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Cited by 41Adrenal and Paraganglionic TumorsNeuroblastoma Research and TreatmentsNeurofibromatosis and Schwannoma Cases

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

Wagner, Aaron Scott, Fleitz, Julie M., Kleinschmidt-DeMasters, B. K.·2005·Journal of Neuro-Oncology
DOI 10.1007/s11060-005-0376-z
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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.

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Crossref · 10.1007/s11060-005-0376-z

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  • There are several reports of ACCs in patients with FAP, neurofibromatosis type 1, and Werner syndrome.

    “There are several reports of ACCs in patients with familial adenomatous polyposis (FAP), neurofibromatosis type 1, and Werner syndrome (63–72).”

    From Adrenocortical carcinoma (2014) · cites this paper as [72]

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Authors
Wagner, A. S., Fleitz, J. M., & Kleinschmidt-DeMasters, B. K.
Affiliations
University of Colorado Health (US)
Venue
Journal of Neuro-Oncology
Publisher
Springer Science and Business Media LLC
Published
2005-11
Volume
75
Issue
2
Pages
127-133
ISSN
  • 0167-594X (ISSN-L)
  • 1573-7373
DOI
10.1007/s11060-005-0376-z
OpenAlex
W2082065488
Cited by
41
References
30
Citations by year
2026: 3 · 2024: 3 · 2023: 3 · 2022: 2 · 2021: 5 · 2020: 2 · 2019: 1 · 2018: 3 · 2017: 1 · 2016: 2 · 2015: 1 · 2014: 3 · 2013: 4 · 2012: 2
FWCI
0.63
Type
journal article
Language
en
Open access
closed
Licence
www.springer.com/tdm (tdm)
Locations
  • Journal of Neuro-Oncology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Health Sciences › Medicine › Neurology › Neuroblastoma Research and Treatments
  • Health Sciences › Medicine › Neurology › Neurofibromatosis and Schwannoma Cases
Keywords
adrenocortical carcinoma, pediatric adrenal tumors, brain metastases, neurofibromatosis type 1, hamartomatous lesions, masculinization, adrenalectomy, Li-Fraumeni syndrome, neurological symptoms
MeSH terms
Adrenal Cortex Neoplasms, Antineoplastic Combined Chemotherapy Protocols, Brain Neoplasms, Child, Child, Preschool, Chromosome Aberrations, Chylothorax, Diagnosis, Differential, Female, Follow-Up Studies, Hamartoma, Humans, Liver Neoplasms, Lung Neoplasms, Magnetic Resonance Imaging, Male, Neoplasm Metastasis, Neurofibromatosis 1, Time Factors, Treatment Outcome, Fatal Outcome, Adrenocortical Carcinoma, ErbB Receptors

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