原发性腹膜后副神经节瘤14例报告并文献复习

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手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。

Clinical Analysis of Primary Retroperitoneal Paraganglioma: Report of 14 Cases and Review of the Literature

Objective:To evaluate the clinical and pathological character,diagnosis,treatment and prognosis of primary retroperitoneal paraganglioma.Methods:Tumor location,clinical manifestation,auxiliary examination and surgical treatment of 14 cases who were diagnosed as retroperitoneal paraganglioma by pathology were retrospectively analyzed.There were 8 males and 6 females with an age range of 14 to 66 years.9 patients had symptom of hypertension,with 8 cases of elevated urine vanilmandelic acid and 9 cases of elevated urine catecholamine.All patients received operation.Follow-up after surgery were done from 13 months to 5 years.Literatures in the latest 50 years were also reviewed.Results:Tumor of 4 cases located in renal hilum,5 in upper pole of kidney,2 in inferior pole of kidney,and 3 in anterior-lateral to the adrenal gland.The tumor of 13 cases were totally resected,and one with liver metastasis received subtotal tumor and caudate lobe resection.Besides one case of malignancy,the pathologic diagnoses of other 13 cases were innocent.By follow-up to 12 patients.we found that the blood pressure of 7 patients among 9 cases with hypertension returned to normal.9 cases survived without tumor,1 case recurred and 2 died of metastasis.Five-year survival rate after operation was 83.5%according to literature analysis.Conclusion: Surgical resection might be the most effective method for treatment of retroperitoneal paraganglioma.Sufficient preoperative preparation and perioperative management might be the key point of successful surgery.Recurrent tumor could be resected again.Survival was determined by lymph node status or the presence of metastasis.