Pediatric and elderly polymorphous low‐grade neuroepithelial tumor of the young: Typical and unusual case reports and literature review

Polymorphous low‐grade neuroepithelial tumor of the young (PLNTY), one of the pediatric‐type diffuse low‐grade gliomas, is characterized by a diffuse infiltrating pattern of oligodendroglioma‐like tumor cells showing CD34 positivity and harbors mitogen‐activated protein kinase (MAPK) alteration, suc...

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Veröffentlicht in:Neuropathology 2023-08, Vol.43 (4), p.319-325
Hauptverfasser: Furuta, Takuya, Moritsubo, Mayuko, Muta, Hiroko, Shimamoto, Hotetsu, Ohshima, Koichi, Sugita, Yasuo
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container_end_page 325
container_issue 4
container_start_page 319
container_title Neuropathology
container_volume 43
creator Furuta, Takuya
Moritsubo, Mayuko
Muta, Hiroko
Shimamoto, Hotetsu
Ohshima, Koichi
Sugita, Yasuo
description Polymorphous low‐grade neuroepithelial tumor of the young (PLNTY), one of the pediatric‐type diffuse low‐grade gliomas, is characterized by a diffuse infiltrating pattern of oligodendroglioma‐like tumor cells showing CD34 positivity and harbors mitogen‐activated protein kinase (MAPK) alteration, such as vRAF murine sarcoma viral oncogene homolog B1 (BRAF) p.V600E or fibroblast growth factor fusion genetically. It occurs mainly in pediatric and adolescents with seizures due to the dominant location of the temporal lobe. However, there have been a few cases of PLNTY in adult patients, suggesting the wide range of this tumor spectrum. Here, we describe two cases of PLNTY, one in a 14‐year‐old female and the other in a 66‐year‐old female. The pediatric tumor showed typical clinical course and histopathology with BRAF p.V600E mutation, whereas the elderly tumor was unusual because of non‐epileptic onset clinically and ependymal differentiation histopathologically harboring KIAA1549‐BRAF fusion. There might be unusual but possible PLNTY, as in our elderly case. We also compared typical pediatric and unusual elderly tumors by reviewing the literature.
doi_str_mv 10.1111/neup.12889
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source MEDLINE; Wiley Online Library Journals Frontfile Complete
subjects Adolescent
Aged
Animals
Brain cancer
Brain Neoplasms - genetics
Brain Neoplasms - pathology
Brain tumors
Case reports
CD34 antigen
Child
Epilepsy
Female
Glioma
Glioma - genetics
glioneuronal tumor
Humans
Kinases
Literature reviews
long‐term epilepsy‐associated tumors
low‐grade glioma
MAP kinase
Mice
Mutation
Neoplasms, Neuroepithelial - genetics
Neoplasms, Neuroepithelial - pathology
Older people
Oligodendroglioma
Oligodendroglioma - genetics
Pediatrics
polymorphous low‐grade neuroepithelial tumor of the young
Proto-Oncogene Proteins B-raf - genetics
Sarcoma
Seizures
Temporal lobe
Tumor cells
title Pediatric and elderly polymorphous low‐grade neuroepithelial tumor of the young: Typical and unusual case reports and literature review
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