【摘要】 目的提高肾上腺外嗜铬细胞瘤的诊断和治疗水平。方法分析1986年至2005年资料完整、经病理证实的肾上腺外嗜铬细胞瘤48例。其中高血压43例、血尿6例,体检发现肿瘤3例。48例患者中尿VMA及血、尿儿茶酚胺升高分别为44例(91%)和40例(83%)。所有患者经B超检查,阳性率为92%、经CT扫描阳性率为100%。48例患者均行手术治疗。结果切除肿瘤40例,包膜下剜除肿瘤8例。手术切除瘤体直径2-9cm。随访6个月-19年,43例高血压患者术后血压正常31例、仍有高血压2例。肿瘤复发、转移6例,恶性嗜铬细胞瘤12例,死亡7例。结论尿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,经腹径路手术暴露良好,肿瘤外侵时可作囊内剜除,术后应密切随访。更多还原

【Abstract】 Objective To highlight the diagnostic and therapeutic procedures for extra-adrenal pheochromocytoma.Methods 48 cases of extra-adrenal pheochromocytoma proven pathologically from 1986 to 2005 were reviewed and studied.Hypertension was observed in 43 cases and intermittent macrohematuria in 6.3 cases were admitted to hospital because of abdomen masses detected through routine physical examination.The level of urine VMA elevated in 44 cases(91%)and serum or urinary catecholamine elevated in 40(83%).All cases had B-model ultrasound examination and CT scan.The positive rates of localization of B-ultrasound and CT were 92% and 100%,respectively.All cases had been treated with operation.Results Among the 40 cases with the tumor incised,the specimens were 2 to 9 cm in size.Among 40 cases with hypertension,the blood pressure of 31 patients returned to normal and 2 cases were still hypertensive.12 cases were assayed as malignant pheochromocytoma.There were 6 cases of recurrence or metastases and 7 patients died during 6 months to 19 years of followed-up.Conclusion Urine VMA level is a useful marker to detect extra-adrenal pheochromocytoma and CT scan is the most reliable in localizing the lesions.131I-MIBG scintigraphy is of great value for the localization and quantitative for extra-renal pheochromocytoma with high sensitivity and accuracy.It may also be used as a therapeutic method.Surgical manipulation of the tumor is the best management and meticulous perioperative management is extremely important.Measuring the level of urinary catecholamine can monitor the recurrence or metastasis of the tumor.更多还原