肾上腺皮质腺癌39例诊断与治疗体会

The diagnosis and treatment of adrenocortical carcinoma: report of 39 cases

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。

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.