29例恶性嗜铬细胞瘤诊治分析

目的总结恶性嗜铬细胞瘤的诊治经验。方法回顾性分析2003年4月至2011年1月诊治的29例恶性嗜铬细胞瘤患者的临床资料。高血压26例（89．7％），血浆游离肾上腺素类物质（MNs）和24h尿儿茶酚胺（CA）升高者分别为92．3％（24／26）和86．2％（25／29）。B超、CT、MRI和^131I-间碘苄胍扫描（^131I-MIBG）定位准确率分别为86．2％（25／29）、96．6％（28／29）、88．9％（8／9）和81.2％（18／22），拟诊恶性者分别为24．1％（7／29）、55．2％（16／29）、44．4％（4／9）和40．9％（9／22）。1例行放射治疗，27例行开放手术，1例行后腹腔镜手术。结果29例患者位于肾上腺17例（58．6％），肾上腺外12例（41．4％）。首次手术根据肿瘤结构破坏、浸润生长及淋巴结转移确诊恶性13例（44．8％）。病理检查28例，确诊恶性23例（82．1％）。术后随访24例，时间4个月～8年，肿瘤复发11例，其中9例行2次或多次手术。肺转移4例，肝转移4例，骨转移3例，局部淋巴结转移4例，广泛淋巴结转移2例，2个或多个脏器同时发生转移3例。死亡4例：其余存活至今。结论病理难以区分嗜铬细胞瘤的良恶性，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恶性嗜铬细胞瘤最有效的方法，肿瘤复发时再次手术仍然有效。长期随访观察肿瘤的转移情况对最终确诊疑似病例和及时治疗尤为重要。

Diagnosis and treatment of malignant pheochromocytoma: report of 29 cases

Objective To review the experience on the diagnosis and treatment of malignant pheochromocytoma （MP）. Methods The clinical data of 29 cases of MP from April 2003 to Jan. 2011 were retrospectively analyzed. Hypertension was observed in 26 cases （89.7%）. The positive rate of plasma-free MNs and 24-hour urine CA was 92.3% （24/26） and 86.2% （25/29） respectively. The accuracy rate of localization of B-Ultrasound, CT, MRI and 1311-MIBG was 86.2% （25/29）, 96.6% （28/29）, 88.9% （8/9） and 81.2%（18/22）, and suspected malignancy was 24.1% （7/29）, 55.2% （16/29）, 44.4% （4/9） and 40. 9 % （9/22） respectively. One case underwent radiotherapy, 27 cases open surgery and 1 case retroperitoneal laparoscopic surgery. Results Of the 29 cases, 17 tumors were on the adrenal glands and 12 outside of the adrenal glands. 13 cases were diagnosed as MP intraoperatively according to tumor features, including structural damage, invasive growth, lymph node me- tastasis and so on. 23 cases were diagnosed as MP with pathological examination. 24 patients were followed up for 4 months to 8 years, during which 11 cases had tumor recurrences and 9 cases underwent re-operation. The metastatic sites of tumor included lung （4 cases）, liver （4 cases）, bone （3 cases）, local lymph node （4 cases）, extensive lymph node （2 cases）, and multiple organs （3 cases）. 4 cases died and the rest survived. Conclusions Pathological examination is difficult to distinguish benign and malignant pheochromocytoma, which should be combined with imaging examination, laboratory examination and intraoperative findings. Radical tumor resection is the preferred choice to prolong survival and re-operation is effective to recurrent cases. Long-term follow-up is significant for the ultimate diagnosis and timely treatment of suspected cases.