be useful, and which radiopharmaceutical (⁹⁰Y-DOTATOC or ¹³¹I-MIBG) would be more effective.Methods Eight patients (four men, four women; mean age 61 years) with metastatic MTC were included. Treatments were performed with 3,330 MBq ⁹⁰Y-DOTATOC at 6-week intervals, or 11.1 GBq ¹³¹I-MIBG with a minimum interval of 3 months.Results The imaging procedure was positive in all eight patients: ¹¹¹In-DTPA-octreotide imaging in five patients, ¹³¹I/¹²³I-MIBG imaging in four patients. With respect to the number of metastatic lesions, MIBG imaging was less effective than octreotide. According to the results of combined imaging, we identified one patient to be treated with ⁹⁰Y-DOTATOC, and three patients with ¹³¹I-MIBG. An overall antitumor effect was observed in all four patients, one with partial remission and three with stable disease. No relevant toxicity was observed.Conclusions The combined imaging can increase the detection rate of metastatic foci in patients with MTC and identify more patients for effective radionuclide treatment. The treatment with ⁹⁰Y-DOTATOC or ¹³¹I-MIBG is well tolerated and may improve the fate of patients with metastatic MTC.     

Biochemical tests for pheochromocytoma

Objective:To determine the optimal clinical strategy utilizing either 1) serum catecholamines, 2) urinary metaphrines, or 3) urinary vanillylmandelic acid measurements in the evaluation of hypertensive patients with suspected pheochromocytoma. Design:Prospective clinical determination of test-operating characteristics. Setting:Tertiary care university medical center. Patients/Participants:415 patients referred for evaluation of suspected pheochromocytoma. Interventions:All subjects bad measurements of the three above-mentioned biochemical tests with the diagnosis of pheochromocytoma established by tissue confirmation. Results:All three biochemical tests were similar in sensitivity (0.70–0.75), specificity (0.90–0.95), and receiver operating characteristics. With an estimated disease prevalence of 5.9% in symptomatic hypertensive patients, the predictive value of any single negative test would be 98% in ruling out disease. Conclusions:All three biochemical tests have similar performance characteristics and only a single test need be performed to exclude pheochromocytoma in most symptomatic hypertensive patients in a primary care population.     

嗜铬细胞瘤分子病因学研究进展

嗜铬细胞瘤/副神经节瘤(pheochromocytoma/paraganglioma, PPGL)是一种罕见的神经内分泌肿瘤,约40%的PPGL具有家族遗传性,其发病与已知致病基因的胚系突变相关。此外,肿瘤的体细胞基因突变、拷贝数变异、甲基化程度和非编码RNA也参与PPGL的发生。全面和深入了解PPGL的发病机制,将为...     

嗜铬细胞瘤20例临床分析

目的：探讨嗜铬细胞瘤临床特点，提高诊治水平。方法：对20例嗜铬细胞瘤的临床资料总结分析。结果：20例均经手术治疗，单侧17例，家族性双侧3例，恶性嗜铬细胞瘤4例，肾上腺外嗜铬细胞瘤1例，无症状嗜铬细胞瘤4例。结论：嗜铬细胞瘤典型表现是高血压、头痛、心悸和出汗，而无症状性、家族性和其他非典型的特殊表现需引起注意。儿茶酚胺及影像学检查是主要的诊断手段。     

Sensitivity of diagnostic and localization tests for pheochromocytoma in clinical practice

BACKGROUND: Although pheochromocytomas are believed to account for fewer than 0.3% of all cases of hypertension, aggressive diagnostic and surgical intervention is recommended whenever a pheochromocytoma is suspected because uncontrolled catecholamine release from the tumors can lead to catastrophic consequences. Many biochemical diagnostic and imaging localization tests exist for detecting pheochromocytomas. We sought to evaluate the sensitivity of these tests used over a 35-year period at a single institution. METHODS: Thirty-five patients with complete medical records who had pathologically confirmed pheochromocytomas between 1962 and 1997 at the University of Chicago Hospitals were identified. Sensitivity and 95% confidence intervals were calculated for 12 laboratory diagnostic tests and 5 imaging studies. RESULTS: The most sensitive laboratory diagnostic tests in our study were plasma total catecholamines (95%) and urine total metanephrines (100%). Testing for urine vanillylmandelic acid, while less expensive and easier to perform than many other tests, had a slightly lower sensitivity (89%). The most sensitive imaging tests in the study were magnetic resonance imaging (100%) and iodine I-131 metaiodobenzylguanidine scintigraphy (100%). The more often used computed tomography had only 88% sensitivity. Localization was safely and successfully performed on two pregnant patients using magnetic resonance imaging and ultrasound. CONCLUSIONS: By properly choosing from the wide array of laboratory diagnostic and imaging tests, pheochromocytomas can be identified and localized with nearly 100% sensitivity. These tests should be performed in any patient for whom the diagnosis of pheochromocytoma is being considered.     

核素心肌灌注显像与三种心电方法诊断局灶性心肌梗死的对比研究

目的 :对比评价核素心肌灌注显像与心电图 （ECG）、高频心电图 （HFECG）及体表电位标测 （BSPM）三种心电方法对局灶性心肌梗死的诊断价值。方法 :采用结扎法 ,建立 10只局灶性心肌梗死犬动物模型。术后 2周 ,依次行核素心肌灌注显像、ECG,HFECG及 BSPM检查 ,术前行 HFECG和 BSPM检查 ,以作对比。结果 :ECG和HFECG检测局灶性心肌梗死的阳性率较低 ,仅为 5 0 % ,BSPM各单项参数检出的阳性率高于 ECG和 HFECG,多项指标平行试验诊断的敏感性与核素心肌显像相当 ,均为 90 %。结论 :BSPM和核素心肌显像对局灶性心肌显像均有较高的诊断价值 ,且核素心肌显像又能直观地反映梗死部位、范围和程度。     

嗜铬细胞瘤的诊治

一例34岁男性因波动性高血压疑为嗜铬细胞瘤而转至本科就诊。该患者临床表现典型,人院查血浆甲氧基肾上腺素(MN)及甲氧基去甲肾上腺素(NMN)均明显升高,CT和PET-CT检查均发现左侧肾上腺占位,诊断为嗜铬细胞瘤。术前予以甲磺酸多沙唑嗪控释片4 mg/d口服2周,血压平稳,在腹腔镜下行左侧肾上腺肿瘤切除术。术后病理提示嗜铬细胞瘤,MN、NMN、血压恢复正常,症状缓解。该患者术后随访3年,血压、血浆MN、NMN水平均正常,肾上腺CT未见肿瘤复发。