Pediatric tectal glioma [Neurosurgery Wiki]

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====== Pediatric tectal glioma ======

{{rss>https://pubmed.ncbi.nlm.nih.gov/rss/search/1DK1DQN9MQ-BeubHAwIoICWbi1a9Orxc9hjrtryjp8Y1FktEu3/?limit=15&utm_campaign=pubmed-2&fc=20240206193230}}

[[Tectal glioma]] represents a subset of low-grade tumors that arise in the [[tectum]] at the roof of the [[brainstem]]. 
Göktürk et al. from the Department of Neurosurgery, University of Health Sciences, Kayseri City Hospital, [[Kayseri]], [[Turkey]].
 present two cases of pediatric patients with mesencephalic tectal plate tumors. An 11-year-old boy and a 15-year-old girl applied to the Emergency Department with different complaints. The 11 year-old-boy was treated with VP-shunt due to [[acute hydrocephalus]]
((Göktürk Y, Göktürk Ş, Kamaşak K. Pediatric tectal glioma presented with acute hydrocephalus and ventriculomegaly. Two case reports. Arch Argent Pediatr. 2024 Feb 15:e202310244. English, Spanish. doi: 10.5546/aap.2023-10244.eng. Epub ahead of print. PMID: 38320212.))
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4 children (diagnosed between 1 and 8 y, 3 females and 1 male) with molecularly distinct tectal gliomas (2 KRAS mutant, 1 EGFR mutant, 1 [[SRGAP3]]-RAF-1 fusion) that contributes to the growing literature of this uncommonly biopsied tumor. The patient with EGFR R222C mutation had a more severe course, earlier diagnosis, subsequent leptomeningeal metastatic disease, required more aggressive therapies, and died 9 years after diagnosis. Patients with KRAS mutations and SRGAP3-RAF-1 fusion had a more indolent course. Our series expands the molecular phenotype of tectal glioma with the potential for leptomeningeal dissemination. Future studies on establishing genotypic/phenotypic correlation from those who undergo biopsy are needed
((Yakir MJ, Elster JD, Paul MR, Khanna PC, Malicki DM, Levy ML, Crawford JR. Atypical Molecular Features of Pediatric Tectal Glioma: A Single Institutional Series. J Pediatr Hematol Oncol. 2023 Apr 1;45(3):e410-e414. doi: 10.1097/MPH.0000000000002596. Epub 2022 Nov 22. PMID: 36730386.))