目的:探讨肾上腺嗜铬细胞瘤的诊治方法。方法:回顾性分析我院1995年10月～2008年10月收治37例肾上腺嗜铬细胞瘤的临床资料,总结其主要临床表现、手术方法及围手术期血压的控制方法。结果:37例患者均行手术治疗,其中35例术后血压恢复正常,2例术后血压未降至正常水平。结论:肾上腺嗜铬细胞瘤的诊断主要依靠多种内分泌激素的测定及B超、CT的定位检查,确诊依赖于病理检查,手术切除是有效的治疗方法。术前的降压、扩容和术后的补液升压等围手术期的处理是手术治疗成功的关键。
Objective To report the experiences using orthotopic heart transplantation （HTX） to treat a patient with primary cardiac pheochromocytoma （PCT）. Methods On June 2, 2005, a 48-year-old woman received orthotopic HTX because she suffered from cardiac PCT which can not be resected. The procedure for the recipient was uneventful. The aortic crossclamp time was 95 min, assist circulation time 64 min. Results Twenty days after the operation, the patient was discharged without any events, and serum norepinepherine dropped to 1. 339 ng/ml, and urinary norepinepherine 108μg/24h. Conclusion Orthotopic HTX is an effective treatment for cardiac PCT when it cannot be resected.
Objective To retrospectively analyze the clinical information of a series of patients with atypical adrenal phaeochromocytoma in order to improve the diagnosis of atypical phaeochromocytoma. Methods Ninety patients diagnosed pathologically as adrenal phaeochromocytoma from January 1998 to December 2004 in the Affiliated Hospital of Inner Mongolia Medical College were included in the study. Patients with phaeochromocytoma were classified into the typical group and atypical group based on their clinical manifestations. The differences were analyzed between the two groups in terms of patient age, tumor location and maximal diameter, incidence of hypertension, and plasma catecholamine level. Results About 24.4% (22/90) of the patients with phaeochromocytoma were classified as atypical. There was no significant difference in the maximal tumor diameter between the two groups. The plasma norepinephrine level of the atypical group was significantly lower than that of the typical group (P=0.001), and the positive rate of plasma norepinephrine of the atypical group was also lower than that of the typical group (P=0.003). Conclusion Compared with typical phaeochromocytoma, atypical phaeochromocytoma is associated with a lower plasma norepinephrine level. The size of adrenal tumor cannot contribute to the diagnosis of atypical phaeochromocytoma. Based on atypical manifestations, CT examination results, and plasma catecholamine level, most atypical phaeochromocytoma should be diagnosed correctly.
Catecholamine-induced cardiomyopathy in pheochromocytoma/paraganglioma (PPGL) is a potential fatal cardiovascular complication caused by excessive secretion of catecholamines by PPGL, leading to structural changes and functional abnormalities in the heart. According to the morphology and function of the heart, it is clinically divided into three types: dilated cardiomyopathy, Takotsubo cardiomyopathy, and hypertrophic cardiomyopathy. The treatment of catecholamine-induced cardiomyopathy in PPGL requires attention to drug selection, application of life support equipment, and perioperative management. Most patients with cardiac dysfunction can effectively improve after tumor resection. This article mainly reviews the diagnosis and treatment of catecholamine-induced cardiomyopathy in PPGL.