Gangliogliomas in the pediatric population
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ORIGINAL ARTICLE
Gangliogliomas in the pediatric population Arnold R. Quiroz Tejada 1 & Pablo Miranda-Lloret 1 & Margarita Llavador Ros 2 & Estela Plaza Ramirez 1 & Giovanni Pancucci 1 & Amparo Roca Barber 1 & Juan A. Simal-Julián 1 & Carlos Botella-Asunción 1 Received: 3 August 2020 / Accepted: 22 September 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Background Gangliogliomas are neoplastic lesions composed by a mixed population of neoplastic glial and dysplastic neural cells. They represent around 5% of all CNS tumors in the pediatric population. These usually are well-differentiated, slowgrowing tumors, meaning that complete resection could cure most of these patients. Although most lesions remain stable over time after incomplete resection, some patients develop progression of the residual lesions: the optimal approach to treat these tumors is still to be defined. Methods This is a retrospective study in which we obtained data from medical records of pediatric patients who had a histological diagnosis of ganglioglioma following surgical treatment at a single center between 2001 and 2020. Results We included 17 pediatric subjects with gangliogliomas. The median age at diagnosis was 6.7 years, and the median follow-up duration was 60 months. The most common clinical presentation was epileptic seizures (41.1%). Hydrocephalus was present in 29.4% of cases. 52.9% of tumors involved exclusively the cerebral hemispheres, with the temporal lobe being the most affected location. Gross total tumor resection (GTR) was accomplished in 47% of all cases and in 75% of hemispheric tumors. Of patients, 33% in whom GTR could not be achieved showed progression of the residual tumor. BRAF V600E mutation was present in 44.4% of cases. Conclusion Gangliogliomas are typically grade I tumors that occasionally affect children. They classically localize in the cerebral hemisphere but may involve deep structures like the basal ganglia, brain stem, and cerebellum, which seems to be particularly frequent in the pediatric population, implying further challenge to achieve adequate oncological control with surgery as the only treatment modality. Although most cases in which GTR could not be performed remained stable over the follow-up, significant progression of the tumor remains was observed in some patients. BRAF inhibitors should be considered as a feasible treatment option in this setting. Keywords Low-grade tumors . Child . BRAF V600E mutation
Introduction Gangliogliomas are neoplastic lesions composed by a mixed population of neoplastic glial and dysplastic neural cells. They represent around 5% of all CNS tumors in the pediatric population. The most frequent clinical presentation of these tumors is an epileptic syndrome because most of these tumors originate in the cerebral hemispheres, most commonly in the temporal lobe [1].
* Arnold R. Quiroz Tejada [email protected] 1
Department of neurological surgery, HUiP La Fe, Valencia, Spain
2
Department of Pathology, HUiP La Fe, Valencia, Spain
The
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