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Related Experiment Videos

Central neurofibromatosis: a clinical-pathological correlation.

L R Grobman1, U Fisch, A Pollack

  • 1University of Miami School of Medicine, Florida.

The American Journal of Otology
|March 1, 1990
PubMed
Summary

Central neurofibromatosis, a rare genetic disorder, involves central nervous system tumors like bilateral acoustic neuromas. This case highlights a unique presentation without typical peripheral signs, emphasizing surgical approach considerations.

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Area of Science:

  • Neuro-oncology
  • Genetics
  • Otolaryngology

Background:

  • Central neurofibromatosis is a genetic disorder affecting neural crest derivatives, distinct from peripheral forms.
  • It commonly presents with central nervous system tumors, including bilateral acoustic neuromas, meningiomas, and gliomas.
  • Unlike peripheral neurofibromatosis, it often lacks peripheral neurofibromas and café au lait spots.