Medical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences and Public Health, University of Brescia at ASST Spedali Civili, Brescia, Italy.
Endocrine. 2021 Dec;74(3):475-477. doi: 10.1007/s12020-021-02874-z. Epub 2021 Sep 24.
Pediatric and adult adrenocortical carcinomas differ in many respects but treatment is often similar in both age groups. The Journal of Clinical Oncology recently published the results of a risk-stratified single-arm interventional trial conducted by the Children's Oncology Group in which 77 patients were treated in three different interventional cohorts. In this Point of View paper we comment on the treatment strategies adopted within the ARAR0332 trial in terms of surgery approach, duration of adjuvant therapies, and palliative chemotherapy. We focus on the differences in the treatment of pediatric ACC patients compared to the ESE/ENSAT and ESMO guidelines released in 2018 for adult patients. For example, patients in stratum 3 and 4 received 8 (instead of 6) cycles of EDP chemotherapy but 8 months (instead of 24) of mitotane adjuvant therapy. Bearing clearly in the mind that pediatric and adult ACC patients represent different settings, we wonder whether there could be some areas of intervention overlapping to constitute a continuum of disease across ages. Thus, pediatric and adult cohoperative groups should be encouraged to collaborate in order to reach common guidelines for the treatment of such a rare disease.
儿科和成人肾上腺皮质癌在许多方面存在差异，但两种年龄组的治疗方法通常相似。《临床肿瘤学杂志》最近发表了儿童肿瘤学组进行的一项风险分层单臂干预性试验的结果，该试验中 77 名患者分为三个不同的干预队列进行治疗。在这篇观点文章中，我们根据手术方法、辅助治疗持续时间和姑息性化疗，对 ARAR0332 试验中采用的治疗策略进行了评论。我们重点关注与 2018 年发布的针对成人患者的 ESE/ENSAT 和 ESMO 指南相比，儿科 ACC 患者的治疗差异。例如，3 级和 4 级患者接受了 8（而不是 6）个周期的 EDP 化疗，但接受了 8 个月（而不是 24 个月）的米托坦辅助治疗。我们清楚地认识到，儿科和成人 ACC 患者代表了不同的情况，我们想知道是否有一些干预领域可能会重叠，从而构成跨越年龄的疾病连续体。因此，应鼓励儿科和成人合作组合作，制定治疗这种罕见疾病的共同指南。
