CAJ | 학술논문

目的：探讨肾上腺皮质腺癌（ ACC）的诊断与治疗。方法回顾性分析39例ACC患者（ ACC组）诊断与治疗的临床资料，并随机调取同期39例肾上腺腺瘤（ ACA）患者（ ACA组）临床资料作对照。结果 ACC组肿瘤最大径（11.66±4.21）cm；Weiss评分：3～5分13例，＞5分26例。 ACA组肿瘤最大径（2.34±1.27）cm；Weiss评分：＜3分37例，3～5分2例，＞5分0例。 ACC组34例行肿瘤完整切除，5例仅行姑息性切除或活检术，术后1周内死亡2例；口服舒尼替尼治疗2例；随访32例，随访时间1～80个月，中位生存时间12个月，1年与5年生存率分别为45.7％和32.7％。 ACA组手术均顺利，术后随访33例，失访6例，随访时间2～176个月，均健在。结论 ACC分期晚，预后差，与ACA鉴别较困难，肿瘤大小应作为良恶性鉴别指标之一，根治性切除术后易复发转移，可尝试舒尼替尼分子靶向药物治疗。
목적：탐토신상선피질선암（ ACC）적진단여치료。방법회고성분석39례ACC환자（ ACC조）진단여치료적림상자료，병수궤조취동기39례신상선선류（ ACA）환자（ ACA조）림상자료작대조。결과 ACC조종류최대경（11.66±4.21）cm；Weiss평분：3～5분13례，＞5분26례。 ACA조종류최대경（2.34±1.27）cm；Weiss평분：＜3분37례，3～5분2례，＞5분0례。 ACC조34례행종류완정절제，5례부행고식성절제혹활검술，술후1주내사망2례；구복서니체니치료2례；수방32례，수방시간1～80개월，중위생존시간12개월，1년여5년생존솔분별위45.7％화32.7％。 ACA조수술균순리，술후수방33례，실방6례，수방시간2～176개월，균건재。결론 ACC분기만，예후차，여ACA감별교곤난，종류대소응작위량악성감별지표지일，근치성절제술후역복발전이，가상시서니체니분자파향약물치료。
Objective To investigate the clinicopathological characteristics and treatment of patients with adreno-cortical carcinoma ( ACC) .Methods Thirty-nine patients diagnosed with ACC between January 1999 and march 2014 were evaluated, with 39 time-matched adrenocortical adenoma ( ACA) were randomly drawn as control group.The clini-cal features and outcomes were reviewed.Results In ACC group, median age was 51.72 years (19 to 76 years) , among whom Weiss score was between 3 and 5 in 13 cases and over 5 in 26 case, with the tumor diamer of(11.66 ±4.21)cm. In ACA group, Weiss score was lower than 3 in 27 cases and between 3 and 5 in 2 cases, with the tumor diamer of(2.34 ± 1.27)cm.Endocrinolgical evaluation was performed in all patients.En bloc resetion was performed in 34 cases in ACC group, and palliative resection or biopsy was performed in 5 patients whom detected metastases at initial diagnosis.Two deaths were reported during the 1st week after surgery.Sorafenib was given to 2 cases in ACC group.With a follow-up of 1~80 months, the median survival time was 12 months, with the 1 -year and 5 -year survival rates of 45.7% and 32.7%, respectively.All patients in ACA group underwent operation successfully with satisfactorily postoperative rehabili-tation.Thirty-three cases were followed up 2 to 176 months with no death.Conclusion ACC is a rare disease with a poor prognosis.Tumor sizes could be used as one of a valuable indicator for distinguishing.Patients with positive VEGF in tumor tissue could try Sunitinib for molecular targeted drug therapy.