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Predictors of survival in pediatric adrenocortical carcinoma: a Surveillance, Epidemiology, and End Results (SEER)

Jarod P McAteer1, Jorge A Huaco, Kenneth W Gow

  • 1Pediatric General and Thoracic Surgery, Seattle Children's Hospital and University of Washington, Seattle, WA 98105, USA. jarodmc@uw.edu

Journal of Pediatric Surgery
|May 25, 2013
PubMed

Insights

Pediatric adrenocortical carcinoma (ACC) is rare. Children aged 4 years or younger had significantly better outcomes and survival rates compared to older children with ACC.

Area of Science:

  • Pediatric Oncology
  • Cancer Epidemiology
  • Clinical Research

Background:

  • Adrenocortical carcinoma (ACC) is a rare malignancy in children, with varying global incidence rates.
  • Limited data exists on pediatric ACC, necessitating further investigation into incidence and prognosis.

Purpose of the Study:

  • To determine national incidence rates of pediatric adrenocortical carcinoma (ACC).
  • To identify independent prognostic indicators for children diagnosed with ACC.

Main Methods:

  • Utilized the SEER database (1973-2008) to identify pediatric patients (<20 years) with ACC.
  • Analyzed incidence rates and survival data, considering clinical and demographic factors.
  • Employed Cox proportional-hazards regression to identify predictors of disease-specific survival.

Main Results:

  • Identified 85 pediatric ACC cases; annual incidence was 0.21 per million.
  • Younger patients (≤4 years) presented with more favorable features: localized disease (76% vs. 31%) and smaller tumors (<10 cm, 69% vs. 31%).
  • Younger age (≤4 years) and localized disease were significant predictors of better survival; age remained significant after adjustment for tumor size.

Conclusions:

  • This study is one of the largest reviews of pediatric adrenocortical carcinoma (ACC).
  • Diagnosis at age 4 years or younger is associated with a significantly better prognosis.
  • Patient and tumor-related factors likely contribute to the improved outcomes observed in younger children.
Abstract

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