Spectrum of qualitative and quantitative imaging of pilomyxoid, intermediate pilomyxoid and pilocytic astrocytomas in relation to their genetic alterations View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2022-08-19

AUTHORS

Sandra Abi Fadel, Marc von Reppert, Eve Kazarian, E. Zeynep Erson Omay, Asher Marks, Nicolas Linder, Karl-Titus Hoffmann, Armine Darbinyan, Anita Huttner, Mariam S. Aboian

ABSTRACT

PurposePilomyxoid astrocytomas (PMA) are pediatric brain tumors predominantly located in the suprasellar region, third ventricle and posterior fossa, which are considered to be more clinically aggressive than pilocytic astrocytomas (PA). Another entity, intermediate pilomyxoid tumors (IPT), exists within the spectrum of pilocytic/pilomyxoid astrocytomas. The 2021 WHO CNS classification refrained from assigning grade 1 or 2 status to PMA, thereby reflecting the need to further elucidate their clinical and imaging characteristics.MethodsWe included a total of 15 patients with PMA, IPT and suprasellar PA. We retrospectively evaluated immunohistochemistry, imaging findings and diffusion characteristics within these tumors as well as whole exome sequencing for three of the cases.Results87% of the tumors were supratentorial with 11 cases suprasellar in location, 1 case located in the frontal white matter and 1 in the hippocampus. 6 cases demonstrated intraventricular extension. ADC values were higher in PMA and IPT than PA. 3 cases demonstrated KIAA1549-BRAF-fusion, 2 had BRAFV600E\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$^{V600E}$$\end{document}-mutation and 6 were BRAF-wildtype. All cases had recurrence/progression on follow-up.ConclusionPMA and IPT do not demonstrate aggressive imaging characteristics in respect to their diffusion imaging with ADC values being higher than PA. Lack of BRAF-alteration in PMA corresponded to atypical location of tumors with atypical driver mutations and mechanisms. More... »

PAGES

1-11

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00234-022-03027-3

DOI

http://dx.doi.org/10.1007/s00234-022-03027-3

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https://app.dimensions.ai/details/publication/pub.1150356605

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/35984480


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