肾上腺外嗜铬细胞瘤的诊断与治疗
【摘要】 目的 探讨肾上腺外嗜铬细胞瘤的诊断和治疗方法。方法 对1991-2006年间诊治的10例肾上腺外嗜铬细胞瘤患者病例资料进行回顾分析，总结其临床特点。结果 均行手术治疗，切除肿瘤8例，包膜下剜除肿瘤2例。手术切除瘤体直径2~9 cm。随访7个月~10年，9例高血压患者术后血压正常8例、仍有高血压1例。肿瘤复发、转移3例，恶性嗜铬细胞瘤3例，死亡1例。结论 VMA及血、尿儿茶酚胺是定性诊断异位嗜铬细胞瘤的主要依据，CT诊断定位准确、131碘-间位碘代苄胍（131I-MIBG）定位准确、敏感性高，且可作为治疗措施。术前降压、扩容、纠正心律失常是手术成功的关键，手术治疗是本病的首选，术后应长期随访。
【Abstract】 Objective To study the diagnosis and management of extra-adrenal pheochromocytoma.Methods The clinical date of 10 cases of extra-adrenal pheochromocytoma were retrospectively studied.Results All cases had been treated with operation.There were 2 cases operated under epidural anesthesia and 8 general anesthesia.the specimens were from 2 to 9 cm in size．Among 9 cases with hypertension，the blood pressure of 8 patients return to normal and 1 cases were still hypertensive.3 cases were assayed as m alignant pheochromocytoma.There were 3 cases of recurrence or metastases and 1 patients died during followed-up from 7 months to 10 years.Conclusion Urine VMA level is a useful marker to detect extra-adrenal pheochromocytoma and CT scan is the most reliable in localizing the lesions．131-MIBG scintigraphy is of great value for the localiza-tion and quantitative for extrarenal pheochromocytoma with high sensitivity and accuracy．It may be also used as a therapeutic method．Preoperative,step down piesis,expand blood-volume,correct arrhythmia are the successful key points of the operation.Surgical operation is the best treatment option for extra-adrenal pheochromocytoma and the patients need to be followed up closely after surger.
【Key words】 Pheochromocytoma；Extrarenal;Diagosis;Treatment
肾上腺外嗜铬细胞瘤来源于肾上腺外嗜铬组织或副神经节系统，临床较少见，且诊断与治疗也较困难。本院1991-2006共收治经手术和病理证实的嗜铬细胞瘤50例，其中肾上腺外嗜铬细胞瘤10例。现报告如下。
1 资料与方法
1.1 临床资料 本组10例。男8例，女2例。年龄18~72岁，平均38岁；其中年龄＜20岁者2例，20~40岁者5例，＞40岁者3例。病程：10 d~14年，平均19个月。
1.2 临床表现 持续性高血压3例，阵发性高血压4例，持续性高血压阵发性发作2例，血压正常1例，1例以高血压危象入院。主要症状有心悸3例(30%)，
肾上腺外嗜铬细胞瘤的诊断与治疗
【摘要】 目的 探讨肾上腺外嗜铬细胞瘤的诊断和治疗方法。方法 对1991-2006年间诊治的10例肾上腺外嗜铬细胞瘤患者病例资料进行回顾分析，总结其临床特点。结果 均行手术治疗，切除肿瘤8例，包膜下剜除肿瘤2例。手术切除瘤体直径2~9 cm。随访7个月~10年，9例高血压患者术后血压正常8例、仍有高血压1例。肿瘤复发、转移3例，恶性嗜铬细胞瘤3例，死亡1例。结论 VMA及血、尿儿茶酚胺是定性诊断异位嗜铬细胞瘤的主要依据，CT诊断定位准确、131碘-间位碘代苄胍（131I-MIBG）定位准确、敏感性高，且可作为治疗措施。术前降压、扩容、纠正心律失常是手术成功的关键，手术治疗是本病的首选，术后应长期随访。
【Abstract】 Objective To study the diagnosis and management of extra-adrenal pheochromocytoma.Methods The clinical date of 10 cases of extra-adrenal pheochromocytoma were retrospectively studied.Results All cases had been treated with operation.There were 2 cases operated under epidural anesthesia and 8 general anesthesia.the specimens were from 2 to 9 cm in size．Among 9 cases with hypertension，the blood pressure of 8 patients return to normal and 1 cases were still hypertensive.3 cases were assayed as m alignant pheochromocytoma.There were 3 cases of recurrence or metastases and 1 patients died during followed-up from 7 months to 10 years.Conclusion Urine VMA level is a useful marker to detect extra-adrenal pheochromocytoma and CT scan is the most reliable in localizing the lesions．131-MIBG scintigraphy is of great value for the localiza-tion and quantitative for extrarenal pheochromocytoma with high sensitivity and accuracy．It may be also used as a therapeutic method．Preoperative,step down piesis,expand blood-volume,correct arrhythmia are the successful key points of the operation.Surgical operation is the best treatment option for extra-adrenal pheochromocytoma and the patients need to be followed up closely after surger.
【Key words】 Pheochromocytoma；Extrarenal;Diagosis;Treatment
肾上腺外嗜铬细胞瘤来源于肾上腺外嗜铬组织或副神经节系统，临床较少见，且诊断与治疗也较困难。本院1991-2006共收治经手术和病理证实的嗜铬细胞瘤50例，其中肾上腺外嗜铬细胞瘤10例。现报告如下。
1 资料与方法
1.1 临床资料 本组10例。男8例，女2例。年龄18~72岁，平均38岁；其中年龄＜20岁者2例，20~40岁者5例，＞40岁者3例。病程：10 d~14年，平均19个月。
1.2 临床表现 持续性高血压3例，阵发性高血压4例，持续性高血压阵发性发作2例，血压正常1例，1例以高血压危象入院。主要症状有心悸3例(30%)，