肾上腺区神经节细胞瘤(附18例报告)

Ganglioneuromas in the adrenal area (report of 18 cases)

目的较全面的了解神经节细胞瘤的临床特点及治疗方法.方法回顾性分析18例病理确诊的神经节细胞瘤患者临床表现、实验室检查、影像学检查和治疗效果.结果 15例是无临床症状的影像学偶然发现病例,内分泌检查均在正常范围;另外3例以阵发性高血压为主要临床表现,其中1例尿儿茶酚胺升高,肾上腺髄质显像(MIBG)阳性.18例B型超声检查均为低回声;13例CT平扫见散在点状高密度影,12例CT增强扫描肿瘤周边强化明显.手术前诊断为嗜铬细胞瘤12例,神经节细胞瘤4例,肾上腺无功能腺瘤2例.18例行肿瘤切除,随访6个月～7年,高血压症状消失,2例肿瘤复发.结论肾上腺区神经节细胞瘤与肾上腺髄质肿瘤难以鉴别,综合各项影像学检查对诊断有较高的参考价值.手术治疗是唯一的有效方法.

Objective To study the characteristics of prevalence and clinical manifestation and management principles of ganglioneuromas. Methods The clinical manifestation, laboratory and imaging examination and therapy of 18 cases of ganglioneuromas diagnosed by pathology were retrospectively analyzed. Results 15 cases of asymptomatic ganglioneuromas were incidentally discovered by imaging, while the endocrine examination showed negative results. 3 of 18 mainly presented paroxysmally hypertension, one of them had a high level of urinary catecholamine, and meta-iodobenzylguanidine （MIBG） scan showed positive results. The B-ultrasonic showed low-echo in all cases. With unenhanced CT, 13 cases showed punctuate calcification. 12 of the 18 cases undertook the enhanced CT, and the results showed distinct enhancement of the periphery of tumors. Before operation, 12 of the 18 cases were diagnosed as pheochromocytoma, 4 ganglioneuromas, 2 adrenal nonfunctional tumor. Tumor excision was performed in all patients. Follow-up ranged from 6 to 84 months. None had hypentension symptoms. The tumors recurredin two cases. Gonclusion It is difficult to differentiate adrenal medullary tumor and ganglioneuromas. The combination of imaging data is helpful in the diagnosis of ganglioneuromas, the key to cure is complete surgical excision.