异位嗜铬细胞瘤

目的了解异位嗜铬细胞瘤患者的临床特点,探讨诊断和治疗方法.方法回顾性分析12例异位嗜铬细胞瘤患者的临床表现、定位和定性检查以及治疗效果的资料.结果 11例患者表现为持续性高血压,伴阵发性加重.11例测定了血儿茶酚胺,2例同时测定了24 h尿儿茶酚胺.6例进行了腔静脉分段取血.8例患者手术治愈,4例患者药物控制良好.结论异位嗜铬细胞瘤以持续性高血压伴阵发性加重表现为主.血儿茶酚胺检查有助于病例诊断.无禁忌症患者应手术治疗.     

嗜铬细胞瘤23例临床特征分析

目的通过总结嗜铬细胞瘤的临床表现,提高该病的诊治水平。方法回顾性分析23例嗜铬细胞瘤患者的临床表现、生化检查、影像学检查及辅助检查。结果 23例病例的主要临床表现为高血压伴头痛、心悸,其中阵发性高血压18例,占78.3%;持续性高血压5例,占21.7%。24小时尿儿茶酚胺均高于正常值。追踪观察手术治疗效果,疗效明显。结论对有高血压并伴有头痛、心悸的患者,应考虑嗜铬细胞瘤的诊断,以减少漏诊误诊。     

儿茶酚胺心肌病并发室颤1例

正嗜铬细胞瘤是一种较为少见的神经内分泌肿瘤,其分泌高浓度的儿茶酚胺类物质入血,导致持续性或间歇性的高血压,同时入血的大量儿茶酚胺也可导致心肌的损害[1],嗜铬细胞瘤中儿茶酚胺心肌病的发病率为32%～65.4%,其心肌损害通常预后良好,尽早手术切除肿瘤以及积极的药物治疗可使心脏损害恢复[2]。本文报告嗜铬细胞瘤伴儿茶酚胺心肌病并发室颤1例的诊断及治疗过程,并结合近年来国内外     

嗜铬细胞瘤的心血管表现(待续)

嗜铬细胞瘤心血管的临床表现多样,但通常表现为持续性或阵发性高血压,同时还伴有儿茶酚胺分泌过量的其他相关症状和体征。其中最危及生命的心血管表现,如高血压急症,是由于肿瘤组织突然分泌大量儿茶酚胺所致。嗜铬细胞瘤患者还可能表现为低血压甚至休克,这些表现可能出现在多系统危象之前。嗜铬细胞瘤中最常见的心律失常是窦性心动过速和心悸,也可表现为更严重的室性心律失常或传导障碍。随着对应激性心肌病关注的日益增多,人们发现嗜铬细胞瘤还可表现为可逆性的扩张型或肥厚型心肌病。该文对嗜铬细胞瘤心血管表现的病因、临床表现和治疗进行综述。由于嗜铬细胞瘤的心血管并发症可危及生命,对有临床表现的患者,即使儿茶酚胺分泌正常,也应进行嗜铬细胞瘤的筛查。     

197例嗜铬细胞瘤临床分析

目的提高嗜铬细胞瘤的诊治水平。方法回顾性分析郑州大学第一附属医院1986～2005年所有病理诊断为嗜铬细胞瘤的197例患者的临床资料。结果197例患者均经手术治疗。良性嗜铬细胞瘤184例,恶性13例;肾上腺原发肿瘤171例,异位嗜铬细胞瘤24例,多发内分泌腺瘤（MEN）Ⅱ（嗜铬细胞瘤伴甲状腺髓样癌）2例。结论完善检查手段可提高嗜铬细胞瘤的检出率,确诊需病理检查,手术是根本的治疗方法。     

嗜铬细胞瘤26例临床分析

目的本文对２６例经手术病理证实的嗜铬细胞瘤进行回顾性分析。结果嗜铬细胞瘤临床主要表现为阵发性高血压，头痛、心悸、大汗等症状。不典型临床表现有：（１）无症状；（２）进行性视力下降；（３）脑血管意外。综合本组病例的临床资料，诊断嗜铬细胞瘤的要点有：（１）病史中存在阵发性高血压的线索；（２）血中儿茶酚胺浓度增高；（３）１３１Ｉ－ＭＩＢＧ对诊断嗜铬细胞瘤具有高的敏感性和特异性，并兼有定位及定性诊断价值     

60例嗜铬细胞瘤的心电图分析

嗜铬细胞瘤(PHEO)分泌过多的儿茶酚胺,除引起高血压外,还对心脏产生损害,有人称之为儿茶酚胺心肌病。本文对60例PHEO患者手术前后心电图变化进行分析。 病例为1970～1995年入院确诊的病人,其中男性32例,女性28例,年龄10～70岁,平均年龄41.4岁。59例均经手术切除治疗及病理证实为PHEO(良性51例,恶性8例),另一例为临床诊断,CT及MRI显示肿瘤位于右侧肾上腺。本组临床表现:阵发性高血压30例,持续性高血压10例,持续性高血压伴阵发性加剧20例,胸闷气促30例,视力下降13例,昏厥7例,胸痛     

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。     

嗜铬细胞瘤伴心血管损害的临床分析

目的了解嗜铬细胞瘤(pheochromocytoma,PHEO)心血管损害的临床特点。方法回顾分析74例嗜铬细胞瘤患者的临床症状,血生化、心肌酶谱、血常规、血与尿儿茶酚胺、心电图及治疗情况等临床资料。嗜铬细胞瘤的定位诊断采用超声、CT和/或MRI,定性采用病理诊断。结果入选的74例嗜铬细胞瘤患者中男53例,女21例,年龄13~76岁,平均(48.24±1.62岁),病程1天~30年。以嗜铬细胞瘤三联症之一为首发表现的患者有27例(36.49%)、高血压56例(75.68%)、低血压3例(4.05%)、急性左心衰竭3例(4.05%)、合并冠心病3例(4.05%)、儿茶酚胺心肌病4例(5.41%)。心电图检查窦性心动过速10例(13.51%),窦性心动过缓5例(6.76%),低钾血症5例(6.76%)。18例患者行心肌酶谱检查,其中16例增高(88.89%)。结论嗜铬细胞瘤患者多伴有高血压、少数可伴有低血压,心律失常以快速性心律失常为主,严重者可并发儿茶酚胺心肌病及心力衰竭。     

嗜铬细胞瘤患者血压与儿茶酚胺分泌的昼夜变化

目的　研究嗜铬细胞瘤患者手术前后血压及尿儿茶酚胺排泄量的昼夜变化及两者之间的关系。方法　 2 7例嗜铬细胞瘤患者术前进行动态血压监测 ,其中 12例在术前及术后 10天进行动态血压监测并同日留取十段尿待测尿儿茶酚胺排泄量 ,应用Cosinor方法分析收缩压、舒张压、心率及去甲肾上腺素、肾上腺素和多巴胺排量的昼夜变化。结果　术前不同血压类型患者的血压昼夜变化有所不同 ,持续性高血压患者的血压昼夜变化消失 ,阵发性高血压及正常血压患者血压昼夜变化存在。去甲肾上腺素、肾上腺素和多巴胺分泌的昼夜变化存在 ,肾上腺素、多巴胺的分泌高峰相有所后延。手术切除肿瘤后 ,患者的尿儿茶酚胺排泄量与血压的昼夜变化均恢复正常。 12例嗜铬细胞瘤患者术前的 2 4h平均收缩压、舒张压与 2 4h尿平均去甲肾上腺素排泄量呈明显正相关 (r =0 .83、0 .91,P <0 .0 0 1)。结论　嗜铬细胞瘤患者的儿茶酚胺分泌的昼夜变化存在 ;持续性高血压患者血压的昼夜变化消失 ,阵发性高血压患者血压的昼夜变化存在。     

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。     

严重发作性高血压-伪嗜铬细胞瘤的治疗

目的报道严重发作性高血压-伪嗜铬细胞瘤的治疗。方法总结一组酷似嗜铬细胞瘤的严重发作性高血压：伪嗜铬细胞瘤,男女各两例,平均年龄42.50±8.06岁,归纳其临床特点。结果伪嗜铬细胞瘤具有嗜铬细胞瘤的类似症状：①突然血压升高、②伴随躯体不适症状。伪嗜铬细胞瘤区别于嗜铬细胞瘤及其他高血压之处：①无儿茶酚胺代谢物指标升高。②并非由情感因素直接引起,多数患者具有创伤史或消极的对应方式,③排除嗜铬细胞瘤及酷似嗜铬细胞瘤症状体征的疾患。④β、α1受体阻滞剂、心理治疗必要时联合抗抑郁药、抗焦虑药有效。结论伪嗜铬细胞瘤有别于嗜铬细胞瘤及一系列酷似嗜铬细胞瘤的临床情况,β、α1受体阻滞剂、心理治疗必要时联合抗抑郁药、抗焦虑药有效。     

Long Term Follow-Up After Surgical Removal of Pheochromocytoma - Observations in 61 Patients

89 patients were operated on for pheochromocytoma. 61 patients (37 women and 24 men) were available for extended follow-up. The final survey, performed 79.1±66.9 months postoperatively, provided data on survival, blood pressure tumor recurrence, malignant metastatic lesions, cardiovascular complications and coexisting diseases. There were 4 deaths during the follow-up period, including 2 instances of malignant pheochromocytoma. Permanent normalization of blood pressure was achieved in 38 patients (62.3%). This hypotensive effect was noted in 79.2% of patients with preoperative paroxysmal hypertension and in 40.8% of those with sustained hypertension. Permanent or re-developing postoperative hypertension was noted in 23 (37.7%) patients. This includes 4 cases of malignant pheochromocytoma, 4 cases of recurrent benign pheochromocytoma and 15 cases of essential hypertension. Cardiovascular complications during follow-up were rare and concerned the patients with essential hypertension diagnosed postoperatively.     

Increased plasma atrial natriuretic factor in catecholamine-producing tumor patients.

The aim of this study was to evaluate plasma levels of ANF in patients with catecholamine-secreting tumors with and without hypertension and to relate ANF secretion to levels of plasma and urinary catecholamines and blood pressure. Twenty-one pheochromocytoma (15 with sustained, 6 with paroxysmal hypertension), 6 neuroblastoma (1 hypertensive) patients and 28 aged-matched controls were studied in basal conditions. Plasma and urinary norepinephrine (NE),epinephrine (E), dopamine (DA) and DOPA were determined by HPLC-ED and plasma ANF by RIA. Both neuroblastoma and pheochromocytoma patients had significantly higher plasma ANF levels than controls. Neuroblastomas showed higher ANF concentration than pheochromocytomas. No differences were found in plasma ANF between hypertensive and normotensive patients. Pheochromocytomas with ANF levels within the normal range had plasma and urinary NE and urinary DA and DOPA levels significantly higher than patients with high ANF. Plasma ANF levels were unrelated to systolic or diastolic blood pressure or heart rate. A negative correlation between plasma ANF and urinary DA was found only in the patients groups. In conclusion, plasma ANF was increased in pheochromocytoma and neuroblastoma patients. Our data suggest that the excessive catecholamine secretion is not responsible for the increased ANF secretion in these patients. The significance of the relationships among plasma ANF and urinary and plasma catecholamines requires further investigation.     

Increased Plasma Atrial Natriuretic Factor in Catecholamine-Producing Tumor Patients

The aim of this study was to evaluate plasma levels of ANF in patients with catecholamine-secreting tumors with and without hypertension and to relate ANF secretion to levels of plasma and urinary catecholamines and blood pressure. Twenty-one pheochromocytoma (15 with sustained, 6 with paroxysmal hypertension), 6 neuroblastoma (1 hypertensive) patients and 28 aged-matched controls were studied in basal conditions. Plasma and urinary norepinephrine (NE),epinephrine (E), dopamine (DA) and DOPA were determined by HPLC-ED and plasma ANF by RIA. Both neuroblastoma and pheochromocytoma patients had significantly higher plasma ANF levels than controls. Neuroblastomas showed higher ANF concentration than pheochromocytomas. No differences were found in plasma ANF between hypertensive and normotensive patients. Pheochromocytomas with ANF levels within the normal range had plasma and urinary NE and urinary DA and DOPA levels significantly higher than patients with     

Reversible cerebral ischemia in patients with pheochromocytoma

Cerebral ischemia and symptoms of stroke can occur as a rare manifestation in patients with pheochromocytoma. We describe a 45-year-old woman who was admitted because of a right-sided hemiparesis due to an ischemic lesion in the left hypothalamus. The clinical diagnosis of a pheochromocytoma was proven by highly elevated urinary catecholamines and confirmed histologically after operation. The successful removal of the tumor led to the almost complete recovery of the neurological deficiencies. It is of vital importance to know this atypical presentation of pheochromocytoma. The diagnosis of pheochromocytoma should be suspected in patients with focal cerebral symptoms, particularly in the presence of intermittent hypertension or other paroxysmal symptoms suggestive of pheochromocytoma.     

Differences in left ventricular structural and functional changes between pheochromocytoma and essential hypertension. Role of elevated circulating catecholamines.

Experimental findings suggest that catecholamines increase protein synthesis and play a role in cardiac hypertrophy. We hypothesize that elevated circulating plasma catecholamines in pheochromocytoma influence cardiac structural and functional remodeling. We compared 15 patients with surgically proven pheochromocytoma and 15 with untreated essential hypertension; we matched the patients for age, sex, body surface area, and blood pressure (BP) levels. Left ventricular hypertrophy (LVH) was identified by M-mode echocardiography in six patients with pheochromocytoma and in four with essential hypertension. Among both groups there were no differences in cardiac structure, no correlation between left ventricular mass and BP, no significant differences in mitral E-F slope, no correlation between either plasma norepinephrine or plasma epinephrine levels, and no differences in the left ventricular structural indices measured. In the pheochromocytoma group, left ventricular end systolic stress and end systolic diameter were significantly lower and left ventricular percent fractional shortening was higher. Plasma norepinephrine levels were higher in the pheochromocytoma group, but did not differ among patients of that group with and without LVH. We conclude that in both pheochromocytoma and essential hypertension, only a subset of patients develop evidence of LVH, and that in pheochromocytoma, the elevation of circulating plasma catecholamines is not necessarily associated with LVH. These results indicate that factors other than catecholamines and BP determine the development of LVH in pheochromocytoma.     

Chromogranin A storage and secretion: sensitivity and specificity for the diagnosis of pheochromocytoma

Chromogranin A, co-stored and co-released with catecholamines from adrenal medullary and sympathetic neuronal vesicles, is elevated in the plasma of patients with pheochromocytoma. The usefulness of the hormone in the differential diagnosis of hypertension is examined. An elevated level of chromogranin A had comparable diagnostic sensitivity (83%, 24/29) to, but greater diagnostic specificity (96%, 86/90) than the level of plasma catecholamines when subjects with pheochromocytoma (n = 29) were evaluated in comparison to several reference groups, including normotensive controls (n = 49), subjects with essential hypertension (n = 28), subjects with renovascular hypertension (n = 5), and subjects with primary aldosteronism (n = 3). Subjects with signs or symptoms suggesting pheochromocytoma, but in whom the diagnosis was ultimately ruled out (n = 5) had normal plasma levels of chromogranin A. A modest rise in chromogranin A in those with essential hypertension, and correlation of chromogranin A with diastolic blood pressure in normotensive patients and patients with essential hypertension did not impair the diagnostic usefulness of chromogranin A for pheochromocytoma. Renal failure was associated with an elevated plasma chromogranin A independently of blood pressure. Plasma chromogranin A correlated with tumor mass, tumor chromogranin A content, tumor norepinephrine content, and urinary vanillylmandelic acid excretion; it did not correlate with plasma or urinary catecholamines, nor with blood pressure in patients with pheochromocytoma. Plasma chromogranin A levels did not differ in subjects with pheochromocytoma when stratified by age, sex, tumor location, or tumor pathology. Several drugs used in the diagnosis or treatment of pheochromocytoma (clonidine, metoprolol, phentolamine, and tyramine) had little effect on plasma chromogranin A concentration. Within the pheochromocytoma, chromogranin A was localized along with catecholamines to the soluble core of chromaffin granules, where it accounted for 18 +/- 5% of vesicle soluble protein. We conclude that 1) chromogranin A emerges along with catecholamines from pheochromocytoma chromaffin granules; 2) plasma chromogranin A is a sensitive and specific diagnostic tool in evaluation of actual or suspected pheochromocytoma; 3) plasma chromogranin A predicts pheochromocytoma tumor size and overall catecholamine production; and 4) drugs commonly employed in the diagnosis or treatment of pheochromocytoma have little effect on plasma chromogranin A level, preserving the usefulness of chromogranin A in evaluating pheochromocytoma. Thus, measurement of chromogranin A provides a useful adjunct to the diagnosis of pheochromocytoma.     

Ectopic ACTH syndrome caused by pheochromocytoma: computed tomography-guided percutaneous ethanol injection as an alternative treatment

Ectopic ACTH secretion represents 8-18% of the cases of endogenous hypercortisolism. Pheochromocytomas correspond to 2-25% of the cases and surgery is the indicated treatment. We describe a case of ACTH-secreting pheochromocytoma treated with percutaneous ethanol injection (PEI) guided by computed tomography (CT). A 71-yr-old man presented with diabetes, severe hypokalemia, weight loss, muscle weakness, and hypertension. Hormonal evaluation revealed elevated levels of urinary cortisol, ACTH, catecholamines, and urinary metanephrines. There was no cortisol or ACTH response to desmopressin stimulation test. Magnetic resonance revealed bilateral adrenal nodules, larger on the left side. The suspected diagnosis was ectopic ACTH syndrome caused by pheochromocytoma. Ketoconazole treatment resulted in reduction of urinary cortisol levels but was followed by severe cholestasis and hepatic dysfunction, preventing surgery; it was substituted by octreotide with reduction of ACTH and cortisol levels, but without improvement of cholestasis. The patient presented cachexia and developed multiple pulmonary abscesses that also prevented surgical treatment, thus he was treated with percutaneous ethanol injection guided by CT of the left adrenal tumor. During the procedure, the patient had an increase in blood pressure controlled by the infusion of sodium nitroprusside followed by hypotension that required infusion of dopamine and volume expansion. Afterwards, he presented hormonal normalization, normal catecholamines levels, and clinical improvement. Histological tissue analysis confirmed pheochromocytoma. We concluded that CT-guided PEI represents an efficient alternative therapy to ectopic ACTH-secreting pheochromocytomas in patients without clinical conditions for surgery.