CAJ | 학술논문

目的提高对多发性内分泌腺瘤病2A型(multiple 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2.7～7.0 cm,平均4.4 cm.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 ～ 240 min,平均181 min.术中出血量100～300 ml,平均171 ml.2例术中发生血压波动,最高180～210/100～110 mm Hg(1 mm Hg=0.133 kPa),最低70～90/40～45 mm Hg.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.
목적 제고대다발성내분비선류병2A형(multiple endocrine neoplasia type 2A,MEN2A)쌍측신상선기락세포류(adrenal pheochromocytoma,PHEO)림상특점적인식,탐토일기절제쌍측PHEO적안전성화가행성.방법 회고성분석2003년11월지2011년4월수치적8례MEN2A쌍측PHEO환자적림상자료,남6례,녀2례.년령35～56세,평균44세.4례반유고혈압증상,4례혈압무명현이상.수발증상이갑상선수양암(medullary thyroid carcinoma,MTC)취진4례,이고혈압취진1례,가계조사후발현3례,소유환자균선후리환MTC.혈인다분알정성진단양성4례.B초화CT검사균제시쌍측신상선점위병변:6례위쌍측다발,2례위일측단발、일측다발；증강CT검사가견종류강화,기중3례종류중앙유액화구；가견신、비혈관수압、변형；미발현국부림파결종대.종류최대경2.7～7.0 cm,평균4.4 cm.RET기인검측:8례균위RET기인적제11외현자C634Y돌변.전마복강경하2례행쌍측신상선전절술,4례행쌍측신상선부분절제혹종류절제술；2례행개방수술쌍측PHEO완제술.결과 8례수술균성공.수술공절제종류43매,수술시간150 ～ 240 min,평균181 min.술중출혈량100～300 ml,평균171 ml.2례술중발생혈압파동,최고180～210/100～110 mm Hg(1 mm Hg=0.133 kPa),최저70～90/40～45 mm Hg.병리진단균위쌍측신상선PHEO.술후1～3개월환자혈압균회복정상.수방3～90개월,평균35개월,종류무국부복발혹전이,단3례환자수용피질순체대치료.결론 MEN2A적쌍측PHEO행일기절제술시안전、가행적,가우선선택복강경하쌍측신상선부분절제술.
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 patie.nts,while in other 6 patients,PHEO were removed by partial adrenalectomy or adrenal tumorectomy with at least one normal appearing adrenal tissue preserved (4 by laparoscopic approach and 2 by open approach).Results The procedures were successful carried out on 8 cases with bilateral PHEO.There were totally 43 pieces of tumor obtained.The average time of operation was 181 min (range,150 to 240 min).Mean blood loss was 171ml (range,100 to 300 ml).Blood pressure fluctuations happened in 2 cases during operation (maximum:230/110 vs minimum:70/40 mm Hg).Histopathology showed PHEO in all patients.During the follow-up of 35 months (range,3 to 90),local and/or distant metastatic recurrence was not found in all cases,while two treated by total bilateral adrenalectomy and one by adrenal-sparing surgery required corticosteroid replacement.Conclusions SBA for bilateral PHEO in MEN2A is technically safe and feasible.Transperitoneal laparoscopic partial adrenalectomy for the treatment of bilateral PHEO can be considered as preferential choice.