腹膜后巨大副神经节瘤2例报告并文献复习

摘要

Objective:To improve the experience in diagnosis and treatment of vast primary retroperitoneal paraganglioma (PGL). Methods:2 cases of vast primary retroperitoneat paraganglioma were analysised retrospectively. Including clinical feature,ra-diological characteristical,tumou markers,pathological feature,treatment and prognosis. And reviewed the ralative literature. Results:The 2 Cases were admitted with vast abdominal masses,no manufestations were showed except abdominal dull pain in one case,And were indefinited diagnosis before operation. Complete excision of the masses and metastasis lymph node was performed by open surgery. blood presure fluctuations happended in one case during operation(Maximum 220/126mmHg vs minimum 48/30mmHg). During fol-lowed-up of 35months、28months,No reccurence was founded. Conclusion:The primary retroperitoneal PGL is rare,It’s difficult to differ from other retroperitondal tumors. The locolization depended on B ultrosonography、CT、MRI、I-MIBG、PET. The measurement of plasma and urine catecholamines or metaslism may be valuable for diagnosis. Surgical intervention is the best way for patient. To definite the malignant PGL need followed-up.%目的：：探讨腹膜后副神经节瘤的临床特征及诊治方法。方法：回顾性分析2例腹膜后巨大副神经节瘤病例临床资料，结合相关文献复习探讨腹膜后副神经节瘤的临床表现、影像学特征、定性诊断、治疗方法、病理诊断及预后。结果：2例患者均以腹部包块就诊，除1例包块部位钝痛外，无其他临床症状，术前均未能定性诊断。行开放手术，完整切除腹膜后包块及肿大淋巴结。1例术中血压剧烈波动，术后需去甲肾上腺素维持正常血压。另1例术中、术后血压平稳。随访35个月、28个月，无复发。结论：腹膜后副神经节瘤发病率低，与腹膜后其它肿瘤术前鉴别诊断困难。 CT、MRI、功能影像学检查对其诊断有一定价值，血、尿儿茶酚胺及其代谢产物检查是定性诊断的重要手段。手术治疗是唯一有效而可靠的方法。病理检查对肿瘤良恶性诊断缺乏特异性标准。