恶性副神经节瘤的诊治探讨

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24h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131I-间碘苄胍扫描（131I-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。

The Diagnosis and Treatment of Malignant Paraganglioma

Objective：To improve the diagnosis and treatment of malignant paraganglioma（MPGL）. Methods： The clinical data of 12 cases of MPGL from April 2003 to January 2011 were retrospectively analyzed. Hyperten- sion was observed in 10 cases（83.3 %）and abdominal pain was in 3 cases（25.0 %）. The positive rate of plasma-free MNs and 24-hour urine CA in diagnosing MPGL was 100.0% （12/12）and 83.3%（10/12）respectively. The main localization diagnoses included B-Ultrasound, CT, MRI andTM I-MIBG, with the positive rates of 83.3 % （ 10/12 ）, 91.7 %（11 / 12）, 80.0 % （4/5） and 88.9 % （8/9） respectively. One case underwent radiotherapy, and the rest of 11 cases underwent open surgical treatment. Results： Nine cases were primary tumors and 3 recurrent tumors; Eight cases were single tumor and 4 multiple ones. The main anatomic locations of tumors were as follows：6 were adja- cent to the abdominal aorta,2 adjacent to the inferior vena cava,2 in the bladder,2 adjacent to the renal hilum. Five cases（41.7%）were diagnosed MPGL intraoperatively according to tumor features. Eleven cases underwent patho- logical examination and 8（72.7%）were diagnosed MPGL. The metastatic sites of tumor included 1 cases of lung, 1 cases of liver, 1 cases of bone, 3 cases of regional lymph node, 2 cases of extensive lymph node and 2 cases of multi- ple organs simultaneously. Nine patients were followed up for 4 to 84 months（mean, 39 months） , during which 4 o cases had tumor recurrence and underwent re-operation. Two cases died from tumor progression and metastasis and the remaining have been survival to date. Conclusions： As the biological behaviour of MPGL cannot be predicted with certainty from pathology, the diagnosis of malignancy is rather difficult. It is necessary to combine pathological examination with imaging examinations,laboratory inspection and intraoperative findings especially. To medically inoperable patients,radiotherapy and chemotherapy may be useful for controling hypertension and delaying disease progression. Long-term follow-up is significant for the ultimate diagnosis of suspected cases.