Adrenal cortical neoplasms in children: Why so many carcinomas and yet so many survivors?

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Adrenal cortical neoplasms in children are represented by a disproportionate number of cases that have been diagnosed pathologically as adrenocortical carcinomas (ACCs) - as many as 90% of all cortical tumors in some pediatric series. Like other solid malignancies of childhood, over half of ACCs present in the first 4 years of life in over 50% of cases. Most are sporadically occurring neoplasms, but ACCs are a manifestation of Beckwith-Wiedemann and Li-Fraumeni syndromes. Despite the fact that the microscopic features are often quite atypical and identical in many respects to ACCs in adults, the clinical outcome is favorable in 70% or more of cases. Tumor weight is seemingly a significant determinant in prognosis at a threshold of greater than 400 g. A risk assessment system is proposed that incorporates tumor weight, localization of tumor to the gland without invasion into the surrounding tissues or organs, and absence of metastasis.

Original languageEnglish
Pages (from-to)284-291
Number of pages8
JournalPediatric and Developmental Pathology
Issue number4
StatePublished - Jul 1 2009


  • Adrenal gland
  • Adrenocortical adenoma
  • Adrenocortical carcinoma
  • Beckwith-wiedemann syndrome
  • Li-fraumeni syndrome
  • P53


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