Abstract
Background
Gangliogliomas are rare mixed neuronal-glial tumors of the central nervous system, accounting for less than 2% of intracranial tumors.
Case description
This report presents a rare case of ganglioglioma in the sellar region of a 3-year-old and 5-month-old pediatric patient. The patient underwent surgical intervention initially through a transnasal transsphenoidal approach and subsequently through a transcranial pterional craniotomy approach. Subsequently, radiotherapy and chemotherapy were administered for residual tumor tissue. The purpose of this report is to highlight the presence of ganglioglioma as a distinct diagnosis in sellar region tumors, discuss the surgical, radiotherapy, and/or chemotherapy treatment options for sellar region gangliogliomas based on the literature, and contribute the patient’s follow-up and treatment outcomes to the existing literature.
Conclusion
Complete tumor resection may not be feasible in sellar region gangliogliomas, especially in pediatric cases, due to endocrinological and vision-related complications. In cases where complete resection is not possible, radiotherapy and/or chemotherapy may be considered. However, the optimal treatment approach has not yet been established, and further research is needed.
Similar content being viewed by others
Data availability
Not applicable.
References
Durak MA (2019) Turk Norosir Derg. Nöronal ve Mikst Nöronal-Glial Tümörler 29(3):339–346
Lang FF et al (1993) Central nervous system gangliogliomas Part 2: Clinical outcome. J Neurosurg 79(6):867–873
Compton JJ et al (2012) Long-term outcomes for low-grade intracranial ganglioglioma: 30-year experience from the Mayo Clinic. J Neurosurg 117(5):825–830
Deng S et al (2015) Gliomas in the sellar turcica region: a retrospective study including adult cases and comparison with craniopharyngioma. Eur Neurol 73(3–4):135–143
Abuzayed B et al (2020) Ganglioglioma of optic chiasma: a case report and review of literature. Surg Neurol Int 11:392
Hong Y et al (2018) Ganglioglioma of the adenohypophysis mimicking pituitary adenoma: a case report and review of the literature. Medicine (Baltimore) 97(30):e11583
Seeburg DP, Dremmen MH, Huisman TA (2017) Imaging of the sella and parasellar region in the pediatric population. Neuroimaging Clin N Am 27(1):99–121
Zentner J et al (1994) Gangliogliomas: clinical, radiological, and histopathological findings in 51 patients. J Neurol Neurosurg Psychiatry 57(12):1497–1502
Garrido E et al (1978) Gangliogliomas in children. A clinicopathological study Childs Brain 4(6):339–346
Kalyan-Raman UP, Olivero WC (1987) Ganglioglioma: a correlative clinicopathological and radiological study of ten surgically treated cases with follow-up. Neurosurgery 20:428–433
Rumana CS, Valadka AB (1998) Radiation therapy and malignant degeneration of benign supratentorial gangliogliomas. Neurosrugery 42(5):1038–1043
Solomon A et al (2021) BRAF V600E-mutated ganglioglioma of the optic pathway: a case report and review of the literature. J Neuro-Ophthalmol 41(4):e723–e727
Ricard D et al (2007) Dynamic history of low-grade gliomas before and after temozolomide treatment. Ann Neurol 61(5):484–490
Dougherty MJ, Santi M, Brose MS, Ma C, Resnick AC, Sievert AJ, Storm PB, Biegel JA (2010) Activating mutations in BRAF characterize a spectrum of pediatric low-grade gliomas. Neuro Oncol 12:621–630
Shih KC, Shastry M, Williams JT, Jelsma PF, Abram SR, Ayyanar K, Burris HA 3rd, Infante JR (2014) Successful treatment with dabrafenib (GSK2118436) in a patient with ganglioglioma. J Clin Oncol 32(29):e98–e100
Philippe L, Maria K, Tariq A, Sofia M, Christine SM, Jean-Pierre F, Dudley RW, Sébastien P, Nada J, Valérie L (2019) Efficacy of dabrafenib for three children with brainstem BRAFV600E positive ganglioglioma. J Neurooncol 145(1):135–141. https://doi.org/10.1007/s11060-019-03280-2. Epub 2019 Sep 9. Erratum in: J Neurooncol. 2019 Dec;145(3):595. PMID: 31502039
Del Bufalo F, Carai A, Figà-Talamanca L, Pettorini B, Mallucci C, Giangaspero F, Antonelli M, Badiali M, Moi L, Bianco G et al (2014) Response of recurrent BRAFV600E mutated ganglioglioma to Vemurafenib as single agent. J Transl Med 12:356
McDuff SGR et al (2020) Radiation and chemotherapy for high-risk lower grade gliomas: Choosing between temozolomide and PCV. Cancer Med 9(1):3–11
Author information
Authors and Affiliations
Contributions
All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by [Buruc Erkan], [Suat Demir], [Ebubekir Akpinar] and [Tuce Soylemez Akkurt]. The first draft of the manuscript was written by [Buruc Erkan], [Suat Demir], [Osman Tanriverdi], [Omur Gunaldi] and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.
Corresponding author
Ethics declarations
Consent to participate
Informed consent was obtained from the legal guardians.
Conflict of interest
The authors declare no conflict of interest.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Erkan, B., Demir, S., Akpinar, E. et al. A rare tumor in the sellar region: ganglioglioma, a case report and a general overview. Childs Nerv Syst 39, 3621–3626 (2023). https://doi.org/10.1007/s00381-023-06073-1
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-023-06073-1