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目的:提高恶性副神经节瘤(MPGL)的诊治水平。方法:回顾性分析2003年4月～2011年1月诊治的12例MPGL患者的临床资料。高血压10例(83.3%),腰腹部疼痛3例(25.0%)。血浆游离变肾上腺素类物质(MNs)和24 h尿儿茶酚胺(CA)升高者分别为100.0%(12/12)和83.3%(10/12)。B超、CT、MRI和~(131)I-间碘苄胍扫描(~(131)I-MIBG)定位准确率分别为83.3%(10/12)、91.7%(11/12)、80.0%(4/5)和88.9%(8/9)。1例行放射治疗,11例行开放手术治疗。结果:原发性肿瘤9例,复发性肿瘤3例;单发性肿瘤8例,多发性肿瘤4例。根据肿瘤的中心定位:腹主动脉旁6例,下腔静脉后2例,膀胱2例,肾门2例。根据术中所见确诊恶性5例(41.7%)。病理检查11例,确诊恶性8例(72.7%)。肺转移1例,肝转移1例,骨转移1例,局部淋巴结转移3例,广泛淋巴结转移2例,2个或多个脏器同时发生转移2例。9例随访4～84个月,平均39个月;复发4例,再次手术;因多发浸润及远处转移死亡2例。结论:病理难以区分副神经节瘤的良恶性,需结合影像学检查、生化检查及术中所见。根治性肿瘤切除是治疗MPGL最有效的方法,肿瘤复发时再次手术仍然有效;无法手术者可用放化疗控制高血压及延缓疾病进展。长期随访观察肿瘤的转移情况是确诊疑似病例的重要方法。
Objective: To improve the diagnosis and treatment of malignant paraganglioma (MPGL). Methods: The clinical data of 12 patients with MPGL diagnosed and treated from April 2003 to January 2011 were retrospectively analyzed. High blood pressure in 10 cases (83.3%), waist and abdomen pain in 3 cases (25.0%). Plasma free adrenaline (MNs) and 24 h urinary catecholamine (CA) were increased by 100.0% (12/12) and 83.3% (10/12), respectively. The accuracy of localization of B-ultrasonography, CT, MRI and ~ (131) I-m-IABB was 83.3% (10/12), 91.7% (11/12) and 80.0% (4/5) and 88.9% (8/9). 1 routine radiotherapy, 11 cases underwent open surgery. Results: 9 cases of primary tumors and 3 cases of recurrent tumors; 8 cases of solitary tumors and 4 cases of multiple tumors. According to the tumor’s central location: 6 cases of abdominal aorta, 2 cases of inferior vena cava, 2 cases of bladder and 2 cases of renal portal. According to intraoperative findings confirmed malignant in 5 cases (41.7%). Pathological examination in 11 cases, diagnosed malignant in 8 cases (72.7%). 1 case of lung metastasis, 1 case of liver metastasis, 1 case of bone metastasis, 3 cases of local lymph node metastasis, 2 cases of extensive lymph node metastasis and 2 cases of simultaneous metastasis of 2 or more organs. Nine cases were followed up for 4 to 84 months with an average of 39 months. Four cases were relapsed and reoperation was performed. Two cases died of multiple infiltration and distant metastasis. Conclusion: It is difficult to distinguish the paragangliomas from benign and malignant pathologies. Imaging, biochemical examination and intraoperative findings should be combined. Radical tumor resection is the most effective method for the treatment of MPGL, re-operation of the tumor is still valid; those who can not operate radiotherapy and chemotherapy can be used to control high blood pressure and delay the progression of the disease. Long-term follow-up observation of tumor metastasis is an important method for the diagnosis of suspected cases.