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. 2023 Apr 1;45(3):e410-e414.
doi: 10.1097/MPH.0000000000002596. Epub 2022 Nov 22.

Atypical Molecular Features of Pediatric Tectal Glioma: A Single Institutional Series

Maayan Joy Yakir  1   2 , Jennifer D Elster  2   3 , Megan R Paul  2   3 , Paritosh C Khanna  2   4 , Denise M Malicki  2   5 , Michael L Levy  2   6 , John Ross Crawford  1   2   3   7
Affiliations

Atypical Molecular Features of Pediatric Tectal Glioma: A Single Institutional Series

Maayan Joy Yakir et al. J Pediatr Hematol Oncol. .

Abstract

We present 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.

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Conflict of interest statement

The authors declare no conflict of interest.

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