Journal article
Cerebellar Gliomatosis in a Toddler: Case Report of a Challenging Condition and Review of the Literature
Journal of child neurology, v 27(4), pp 511-520
01 Apr 2012
PMID: 21940698
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
Gliomatosis confined to the cerebellum is most unusual. We report such a case in a 20-month-old male who presented with unsteadiness. Magnetic resonance imaging revealed a diffuse area of abnormal signal intensity within both cerebellar hemispheres, which did not enhance after contrast administration. The patient underwent a biopsy, which revealed a diffuse glioma infiltrating the cerebellum. Overall, the tumor cells had oligodendroglioma-like features and exhibited only focal vimentin immunoreactivity. They were negative for glial fibrillary acidic protein, synaptophysin, beta III-tubulin, and neurofilament protein. Immunofluorescence, performed on primary biopsy explants maintained in cell culture without exposure to growth factors or differentiation-promoting agents, revealed widespread nestin immunoreactivity and immunolabeling of occasional cells with antibodies to platelet-derived growth factor-alpha and O1/O4, markers of oligodendrocyte precursor-cells and immature oligodendrocytes, respectively. Fluorescent in situ hybridization performed on explants, touch preparations, and paraffin sections failed to reveal loss of heterozygosity for either 1p36 or 19q13. The patient was treated with temozolomide and remains stable, albeit with residual quiescent tumor, more than 3 years after surgery. This report calls attention to an unusual presentation of gliomatosis confined to the cerebellum of a toddler and addresses salient aspects of clinical and radiological differential diagnosis, as well as therapeutic challenges encountered.
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Details
- Title
- Cerebellar Gliomatosis in a Toddler: Case Report of a Challenging Condition and Review of the Literature
- Creators
- Jean-Pierre de Chadarevian - St. Christopher's Hospital for ChildrenAgustin Legido - St. Christopher's Hospital for ChildrenGregory E. Halligan - St. Christopher's Hospital for ChildrenEric N. Faerber - St. Christopher's Hospital for ChildrenJoseph H. Piatt - St. Christopher's Hospital for ChildrenJennifer D. Morrissette - St Christophers Hosp Children, Dept Pathol & Lab Med, Philadelphia, PA 19134 USAJahan Ara - #N# 6Department of Pediatrics, Neonatal Research Laboratory, Drexel University College of Medicine and St. Christopher’s Hospital for Children, Philadelphia, PA, USAMitzie L. Grant - St. Christopher's Hospital for ChildrenChristos D. Katsetos - St. Christopher's Hospital for Children
- Publication Details
- Journal of child neurology, v 27(4), pp 511-520
- Publisher
- Sage
- Number of pages
- 10
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Psychiatry; Pediatrics
- Web of Science ID
- WOS:000302231900014
- Scopus ID
- 2-s2.0-84859372970
- Other Identifier
- 991019169698804721
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InCites Highlights
Data related to this publication, from InCites Benchmarking & Analytics tool:
- Web of Science research areas
- Clinical Neurology
- Pediatrics