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目的:探讨原发性腹膜后副神经节瘤的临床和病理特点、诊治方法及预后。方法:回顾性分析14例病理检查证实为腹膜后副神经节瘤患者的临床资料:男8例,女6例,年龄14～66岁。有高血压症状9例,其中尿香草扁桃酸(VMA)升高8例,尿儿茶酚胺升高9例。14例均接受手术治疗。术后随访13个月～5年。结果:术中见肿瘤位于肾门区4例,肾上极区5例,肾下极区2例,肾上腺前外上方3例。13例手术完全切除肿瘤,1例伴肝转移者行肿瘤大部切除及肝尾叶切除。病理检查诊断为良性肿瘤13例,恶性肿瘤1例。术后随访12例,9例术前高血压者有7例血压恢复正常;9例无瘤生存,1例复发,2例死亡。结论:手术完全切除肿瘤是腹膜后副神经节瘤最有效的治疗方法,充分的术前准备和围于术期处理是手术成功的关键。术后生存时间取决于肿瘤是否发生淋巴结或远处转移,复发病例可再次手术切除。
Objective: To investigate the clinical and pathological characteristics of primary retroperitoneal paraganglioma, diagnosis and treatment methods and prognosis. Methods: The clinical data of 14 patients with retroperitoneal ganglioma confirmed by pathological examination were retrospectively analyzed. There were 8 males and 6 females, aged from 14 to 66 years. There were 9 cases of hypertension, including 8 cases of urinary vanilloid (VMA) increased, 9 cases of urinary catecholamines increased. All 14 patients underwent surgery. Follow-up 13 months to 5 years. Results: In operation, the tumor was found in 4 cases of nephrotic region, 5 cases of suprapatellar region, 2 cases of hypodermic pole and 3 cases of anterior superior adrenal gland. Thirteen patients underwent complete resection of the tumor. One patient with liver metastasis underwent resection of the tumor and resection of the caudate lobe. Pathological examination diagnosed as benign tumors in 13 cases, 1 case of malignant tumors. Postoperative follow-up of 12 cases, 9 cases of preoperative hypertension were 7 cases of blood pressure returned to normal; 9 cases of tumor-free survival, 1 case of recurrence, 2 patients died. Conclusions: Surgical resection of the tumor is the most effective treatment for retroperitoneal ganglioma. Adequate preoperative preparation and perioperative management are the keys to successful operation. Survival time depends on whether the tumor lymph node or distant metastasis, recurrence of cases can be re-operated resection.