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Adrenocortical carcinoma in children: first population-based clinicopathological study with long-term follow-up

T M A Kerkhofs1, M H T Ettaieb1, R H A Verhoeven2

  • 1Department of Internal Medicine, Máxima Medical Centre, Eindhoven/Veldhoven, The Netherlands.

Oncology Reports
|September 23, 2014
PubMed

Insights

Pediatric adrenocortical carcinoma (ACC) is rare. Younger children (≤4 years) diagnosed with ACC had better survival rates, despite adverse histology, compared to older children (≥5 years).

Area of Science:

  • Pediatric Oncology
  • Endocrinology
  • Cancer Epidemiology

Background:

  • Adrenocortical carcinoma (ACC) is a rare malignancy affecting both pediatric and adult populations.
  • Significant differences exist in presentation, histology, and outcomes between pediatric and adult ACC.
  • There is a lack of comprehensive nationwide data on pediatric ACC.

Purpose of the Study:

  • To determine the incidence, presentation, pathological characteristics, treatment, and survival of pediatric ACC in The Netherlands.
  • To compare findings with existing international literature on pediatric ACC.
  • To identify factors influencing outcomes in pediatric ACC.

Main Methods:

  • A population-based nationwide study utilizing the Netherlands Cancer Registry (1993-2010).
  • Inclusion of all patients under 20 years at diagnosis.
  • Data collection through medical records and archival histological slide review via PALGA.

Main Results:

  • 12 pediatric ACC cases identified (8 female, 4 male) with a median age of 4.1 years.
  • Age-standardized incidence rate of 0.18 per million person-years.
  • Patients ≤4 years at diagnosis (n=7) survived, with a median follow-up of 97 months; patients ≥5 years (n=5) died, with a median survival of 6 months.

Conclusions:

  • Pediatric ACC is extremely rare in Western countries.
  • Younger children (≤4 years) exhibit significantly better survival outcomes than older children (≥5 years), irrespective of adverse histological features.
  • Clinical management for advanced pediatric ACC relies on adult protocols due to limited specific evidence.

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