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Survival Characteristics of Localized Pediatric Adrenocortical Carcinoma Managed with Adenectomy: A National Cancer Center Database Analysis
Jaisa D. Evanoff, BS1, Sanjay G. Patel, MD2, Kyle J. Hickey, BS1, Adam J. Rensing, MD2.
1University of Oklahoma College of Medicine, Oklahoma City, OK, USA, 2University of Oklahoma Department of Urology, Oklahoma City, OK, USA.

Background: 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 and their impact on overall survival. Methods: Using the National Cancer Data Base (NCDB), we identified 49 patients under 18 years old with localized ACC (M0) undergoing adrenalectomy from 2004-2017. 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. Results: The median age of our cohort was 3 years old with a slight female predominance of 61%. The median tumor size was 9.4 cm, and patients age 5 and older were significantly (p=0.03) more likely to present with larger tumors (11.33 cm vs 8.76 cm). 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 younger than 5 was 92.6% vs 61.8% for those 5 and older (p=0.002). Patients presenting with tumor size 9 cm and greater had worse three-year OS compared to those with tumors < 9 cm (95.24% vs 67.1% respectively, p=0.02).
Conclusions: ACC is a rare pediatric malignancy with a female predominance. Those older than 4 years and those with presenting tumor size ≥ 9 cm have decreased overall survival rates after adrenalectomy for localized disease. 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.


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