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Current Management Strategies and Outcomes in Children With Adrenocortical Carcinoma.
Ziogas, Ioannis A; Hills-Dunlap, Jonathan L; Corkum, Kristine S; Cost, Nicholas G; Gosain, Ankush; Roach, Jonathan P.
Afiliação
  • Ziogas IA; Division 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. Electronic address: ioannis.ziogas@cuanschutz.edu.
  • Hills-Dunlap JL; Division 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.
  • Corkum KS; Division 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.
  • Cost NG; Surgical Oncology Program, Children's Hospital Colorado, Aurora, Colorado; Division of Urology, Department of Surgery, University of Colorado School of Medicine, Children's Hospital Colorado, Aurora, Colorado.
  • Gosain A; Division 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.
  • Roach JP; Division 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.
J Surg Res ; 301: 110-117, 2024 Sep.
Article em En | MEDLINE | ID: mdl-38925097
ABSTRACT

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.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Neoplasias do Córtex Suprarrenal / Carcinoma Adrenocortical Limite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male País como assunto: America do norte Idioma: En Ano de publicação: 2024 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Neoplasias do Córtex Suprarrenal / Carcinoma Adrenocortical Limite: Adolescent / Child / Child, preschool / Female / Humans / Infant / Male País como assunto: America do norte Idioma: En Ano de publicação: 2024 Tipo de documento: Article