Abstract
OBJECTIVE: Rosette-forming glioneuronal tumor is a rare, rather recently described tumor featuring a highly distinctive, biphasic histological pattern, including a cytologically uniform neuronal component of Homer-Wright type pseudorosettes and an accompanying astrocytic element resembling pilocytic astrocytoma. Its occurrence in the posterior fossa and association with the fourth ventricle is stereotypical and a feature of all reported cases. CLINICAL PRESENTATION: In this article, we describe the first rosette-forming glioneuronal tumor arising outside this site, a histologically classic example involving the anterior visual pathway and associated with neurofibromatosis type 1. INTERVENTION: Genetic (fluorescent in situ hybridization) studies demonstrated no large deletion in either normal or neoplastic tissue, indicating that the genetic abnormality underlying neurofibromatosis type 1 in this patient is likely a very small deletion or point mutation. CONCLUSION: The relation of the tumor to the underlying neurofibromatosis type 1 cannot be assessed.
Original language | English (US) |
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Pages (from-to) | E771-E772 |
Journal | Neurosurgery |
Volume | 64 |
Issue number | 4 |
DOIs | |
State | Published - Apr 2009 |
Keywords
- Chiasm
- Neurofibromatosis type 1
- Optic nerve
- Rosette- forming glioneuronal tumor
ASJC Scopus subject areas
- Surgery
- Clinical Neurology