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

@article{Dehner2009AdrenalCN,
  title={Adrenal cortical neoplasms in children: why so many carcinomas and yet so many survivors?},
  author={Louis P. Dehner and Dana Ashley Hill},
  journal={Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society},
  year={2009},
  volume={12 4},
  pages={284-91}
}
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… CONTINUE READING