目的总结腹膜后副神经节瘤的临床特征。方法回顾性分析2005年1月至2015年1月期间华西医院血管外科收治的经病理学检查诊断为副神经节瘤的34例患者的临床资料。结果 34例患者的临床表现:5例功能性肿瘤患者以高血压(4例)或头晕(1例)为首发症状;29例无功能性肿瘤患者中,以腹胀(14例)或腹痛(11例)为首发症状者25例,1例表现为消化道梗阻症状,其余3例无症状、意外发现肿瘤。5例功能性肿瘤患者的血液及尿液中儿茶酚胺水平均不同程度增高,2例无功能性肿瘤患者血液中儿茶酚胺水平轻微异常,其余患者的血、尿儿茶酚胺水平均在正常范围内。34例患者均接受手术治疗,其中4例无功能性肿瘤患者行重要血管切除或重建,1例出现血管壁受侵;30例未行血管切除或重建的患者中,2例有脉管侵犯。肿瘤直径3.5~18.5 cm、(9.5±5.1)cm;24例肿瘤有完整包膜,10例肿瘤有部分包膜;分化良好者32例,2例组织呈现细胞核异型性;G1级14例,G2级20例。术后34例患者均获访,随访时间为1~105个月,平均52个月。随访期间复发6例,死亡6例。获访患者的1年和5年累积生存率分别为86.0%和81.5%。结论目前根治性切除可能是治愈腹膜后副神经节瘤的唯一手段,肿瘤联合重要血管切除或重建为既往认为“不可切除的肿瘤”提供了切除机会。 Objective To summarize the clinical features of retroperitoneal paraganglioma. Methods The clinical data of 34 patients diagnosed as paraganglioma by pathology were retrospectively analyzed from January 2005 to January 2015 in West China Hospital. Results The clinical manifestations of 34 patients were as follows: 5 patients with functional tumor were the first symptom of hypertension (4 cases) or dizziness (1 case). Among the 29 patients with non-functioning tumor, abdominal distension (14 cases) or abdominal pain Cases) for the first symptom in 25 cases, 1 case of symptoms of gastrointestinal obstruction, the remaining three cases asymptomatic, unexpectedly found the tumor. The levels of catecholamines in blood and urine of 5 patients with functional tumor increased to different extents. The levels of catecholamine in blood of 2 patients with functional tumor were slightly abnormal. The blood and urinary catecholamine levels of other patients were within the normal range. Thirty-four patients underwent surgical treatment. Of the four patients with non-functioning tumors, major vascular resection or reconstruction was performed, and one of the three patients had vascular wall invasion. Of the 30 patients who underwent revascularization or reconstruction, two had vascular invasion. Tumor diameter was 3.5 ~ 18.5 cm and (9.5 ± 5.1) cm respectively. Twenty-four of the tumors had a complete envelope and 10 of the tumors had a partial envelope. 32 well-differentiated tumors showed nuclear atypia in 2 cases, G1 grade 14, Grade 20 cases. All 34 patients were followed up for 1 ~ 105 months with an average of 52 months. 6 cases were recurred during follow-up and 6 died. The 1-year and 5-year cumulative survival rates of the patients visited were 86.0% and 81.5%, respectively. Conclusions At present, radical resection may be the only way to cure retroperitoneal ganglioma, and the combined tumor resection or reconstruction of important tumor provides the opportunity of resection as “unresectable tumor”.