Related Experiment Video
Updated: Jun 23, 2025
Generation of CAR T Cells for Adoptive Therapy in the Context of Glioblastoma Standard of Care
Published on: February 16, 2015
Current Management Strategies and Outcomes in Children With Adrenocortical Carcinoma
Ioannis A Ziogas1, Jonathan L Hills-Dunlap1, Kristine S Corkum1
1Division of Pediatric Surgery, Department of Surgery, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, Colorado; Surgical Oncology Program, Children's Hospital Colorado, Aurora, Colorado.
Insights
Pediatric adrenocortical carcinoma (ACC) management in the US primarily involves surgery and chemotherapy. Metastasis at diagnosis and older age in nonmetastatic cases are linked to poorer survival outcomes.
Area of Science:
- Pediatric Oncology
- Endocrine Surgery
- Cancer Epidemiology
Background:
- Adrenocortical carcinoma (ACC) is a rare, aggressive pediatric endocrine tumor.
- Recent US national data on pediatric ACC management and outcomes are lacking.
- This study addresses the need for updated information on pediatric ACC.
Purpose of the Study:
- To examine clinical characteristics of pediatric ACC.
- To analyze current management strategies for pediatric ACC.
- To determine outcomes and survival rates for pediatric ACC.
Main Methods:
- Retrospective analysis of the National Cancer Database (2004-2019).
- Inclusion of pediatric patients (<18 years) diagnosed with ACC.
- Statistical analysis using Kaplan-Meier, log-rank tests, and Cox regression.
Main Results:
- 78 pediatric ACC cases were analyzed; median age 10 years, median tumor size 10.2 cm.
- 35.9% of patients had metastasis at diagnosis; 84.6% received surgery, 56.4% chemotherapy.
- Overall survival rates at 1, 3, and 5 years were 87.0%, 62.0%, and 60.1% respectively.
Conclusions:
- US pediatric ACC treatment commonly includes surgery and chemotherapy.
- Metastasis at diagnosis independently predicts inferior overall survival.
- In nonmetastatic ACC, increasing age independently predicts inferior overall survival.
Introduction:
Adrenocortical carcinoma (ACC) is a rare but aggressive pediatric endocrine tumor. However, there is no recent US national report on the management or outcomes of pediatric ACC. We aimed to examine the clinical characteristics, current management strategies, and outcomes of pediatric ACC.
Methods:
In this retrospective National Cancer Database study between 2004 and 2019, children (<18 y) with ACC were included. Overall survival was examined by means of Kaplan-Meier method, log-rank tests, and Cox regression modeling.
Results:
Seventy-eight children with ACC were included. The median age was 10 y, the median tumor size was 10.2 cm, and 35.9% had metastasis at diagnosis. Most patients underwent surgical treatment (84.6%), 56.4% received chemotherapy, and 7.7% received radiation. The 1-, 3-, and 5-y overall survival rates were 87.0%, 62.0%, and 60.1%, respectively. In unadjusted analysis, surgical treatment was associated with improved overall survival (log-rank test, P < 0.001). In multivariable Cox regression, metastasis at diagnosis was associated with inferior overall survival (hazard ratio: 2.72, 95% confidence interval: 1.15-6.40, P = 0.02), when adjusting for age, tumor size, receipt of surgical treatment, and chemotherapy. In patients with nonmetastatic ACC, increasing age was associated with inferior overall survival (hazard ratio: 1.12, 95% confidence interval: 1.00-1.24, P = 0.04), when adjusting for tumor size, receipt of surgical treatment, and chemotherapy.
Conclusions:
Most children with ACC in the USA undergo surgical treatment with about half of these also receiving chemotherapy. Metastasis at diagnosis was independently associated with inferior overall survival; in patients with nonmetastatic ACC, increasing age was independently associated with inferior overall survival.
Related Concept Videos
01:27Adrenal Gland Disorders
Adrenal insufficiency, characterized by insufficient cortisol and aldosterone production, leads to conditions like Addison's disease. This disorder, affecting the adrenal cortex, exhibits symptoms such as skin bronzing, dehydration, low blood pressure, fatigue, and weight loss. Congenital adrenal hyperplasia, a genetic ailment causing...
01:21Cancer Survival Analysis
01:27Tumor Immunotherapy
02:49Cancer Therapies
However, cancer treatments can pose several challenges, as therapies used to kill cancer cells are generally also toxic to normal cells. Moreover, cancer cells mutate rapidly and can develop resistance to chemical agents or radiation therapy. Besides, all types of cancer cells may not respond to the same therapy. Some cancer cells respond to one...
02:50Combination Therapies and Personalized Medicine
The combination of the drug acetazolamide and sulforaphane is a good example of combination therapy to treat cancer. The cells in the interior of a large tumor often die due to the hypoxic and...
01:30Adrenergic Agonists: Therapeutic Uses
Emergency and Intensive Care Unit (ICU) applications: Pressor agents increase blood pressure, heart rate, and contractility in shock and organ failure situations. Dopamine can induce vasodilation and stimulate adrenoceptors. Endogenous catecholamines are effective in treating cardiogenic shock. α2-agonists like clonidine can reverse anesthesia-induced hypertension.
Allergies and...