肾上腺节细胞神经瘤与嗜铬细胞瘤的临床鉴别诊断及治疗

The diagnosis and treatment of adrenal pheochromocytoma and adrenal ganglioneucoma

目的探讨肾上腺节细胞神经瘤与嗜铬细胞瘤的临床特点,诊断、鉴别诊断及治疗。方法回顾性分析1999—2005北京协和医院14例节细胞神经瘤及32例嗜铬细胞瘤患者的临床资料。节细胞神经瘤组男8例,女6例;年龄16～63岁;肿瘤直径2·5～15cm。嗜铬细胞瘤组男14例,女18例;年龄19～74岁;肿瘤直径2～16cm。全部患者均行手术治疗切除肿瘤并经病理证实。结果节细胞神经瘤患者多无临床症状,查体时偶然发现肾上腺区占位;CT多表现为形态规则的圆形或类圆形低密度影,边界光滑,质地均一,增强扫描强化不明显;24h尿儿茶酚胺在正常水平;131I-MIBG检测大部分阴性。嗜铬细胞瘤患者就诊时多有典型的阵发或持续性高血压症状;CT表现多为形态欠规则的实性、囊实性或单纯囊性占位,密度不均,增强后呈不规则明显强化;嗜铬细胞瘤内分泌功能活跃,24h尿儿茶酚胺检查显著升高;131I-MIBG检测大部分呈阳性,浓聚区域与占位病变位置相同。结论肾上腺节细胞神经瘤与嗜铬细胞瘤在临床表现、影像学、内分泌检查和131I-MIBG等均有显著差异。通过仔细询问病史,正确的实验室及辅助检查,术前基本能鉴别诊断。行开放或腹腔镜手术切除可取得良好的治疗效果。

Objective To evaluate the clinical characteristics, diagnosis and treatment procedures for adrenal pheochromocytoma and adrenal ganglioneucoma. Methods Thirty-two cases of adrenal pheochromocytoma and 14 cases of adrenal ganglioneucoma surgical treated between 1999-2005 were analysed, The average of ganglioneucoma was 39 years old （ 16 - 63）. tumor size ranged from 2. 5 cm to 15 cm. The mean age of pheochromocytoma was 42 years old （ 19 - 74）, tumor size ranged from 2cm to 16 cm. Tumor located in the adrenal area. All cases accepted surgical operation and were confirmed by pathology. Results Most cases of adrenal ganglioneucoma did not have any symptom but were founded incidentally. Differentials were obviously between ganglioneucoma and pheochromocytoma. Conclusion Both adrenal ganglioneucoma and pheochromocytoma are rare diseases. CT, urinary CA and ^131 I-MIBG are good methods for diagnosis. The final diagnosis depends on the pathology, surgical resection of tumor is a preferred choice.