Survival characteristics of localized pediatric adrenocortical carcinoma managed with adenectomy: A national cancer center database analysis

Abstract

Pediatric adrenocortical carcinoma (ACC) is a rare malignancy, encompassing less than 0.2% of all childhood malignancies. Due to the scarcity of this diagnosis, it is often managed according to guidelines established for adults, as there is a lack of reliable evidence regarding optimal adjuvant treatment options for pediatric patients. It is our aim to identify recent treatment trends as well as clinical and tumor characteristics and their impact on overall survival. Using the National Cancer Data Base (NCDB), this study identified 49 patients under 18 years old with localized ACC (M0) undergoing adrenalectomy from 2004 to 2017. Kaplan-Meier analysis was performed to determine overall survival (OS) from patient characteristics and treatments received. Comparison of survival was performed using the log rank test. The median age of our cohort was 3 years old with a slight female predominance of 61%. The median tumor size was 9.4cm, and patients older than 4 years were significantly (p=0.03) more likely to present with larger tumors (11.33cm vs 8.76cm). Adjuvant treatment in the form of systemic therapy was administered in 20 of 49 (41%) patients and radiation therapy in 2 of 49 (4%) patients. Three-year OS for patients 4 years old and younger was 92.6% vs 61.8% for those older than 4 years (p=0.002). Patients presenting with tumor size ≥9cm had worse three-year OS compared to those with tumors <9cm (95.24% vs 67.1% respectively, p=0.02, Fig. 1). In patients with tumors≥9cm, younger children age 0-4 years had significantly (p=0.04) higher OS rates than older children age 5-17 years. ACC is a rare pediatric malignancy with a female predominance. Those older than 4 years and those with presenting tumor size ≥9cm have decreased overall survival rates after adrenalectomy for localized disease. Additionally, children older than 4 have poorer prognosis, even after controlling for larger tumor size. This is the largest contemporary series of localized pediatric ACC to date. However, multi-institutional prospective cohort or randomized-controlled trials are necessary to better evaluate relevant prognostic factors and the role of adjuvant therapies following adrenalectomy.