Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.
Chin Med J (Engl). 2009 Aug 5;122(15):1790-3.
Adrenal ganglioneuroma is a rare adrenal pathogenic disease with difficult differential diagnosis from adrenal pheochromocytoma. Currently, very limited literature is available to allow a differential diagnosis of these two conditions from each other. This study aimed to evaluate the clinical profile, differential diagnosis and surgical treatments of both conditions.
Clinical characteristics of 36 patients with adrenal pheocheomocytoma and 18 patients with adrenal ganglioneuroma were analyzed. Data from CT scans and surgical treatments from 1999 to 2007 were collected. Endocrine hormone tests and (131)I-metaiodobenzylguanidine (MIBG) were performed. Neither (131)I-MIBG nor endocrine hormone tests were available in 9 cases of asymptomatic adrenal ganglioneuroma with tumor size less than 4 cm and there were negative findings from contrast enhanced CT scans. The level of urine catecholamine of patients was compared by one-way analysis of variance.
Te mean age of patients in the adrenal ganglioneuroma group was 41.2 years (16 - 67 year) and in the adrenal pheochromocytoma patients 38 years (17 - 74 year). Contrast enhanced CT showed that the foci were intensified in 5 cases (27.8%) of adrenal ganglioneuroma and there were obvious contrast indications in 30 (83.3%) of the pheochromocytoma. Catecholamine levels in a 24-hour urine sample were normal in 4 patients with adrenal ganglioneuroma and increased in 36 (100%) cases with adrenal pheochromocytoma. (131)I-MIBG nuclear scan showed negative results in 4 patients (100%) with adrenal ganglioneuroma and positive results in 25 (96.2%) with adrenal pheochromocytom. Laparoscopy for adrenal tumors was performed through a transperitoneal or retroperitoneal approach during a follow-up period of (43 +/- 6) months, and all cases survived well.
CT, urinary catecholamine and (131)I-MIBG are standard and efficient tools for differential diagnosis of adrenal ganglioneuroma from pheochromocytoma. Laparoscopic surgery can be performed through a transperitoneal or retroperitoneal approach depending on the finding of CT scans. Open surgery is necessary for patients with blood loss of more than 800 ml and violent fluctuation of intraoperative blood pressure.
神经节瘤是一种罕见的肾上腺疾病，与嗜铬细胞瘤的鉴别诊断困难。目前，关于这两种疾病相互鉴别的文献非常有限。本研究旨在评估这两种疾病的临床特征、鉴别诊断和外科治疗。
分析 1999 年至 2007 年期间 36 例嗜铬细胞瘤和 18 例神经节瘤患者的临床特征。收集 CT 扫描和手术治疗数据。进行内分泌激素检测和（131）I-间碘苄胍（MIBG）检查。9 例无症状、肿瘤直径小于 4cm 的神经节瘤患者未进行（131）I-MIBG 和内分泌激素检查，增强 CT 扫描未见异常。采用单因素方差分析比较患者尿儿茶酚胺水平。
神经节瘤组患者的平均年龄为 41.2 岁（16-67 岁），嗜铬细胞瘤组患者的平均年龄为 38 岁（17-74 岁）。增强 CT 显示神经节瘤 5 例（27.8%）病灶强化，嗜铬细胞瘤 30 例（83.3%）有明显对比指征。神经节瘤组 4 例患者 24 小时尿儿茶酚胺正常，36 例（100%）嗜铬细胞瘤患者升高。（131）I-MIBG 核扫描显示神经节瘤组 4 例（100%）阴性，嗜铬细胞瘤组 25 例（96.2%）阳性。在 43±6 个月的随访期间，通过经腹腔或腹膜后途径对肾上腺肿瘤行腹腔镜手术，所有患者均存活。
CT、尿儿茶酚胺和（131）I-MIBG 是鉴别诊断神经节瘤和嗜铬细胞瘤的标准、有效工具。根据 CT 扫描结果可选择经腹腔或腹膜后途径行腹腔镜手术。对于术中出血量超过 800ml、术中血压剧烈波动的患者，需要开腹手术。
