Abstract
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.
Highlights
Adrenocortical carcinoma (ACC) is an endocrine neoplasm arising in the outer part of the adrenal gland
Pediatric ACC is even rarer with a reported incidence of 0.2–0.3 cases per million people/year worldwide [3,4,5,6] except for the cluster associated with the TP53R337H pathogenetic variant identified in Southern Brazil that is 15 times more frequent than non-Brazilian ACC cases [7]
Pediatric ACC has been less studied than the adult counterpart and most of the knowledge derives from studies conducted in the South Brazilian population
Summary
Adrenocortical carcinoma (ACC) is an endocrine neoplasm arising in the outer part of the adrenal gland. Adult ACC is a rare cancer with a reported incidence of 0.7–2 cases per million people/year worldwide [2]. The authors reported the results of clinical management of 77 eligible patients with stage I–IV ACC treated in three different interventional cohorts within the Children’s Oncology Group ARAR0332 trial.
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
