Gliomatosis cerebri: report of 3 cases
Jason F Harrison1, Hope T Richard, Ty W Abel
1College of Medicine, University of South Alabama, Mobile, Alabama 36607-3515, USA.
Insights
Gliomatosis cerebri (GC) is rare in children and hard to diagnose. A stereotactic biopsy is essential for confirming GC when clinical signs and imaging are unclear.
Area of Science:
- Pediatric Neurology
- Neuro-oncology
- Neurosurgery
Background:
- Gliomatosis cerebri (GC) is a rare glial neoplasm with a challenging diagnosis, particularly in pediatric patients.
- Early diagnosis is often hindered by nonspecific clinical presentations and neuroimaging findings.
Observation:
- The study details the presentation and diagnosis of GC in three pediatric patients aged 12, 14, and 16.
- Patients exhibited symptoms of increased intracranial pressure and varied neurological deficits.
Findings:
- Clinical presentation, cerebrospinal fluid (CSF) analysis, and neuroimaging (MRI) were nonspecific in diagnosing GC.
- Stereotactic biopsy was crucial for obtaining tissue for definitive pathological diagnosis.
Implications:
- These pediatric cases highlight the limitations of non-invasive diagnostic methods for GC.
- Neurosurgical intervention, specifically biopsy, plays an essential role in confirming the diagnosis.
- Gliomatosis cerebri should be considered in the differential diagnosis for children with diffuse neurological symptoms and widespread infiltrative brain lesions on MRI.
Abstract:
Gliomatosis cerebri is an uncommon glial neoplasm that is exceedingly rare in children and difficult to diagnose. The authors describe the presentation and diagnosis of GC in 3 children ages 12, 14, and 16 years. These children exhibited signs and symptoms of increased intracranial pressure as well as other vague or site specific neurological signs. Because clinical presentation, CSF analysis, and neuroimaging were nonspecific, a stereotactic biopsy to obtain tissue for pathological review was ultimately necessary to confirm the diagnosis. These pediatric cases underscore the limitations of relying solely on clinical presentation and neuroimaging and call to attention the essential role of neurosurgical intervention. The authors emphasize the need to maintain gliomatosis cerebri in the differential diagnosis of children presenting with diffuse neurological signs and MR imaging evidence of widespread, infiltrative lesions.
