Atypical pediatric ganglioglioma is common and associated with a less favorable clinical course

Abstract

<jats:sec> <jats:title>OBJECT</jats:title> <jats:p>Ganglioglioma (GG) is commonly recognized as a low-grade tumor located in the temporal lobe, often presenting with seizures. Most are amenable to complete resection and are associated with excellent oncological outcome. The authors encountered several GGs in various locations, which seem to have a less favorable clinical course than GGs in the temporal lobe.</jats:p></jats:sec> <jats:sec> <jats:title>METHODS</jats:title> <jats:p>The authors performed a single-center retrospective review of all children with a histological diagnosis of GG who were treated at Children’s Hospital Colorado between 1997 and 2013. Each tumor was categorized by 2 pediatric neuroradiologists as typical or atypical based on preoperative MRI appearance. Typical lesions were cortically based, within a single cerebral lobe, well-circumscribed, and solid or mixed solid/cystic. The treatment and clinical course of each patient was analyzed.</jats:p></jats:sec> <jats:sec> <jats:title>RESULTS</jats:title> <jats:p>Thirty-seven children were identified, with a median age at presentation of 8.2 years and median follow-up of 38.0 months. Eighteen tumors (48.6%) were typical and 19 (51.4%) were atypical. All typical lesions presented with seizures, whereas no atypical lesions did so. Sixteen (88.9%) typical lesions were located in the temporal lobe. In the atypical group, tumor location was variable, including 11 (57.9%) in the brainstem. Death during follow-up was statistically more common in the atypical group (31.6% vs 0%, p = 0.02). Gross-total resection (GTR) was achieved for 15 of 16 typical tumors (93.8%), compared with 3 atypical tumors (15.8%, p &lt; 0.0001). Presentation with seizure or non-brainstem location were each associated with survival (p = 0.02 and 0.004, respectively). The presence of mutation in <jats:italic>BRAF</jats:italic> exon 15 did not differ between the 2 groups.</jats:p></jats:sec> <jats:sec> <jats:title>CONCLUSIONS</jats:title> <jats:p>Pediatric GG with typical imaging features is associated with excellent rates of GTR and overall survival. Atypical GG is commonly encountered, less amenable to GTR, and associated with a worse outcome. This may relate to anatomical or biological characteristics and merits further investigation.</jats:p></jats:sec>

Novel TLE4‐NTRK2 fusion in a ganglioglioma identified by array‐CGH and confirmed by NGS: Potential for a gene targeted therapy

“…2 Mutations in BRAF are recognized as the most frequent genetic alteration in gangliogliomas, particularly BRAF:p.V600E mutation, which has been reported in up to 50% of these tumors. 1 A novel SLMAP-NTRK2 fusion was identified by RNA sequencing in one parietal ganglioglioma. 1 A novel SLMAP-NTRK2 fusion was identified by RNA sequencing in one parietal ganglioglioma.…”

“…1 Ganglioglioma classically arises in the temporal lobe, and patients present with medically refractory seizures. 1 Ganglioglioma classically arises in the temporal lobe, and patients present with medically refractory seizures.…”

“…[1][2][3][4][5] Infratentorial GGs are rather rare with only a few reports in the literature. [1][2][3][4][5][6][7] Gangliogliomas in the posterior fossa or spinal cord have a poorer outcome, but it is unclear whether anatomic location or an inherent variance in biologic behavior accounts for this difference. [1][2][3][4][5][6][7] Gangliogliomas in the posterior fossa or spinal cord have a poorer outcome, but it is unclear whether anatomic location or an inherent variance in biologic behavior accounts for this difference.…”

“…Ganglioglioma of the central nervous system is a rare tumor composed of both neoplastic glial and ganglion cells. 1 Ganglioglioma classically arises in the temporal lobe, and patients present with medically refractory seizures. [1][2][3][4][5] Infratentorial GGs are rather rare with only a few reports in the literature.…”

“…1 Ganglioglioma classically arises in the temporal lobe, and patients present with medically refractory seizures. [1][2][3][4][5] Infratentorial GGs are rather rare with only a few reports in the literature. 6 The histopathological hallmark of gangliogliomas is a combination of neuronal and glial cell elements.…”