Pediatric adrenal cortical carcinoma: brain metastases and relationship to NF-1, case reports and review of the literature

J Neurooncol. 2005 Nov;75(2):127-33. doi: 10.1007/s11060-005-0376-z.

Abstract

Adrenal cortical carcinoma (ACC) is a rare childhood neoplasm that seldom manifests brain metastases; hence few papers in the literature focus on neurological manifestations associated with ACC. Although ACC is known to be a signature tumor type in several inherited cancer predisposition syndromes, particularly Li Fraumeni, ACC has not been previously associated with neurofibromatosis, type 1 (NF-1), an inherited disorder with frequent CNS lesions that might prompt concern for metastatic disease by neuroimaging studies. We present two pediatric patients with ACC and unusual CNS findings. The first child developed metastasis to the brain 4 years after resection of his adrenal primary and 2 and 1 years, respectively, after metastases to the liver and lungs. Soon after our experience with this patient, a girl with known NF-1 presented with virilization; adrenalectomy disclosed an ACC and systemic metastases were found within months. Disseminated disease prompted concern that her complex intracranial lesions identified by neuroimaging studies might represent brain metastases, but this proved to be NF1-related hamartomatous lesions. We review the literature on ACCs in pediatric patients regarding brain metastases and previous associations with NF-1.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adrenal Cortex Neoplasms / diagnosis*
  • Adrenal Cortex Neoplasms / drug therapy
  • Adrenal Cortex Neoplasms / metabolism
  • Adrenal Cortex Neoplasms / pathology*
  • Adrenal Cortex Neoplasms / surgery
  • Adrenal Cortex Neoplasms / ultrastructure
  • Adrenocortical Carcinoma / diagnosis*
  • Adrenocortical Carcinoma / drug therapy
  • Adrenocortical Carcinoma / metabolism
  • Adrenocortical Carcinoma / pathology*
  • Adrenocortical Carcinoma / surgery
  • Adrenocortical Carcinoma / ultrastructure
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Brain Neoplasms / secondary*
  • Child
  • Child, Preschool
  • Chromosome Aberrations
  • Chylothorax / complications
  • Chylothorax / surgery
  • Diagnosis, Differential
  • ErbB Receptors / metabolism
  • Fatal Outcome
  • Female
  • Follow-Up Studies
  • Hamartoma / diagnosis
  • Humans
  • Liver Neoplasms / secondary
  • Lung Neoplasms / secondary
  • Magnetic Resonance Imaging
  • Male
  • Neoplasm Metastasis
  • Neurofibromatosis 1*
  • Time Factors
  • Treatment Outcome

Substances

  • ErbB Receptors