16例儿童肾上腺皮质癌临床特点、治疗及预后的回顾性分析

Retrospective analysis of 16 childhood adrenocortical carcinoma in a single center

目的总结分析儿童肾上腺皮质癌患者的临床特点、治疗及预后。方法回顾性分析2013年12月—2020年12月于上海儿童医学中心确诊的初治肾上腺皮质癌患者的临床特点、治疗及预后情况。结果共收治初发肾上腺皮质癌患者16例。14例患者接受治疗后长期随访,2例患者分别于诊断后34个月和2个月死亡;5例患者出现肿瘤复发,复发中位时间为7个月,2例复发患者二线治疗无效死亡;7例无事件存活至今。14例随访患儿的2年EFS为73%,2年OS为77%。结论儿童肾上腺皮质癌罕见,预后较差;手术切除是主要治疗手段,部分患儿联合化疗、米托坦治疗可改善预后;但仍需寻找更为有效的治疗方案。

Objective To summarize and analyze the clinical characteristics,treatment and prognosis of childhood adrenocortical carcinoma.Methods Patients with adrenocortical carcinoma diagnosed at Shanghai Children′s Medical Center from December 2013 to December 2020 were retrospectively analyzed in terms of clinical characteristics,treatment,and prognosis.Results 16 patients with adrenocortical carcinoma were admitted.14 patients were followed up for a long time after treatment.2 patients died 34 months and 2 months after diagnosis,respectively.A total of 5 patients had tumor recurrence,with a median time of 7 months.Two patients died after second-line treatment.Seven cases have survived without event,and The two-year EFS was 73%,and the two-year OS was 77%.Conclusions Adrenocortical carcinoma in children is relatively rare and has a poor prognosis.Surgical resection is still the main treatment method.For some patients,surgery combined with chemotherapy and mitotane can improve the prognosis.But more cases and further studies are needed to find a more effective treatment.