多发性内分泌腺瘤病2A型双侧肾上腺嗜铬细胞瘤一期切除术的安全性和疗效分析  

Safety and curative effect analysis of bilateral simultaneous adrenalectomy for the treatment of the bilateral adrenal pheochromocytoma associated with multiple endocrine neoplasia type 2A

作　　者：戚晓平[1] 王靖宇[2] 金杭阳[1] 杜振方[3] 陈振光[1] 李峰[1] 王金泉[4] 吕文艳[2] 张咸宁[3] 

机构地区：[1]解放军第一一七医院泌尿外科,杭州310004 [2]解放军第一一七医院麻醉科,杭州310004 [3]浙江大学医学院生化和遗传学系 [4]解放军第一一七医院病理科,杭州310004

出　　处：《中华泌尿外科杂志》2013年第1期5-9,共5页Chinese Journal of Urology

基　　金：南京军区医学科技创新课题基金重点资助项目（092038）;（10Z036）

摘　　要：目的提高对多发性内分泌腺瘤病2A型（muhiple endocrine neoplasia type 2A，MEN2A）双侧肾上腺嗜铬细胞瘤（adrenal pheochromocytoma，PHEO）临床特点的认识，探讨一期切除双侧PHEO的安全性和可行性。方法回顾性分析2003年11月至2011年4月收治的8例MEN2A双侧PHEO患者的临床资料，男6例，女2例。年龄35—56岁，平均44岁。4例伴有高血压症状，4例血压无明显异常。首发症状以甲状腺髓样癌（medullary thyroid carcinoma，MTC）就诊4例，以高血压就诊1例，家系调查后发现3例，所有患者均先后罹患MTC。血儿茶酚胺定性诊断阳性4例。B超和CT检查均提示双侧肾上腺占位病变：6例为双侧多发，2例为一侧单发、一侧多发；增强CT检查可见肿瘤强化，其中3例肿瘤中央有液化区；可见肾、脾血管受压、变形；未发现局部淋巴结肿大。肿瘤最大径2．7～7．0cm，平均4．4cm。RET基因检测：8例均为RET基因的第11外显子C634Y突变。全麻腹腔镜下2例行双侧肾上腺全切术，4例行双侧肾上腺部分切除或肿瘤切除术；2例行开放手术双侧PHEO剜除术。结果8例手术均成功。手术共切除肿瘤43枚，手术时间150～240min，平均181min。术中出血量100—300ml，平均171ml。2例术中发生血压波动，最高180～210／100～110mmHg（1inmHg=0．133kPa），最低70～90／40～45mmHg。病理诊断均为双侧肾上腺PHEO。术后1—3个月患者血压均恢复正常。随访3—90个月，平均35个月，肿瘤无局部复发或转移，但3例患者需用皮质醇替代治疗。结论MEN2A的双侧PHEO行一期切除术是安全、可行的，可优先选择腹腔镜下双侧肾上腺部分切除术。Objective To study the clinical features of the bilateral adrenal pheochromocytomas （PHEO） in patients associated with multiple endocrine neoplasia type 2A （MEN2A） and to evaluate the safety and feasibility of applying simultaneous bilateral adrenalectomy （ SBA）. Methods From November 2003 to April 2011, clinical data of 8 patients from 4 unrelated MEN2A families treated with SBA were analyzed. There were 6 males and 2 females. The mean age was 44 years （range, 35 to 56 years）. 4 patients were asymptomatic and 4 had clinical features related to PHEO. The diagnosis of bilateral PHEO was made after the diagnosis of MTC in 4 cases, previous hypertension in 1 case, and family investigation found 3 MTC and all were found with hyperparathyroidism. Plasma catecholamines were substantially elevated in 4 patients. Ultrasound and computerized tomography （CT） examination showed bilateral adrenal masses, of which 6 were bilateral and multiple tumors, 2 were single tumor in one side and multiple tumors in the other side. On contrast CT, the tumors displayed heterogeneous contrast enhancement and low density area in the center of the tumors observed in 3 cases ;renal, splenic vessels displacement and deformation were observed ; local lymph node enlargement was not found. The mean maximum diameter of PHEO was 4.4 cm （ range, 2.7 to 7.0 cm）. RET screening showed a heterozygous nucleotide substitution on exon 11, c. G1901A （ p. C634Y） in all patients. After adequate adrenergic blockade, the SBA was performed by a single surgeon, PHEO were extirpated by laparoscopic radical adrenalectomy in 2 patients, while in other 6 patients, PHEO were removed by partial adrenalectomy or adrenal tumorectomy with at least one normal appearing adrenal tis- sue preserved （4 by laparoscopic approach and 2 by open approach）. Results The procedures were suc- cessful carried out on 8 cases with bilateral PHEO. There were totally 43 pieces of tumor obtained. The aver- age time of operation was 181 min （range, 150 to 24

关 键 词：多发性内分泌腺瘤2A型 嗜铬细胞瘤 双侧肾上腺切除 RET基因 腹腔镜 

分 类 号：R736.6[医药卫生—肿瘤]