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Making the correct tumor diagnosis in the pontine location is thus critical. Here, we report a case study of a 14-month-old patient initially diagnosed as histone H3 wild-type DIPG. Due to the low age of the patient, the MRI appearance of DIPG, and anaplastic astrocytoma histology, intensive chemotherapy based on the HIT-SKK protocol with vinblastine maintenance chemotherapy was administered. Rapid clinical improvement and radiological regression of the tumor were observed with nearly complete remission with durable effect and excellent clinical condition more than 6.5\u00a0years after diagnosis. Based on this unexpected therapeutic outcome, genome-wide DNA methylation array was employed and the sample was classified into the methylation class \u201cLow-grade glioma, MYB(L1) altered.\u201d Additionally, RT-PCR revealed the presence of <jats:italic>MYB::QKI<\/jats:italic> fusion. Taken together, the histopathological classification, molecular-genetic and epigenetic features, clinical behavior, and pontine location have led us to reclassify the tumor as a pontine MYB-altered glioma. Our case demonstrates that more intensive chemotherapy can achieve long-term clinical effect in the treatment of MYB-altered pontine gliomas compared to previously used LGG-based regimens or radiotherapy. It also emphasizes the importance of a biopsy and a thorough molecular investigation of pontine lesions.<\/jats:p>","DOI":"10.1007\/s00381-023-05976-3","type":"journal-article","created":{"date-parts":[[2023,5,11]],"date-time":"2023-05-11T09:55:49Z","timestamp":1683798949000},"page":"2509-2513","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["DIPG-like MYB-altered diffuse astrocytoma with durable response to intensive chemotherapy"],"prefix":"10.1007","volume":"39","author":[{"given":"Katerina","family":"Trkova","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"David","family":"Sumerauer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lenka","family":"Krskova","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ales","family":"Vicha","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Miroslav","family":"Koblizek","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tomas","family":"Votava","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vladimir","family":"Priban","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michal","family":"Zapotocky","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2023,5,11]]},"reference":[{"key":"5976_CR1","doi-asserted-by":"publisher","first-page":"1190","DOI":"10.1093\/neuonc\/noaa024","volume":"22","author":"A Roux","year":"2020","unstructured":"Roux A, Pallud J, Saffroy R, Edjlali-Goujon M, Debily MA, Boddaert N, Sanson M, Puget S, Knafo S, Adam C et al (2020) High-grade gliomas in adolescents and young adults highlight histomolecular differences from their adult and pediatric counterparts. 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J Neurosurg Pediatr 20:347\u2013351. https:\/\/doi.org\/10.3171\/2017.5.PEDS16402","journal-title":"J Neurosurg Pediatr"}],"container-title":["Child's Nervous System"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00381-023-05976-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00381-023-05976-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00381-023-05976-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,16]],"date-time":"2023-08-16T10:05:09Z","timestamp":1692180309000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00381-023-05976-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5,11]]},"references-count":19,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2023,9]]}},"alternative-id":["5976"],"URL":"https:\/\/doi.org\/10.1007\/s00381-023-05976-3","relation":{},"ISSN":["0256-7040","1433-0350"],"issn-type":[{"value":"0256-7040","type":"print"},{"value":"1433-0350","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,5,11]]},"assertion":[{"value":"28 February 2023","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 April 2023","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"11 May 2023","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Approval for the study herein reported was provided by Institutional Ethics Committee of the Second Faculty of Medicine Charles University in Prague (ethics committee approval 17.6.2020).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"The authors of this publication declare that they have obtained the informed consent of the patient\u2019s legal representatives for the publication of their anonymized data for this case report.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}