Clinical, Pathological, and Molecular Characteristics of Diffuse Spinal Cord Gliomas

Abstract Diffuse spinal cord gliomas (SCGs) are rare tumors associated with a high morbidity and mortality that affect both pediatric and adult populations. In this retrospective study, we sought to characterize the clinical, pathological, and molecular features of diffuse SCG in 22 patients with hi...

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Bibliographic Details
Published in:Journal of neuropathology and experimental neurology 2022-10, Vol.81 (11), p.865-872
Main Authors: Garcia, Mekka R, Feng, Yang, Vasudevaraja, Varshini, Galbraith, Kristyn, Serrano, Jonathan, Thomas, Cheddhi, Radmanesh, Alireza, Hidalgo, Eveline T, Harter, David H, Allen, Jeffrey C, Gardner, Sharon L, Osorio, Diana S, William, Christopher M, Zagzag, David, Boué, Daniel R, Snuderl, Matija
Format: Article
Language:English
Subjects:
Adults
Biopsy
Brain cancer
Chemotherapy
DNA methylation
Genetic testing
Genomes
Glioma
Histology
Hospitals
Laboratories
Medical prognosis
Mutation
Pathology
Pediatrics
Radiation
Spinal cord
Surgery
Tumors
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Summary:Abstract Diffuse spinal cord gliomas (SCGs) are rare tumors associated with a high morbidity and mortality that affect both pediatric and adult populations. In this retrospective study, we sought to characterize the clinical, pathological, and molecular features of diffuse SCG in 22 patients with histological and molecular analyses. The median age of our cohort was 23.64 years (range 1–82) and the overall median survival was 397 days. K27M mutation was significantly more prevalent in males compared to females. Gross total resection and chemotherapy were associated with improved survival, compared to biopsy and no chemotherapy. While there was no association between tumor grade, K27M status (p = 0.366) or radiation (p = 0.772), and survival, males showed a trend toward shorter survival. K27M mutant tumors showed increased chromosomal instability and a distinct DNA methylation signature.
ISSN:0022-3069
1554-6578
DOI:10.1093/jnen/nlac075