{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T17:54:25Z","timestamp":1787162065910,"version":"build-2736575974"},"reference-count":21,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2023,6,9]],"date-time":"2023-06-09T00:00:00Z","timestamp":1686268800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["por-journal.com"],"crossmark-restriction":true},"short-container-title":["Pathol. Oncol. Res."],"abstract":"<jats:p>\n                    <jats:bold>Introduction:<\/jats:bold>\n                    Angiocentric gliomas (AG) in brainstem location are exceedingly rare and might cause differential diagnostic problems and uncertainty regarding the best therapeutic approach. Hereby, we describe the clinicopathological findings in a brainstem AG presenting in a toddler child and review the literature.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Case report:<\/jats:bold>\n                    A 2-year-old boy presented with 5\u00a0weeks history of gait disturbances, frequent falls, left-sided torticollis and swallowing problems. MRI head showed a T2-hyperintense, partly exophytic mass lesion centred in the pontomedullary region, raising the possibility of diffuse midline glioma. The exophytic component was partially resected by suboccipital craniotomy, leaving intact the infiltrative component. Ventriculoperitoneal shunt was implanted due to postoperative hydrocephalus. Histological examination revealed a moderately cellular tumour consisted of bland glial cells infiltrating the brain parenchyma and radially arranged around the blood vessels. By immunohistochemistry, the tumour strongly expressed S100 and GFAP in addition to intense nestin positivity, while OLIG2 was negative in the perivascular tumour cells. DNA methylation array profiled the tumour as \u201cmethylation class diffuse astrocytoma,\n                    <jats:italic>MYB<\/jats:italic>\n                    or\n                    <jats:italic>MYBL1<\/jats:italic>\n                    -altered subtype B (infratentorial)\u201d and an in-frame\n                    <jats:italic>MYB::QKI<\/jats:italic>\n                    fusion was identified by RNA sequencing, confirming the diagnosis of angiocentric glioma. The patient has been initially treated with angiogenesis inhibitor and mTOR inhibitor, and now he is receiving palliative vinblastine. He is clinically stable on 9\u00a0months follow-up.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Conclusion:<\/jats:bold>\n                    Brainstem AG may cause a diagnostic problem, and the surgical and oncological management is challenging due to unresectability and lack of response to conventional chemo-radiation. In the future, genetically-tailored therapies might improve the prognosis.\n                  <\/jats:p>","DOI":"10.3389\/pore.2023.1611231","type":"journal-article","created":{"date-parts":[[2023,6,9]],"date-time":"2023-06-09T00:11:11Z","timestamp":1686269471000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":9,"title":["Case Report: Brainstem angiocentric glioma presenting in a toddler child\u2013diagnostic and therapeutic challenges"],"prefix":"10.3389","volume":"29","author":[{"given":"Zita","family":"Reisz","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bence Laszlo","family":"Radics","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Nemes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ross","family":"Laxton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laszlo","family":"Kaizer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Krisztina Mita","family":"Gabor","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timea","family":"Novak","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pal","family":"Barzo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Safa","family":"Al-Sarraj","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Istvan","family":"Bodi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2023,6,9]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"3077","DOI":"10.1038\/s41467-020-16682-y","article-title":"The integrated genomic and epigenomic landscape of brainstem glioma","volume":"11","author":"Chen","year":"2020","journal-title":"Nat Commun"},{"key":"B2","doi-asserted-by":"publisher","first-page":"30","DOI":"10.1186\/s40478-020-00902-z","article-title":"Pediatric low-grade glioma in the era of molecular diagnostics","volume":"8","author":"Ryall","year":"2020","journal-title":"Acta Neuropathol Commun"},{"key":"B3","doi-asserted-by":"publisher","first-page":"569","DOI":"10.1016\/j.ccell.2020.03.011","article-title":"Integrated molecular and clinical analysis of 1,000 pediatric low-grade gliomas","volume":"37","author":"Ryall","year":"2020","journal-title":"Cancer Cell"},{"key":"B4","doi-asserted-by":"publisher","first-page":"875","DOI":"10.1097\/01.jnen.0000182981.02355.10","article-title":"Monomorphous angiocentric glioma: A distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma","volume":"64","author":"Wang","year":"2005","journal-title":"J Neuropathol Exp Neurol"},{"key":"B5","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1007\/s00401-007-0243-4","article-title":"The 2007 WHO classification of tumours of the central nervous system","volume":"114","author":"Louis","year":"2007","journal-title":"Acta Neuropathol"},{"key":"B6","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1038\/ng.3500","article-title":"MYB-QKI rearrangements in angiocentric glioma drive tumorigenicity through a tripartite mechanism","volume":"48","author":"Bandopadhayay","year":"2016","journal-title":"Nat Genet"},{"key":"B7","doi-asserted-by":"publisher","first-page":"833","DOI":"10.1007\/s00401-016-1539-z","article-title":"Genetic alterations in uncommon low-grade neuroepithelial tumors: BRAF, FGFR1, and MYB mutations occur at high frequency and align with morphology","volume":"131","author":"Qaddoumi","year":"2016","journal-title":"Acta Neuropathol"},{"key":"B8","doi-asserted-by":"publisher","first-page":"732","DOI":"10.1038\/nrneurol.2016.173","article-title":"Low-grade epilepsy-associated neuroepithelial tumours \u2014 The 2016 WHO classification","volume":"12","author":"Bl\u00fcmcke","year":"2016","journal-title":"Nat Rev Neurol"},{"key":"B9","doi-asserted-by":"publisher","first-page":"281","DOI":"10.1111\/j.1750-3639.2005.tb00112.x","article-title":"Angiocentric neuroepithelial tumor (ANET): A new epilepsy-related clinicopathological entity with distinctive MRI","volume":"15","author":"Lellouch-Tubiana","year":"2005","journal-title":"Brain Pathol"},{"key":"B10","doi-asserted-by":"publisher","first-page":"1709","DOI":"10.1097\/PAS.0b013e31804a7ebb","article-title":"Angiocentric glioma: Report of clinico-pathologic and genetic findings in 8 cases","volume":"31","author":"Preusser","year":"2007","journal-title":"Am J Surg Pathol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"671","DOI":"10.1007\/s00401-017-1759-x","article-title":"Angiocentric glioma with MYB-QKI fusion located in the brainstem, rather than cerebral cortex","volume":"134","author":"Chan","year":"2017","journal-title":"Acta Neuropathol"},{"key":"B12","doi-asserted-by":"publisher","first-page":"429","DOI":"10.1159\/000277616","article-title":"Angiocentric glioma-like tumor of the midbrain","volume":"45","author":"Covington","year":"2010","journal-title":"Pediatr Neurosurg"},{"key":"B13","doi-asserted-by":"publisher","first-page":"667","DOI":"10.1007\/s00401-017-1763-1","article-title":"Brainstem angiocentric gliomas with MYB-QKI rearrangements","volume":"134","author":"D\u2019Aronco","year":"2017","journal-title":"Acta Neuropathol"},{"key":"B14","doi-asserted-by":"publisher","first-page":"347","DOI":"10.3171\/2017.5.PEDS16402","article-title":"Brainstem angiocentric glioma: Report of 2 cases","volume":"20","author":"Weaver","year":"2017","journal-title":"J Neurosurg Pediatr"},{"key":"B15","doi-asserted-by":"publisher","first-page":"1231","DOI":"10.1093\/neuonc\/noab106","article-title":"The 2021 WHO classification of tumors of the central nervous system: A summary","volume":"23","author":"Louis","year":"2021","journal-title":"Neuro Oncol"},{"key":"B16","doi-asserted-by":"publisher","first-page":"1641","DOI":"10.3892\/ol.2020.11723","article-title":"Clinical characteristics, treatment and prognosis of angiocentric glioma","volume":"20","author":"Han","year":"2020","journal-title":"Oncol Lett"},{"key":"B17","doi-asserted-by":"publisher","first-page":"1091","DOI":"10.1007\/s00401-019-02081-1","article-title":"A single-center study of the clinicopathologic correlates of gliomas with a MYB or MYBL1 alteration","volume":"138","author":"Chiang","year":"2019","journal-title":"Acta Neuropathol"},{"key":"B18","doi-asserted-by":"publisher","first-page":"416","DOI":"10.17712\/nsj.2020.5.20200026","article-title":"Angiocentric glioma of brainstem","volume":"25","author":"Almubarak","year":"2020","journal-title":"Neurosciences"},{"key":"B19","doi-asserted-by":"publisher","first-page":"389","DOI":"10.1111\/jon.12983","article-title":"Neuroimaging features of angiocentric glioma: A case series and systematic review","volume":"32","author":"Kurokawa","year":"2022","journal-title":"J Neuroimaging Off J Am Soc Neuroimaging"},{"key":"B20","doi-asserted-by":"publisher","first-page":"181","DOI":"10.1007\/s00401-018-1879-y","article-title":"Practical implementation of DNA methylation and copy-number-based CNS tumor diagnostics: The heidelberg experience","volume":"136","author":"Capper","year":"2018","journal-title":"Acta Neuropathol"},{"key":"B21","doi-asserted-by":"publisher","first-page":"638","DOI":"10.1158\/1538-7445.AM2011-638","article-title":"Abstract 638: The impact of PI3K\/Akt pathway inhibition upon the c-myb oncogene","volume":"15","author":"Ho","year":"2011","journal-title":"Cancer Res"}],"container-title":["Pathology and Oncology Research"],"original-title":[],"link":[{"URL":"https:\/\/www.por-journal.com\/articles\/10.3389\/pore.2023.1611231\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,6,9]],"date-time":"2023-06-09T00:11:24Z","timestamp":1686269484000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.por-journal.com\/articles\/10.3389\/pore.2023.1611231\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,6,9]]},"references-count":21,"alternative-id":["10.3389\/pore.2023.1611231"],"URL":"https:\/\/doi.org\/10.3389\/pore.2023.1611231","relation":{},"ISSN":["1532-2807"],"issn-type":[{"value":"1532-2807","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,6,9]]},"article-number":"1611231"}}