Newly Identified Characteristics and Suggestions for Diagnosis and Treatment of Diffuse Leptomeningeal Glioneuronal/Neuroepithelial Tumors: A Case Report and Review of the Literature

Child Neurology Open
Megan R LyleBetty L Herrington

Abstract

Diffuse leptomeningeal glioneuronal tumor is unique for communicating hydrocephalus, diffuse leptomeningeal enhancement, cystic changes, absence of tumor cells in cerebral spinal fluid, and a cell population of both glial and neuronal copositivity. It has likely been misdiagnosed as mixed glioneuronal tumors, oligodendrogliomas, and neuroepithelial tumors. Children with signs of this tumor are often worked up for infection, rheumatologic disease, or disseminated primary malignancy, resulting in unnecessary testing and treatment. We describe a 14-year-old female with recurrent headaches, hydrocephalus, and diffuse leptomeningeal enhancement discovered to be neoplastic 1 year after initial presentation, owing to extensive and unrevealing infectious and immunologic workups. Biopsies revealed atypical cells with markers of both glial and neuronal cells, positivity for OLIG-2, and focal p53 positivity. Great response was seen with temozolomide and craniospinal irradiation. Additionally, we postulate additional diagnostic indicators that may aid in earlier diagnosis and treatment decisions.

References

Jun 27, 2008·Current Treatment Options in Neurology·Jaishri Blakeley, Stuart Grossman
Jun 3, 2009·Brain Pathology·Marina Paola GardimanGiorgio Perilongo
Sep 12, 2009·Brain Pathology·S RossiC Giannini
Dec 7, 2010·Cancer radiothérapie : journal de la Société française de radiothérapie oncologique·S HabererJ-J Mazeron
Oct 3, 2012·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Nozomi MatsumuraYoichi Nakazato

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Citations

Jan 4, 2020·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Wenlin ChenYu Wang
Apr 3, 2019·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Grace I L TanSharon Y Y Low
Sep 12, 2020·AJNR. American Journal of Neuroradiology·D A LakhaniS Pruthi
Nov 1, 2020·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Miguel Sáez-AlegreFernando Carceller Benito
Jan 4, 2022·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Weiqin ChengXiao Fan

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Methods Mentioned

BETA
biopsies
biopsy

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