Cerebellar Pleomorphic Xanthoastrocytoma with BRAF V600E Mutation.


Journal

World neurosurgery
ISSN: 1878-8769
Titre abrégé: World Neurosurg
Pays: United States
ID NLM: 101528275

Informations de publication

Date de publication:
07 2020
Historique:
received: 24 03 2020
revised: 12 04 2020
accepted: 13 04 2020
pubmed: 30 4 2020
medline: 12 9 2020
entrez: 30 4 2020
Statut: ppublish

Résumé

Pleomorphic xanthoastrocytoma (PXA) is a rare type of astrocytoma with a peak incidence from 10 to 19 years of age. PXA is found most commonly in the supratentorium. When it is present in atypical areas such as the infratentorium, it is often overlooked as a potential differential diagnosis. This report discusses the case of an 18-year-old woman with a pure PXA in the left cerebellum. The case consists of interesting pathologic and molecular features which do not align with the literature about PXA: a positive finding for BRAF V600E mutation and a negative finding for cluster of differentiation 34 expression. A review of pure PXA cases in the infratentorium has also been completed. To our knowledge, this is the first case of PXA in the infratentorium with BRAF V600E mutation. The case also highlights the clinical relevance of including PXA as a differential diagnosis for lesions of the infratentorium.

Sections du résumé

BACKGROUND
Pleomorphic xanthoastrocytoma (PXA) is a rare type of astrocytoma with a peak incidence from 10 to 19 years of age. PXA is found most commonly in the supratentorium. When it is present in atypical areas such as the infratentorium, it is often overlooked as a potential differential diagnosis.
CASE DESCRIPTION
This report discusses the case of an 18-year-old woman with a pure PXA in the left cerebellum. The case consists of interesting pathologic and molecular features which do not align with the literature about PXA: a positive finding for BRAF V600E mutation and a negative finding for cluster of differentiation 34 expression. A review of pure PXA cases in the infratentorium has also been completed.
CONCLUSIONS
To our knowledge, this is the first case of PXA in the infratentorium with BRAF V600E mutation. The case also highlights the clinical relevance of including PXA as a differential diagnosis for lesions of the infratentorium.

Identifiants

pubmed: 32348888
pii: S1878-8750(20)30820-2
doi: 10.1016/j.wneu.2020.04.113
pii:
doi:

Substances chimiques

BRAF protein, human EC 2.7.11.1
Proto-Oncogene Proteins B-raf EC 2.7.11.1

Types de publication

Case Reports Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

577-581

Informations de copyright

Copyright © 2020 Elsevier Inc. All rights reserved.

Auteurs

Sang Ho Kim (SH)

School of Medicine, The University of Auckland, Auckland, New Zealand; Department of Neurosurgery, Seoul National University Bundang Hospital, Seongnam-si, Republic of Korea.

Kihwan Hwang (K)

Department of Neurosurgery, Seoul National University Bundang Hospital, Seongnam-si, Republic of Korea. Electronic address: ns.kihwan@gmail.com.

Kyu Sang Lee (KS)

Department of Pathology, Seoul National University Bundang Hospital, Seongnam-si, Republic of Korea.

Gheeyoung Choe (G)

Department of Pathology, Seoul National University Bundang Hospital, Seongnam-si, Republic of Korea; Department of Neurosurgery, Seoul National University College of Medicine, Seongnam-si, Republic of Korea.

Chae-Yong Kim (CY)

Department of Neurosurgery, Seoul National University Bundang Hospital, Seongnam-si, Republic of Korea; Department of Neurosurgery, Seoul National University College of Medicine, Seongnam-si, Republic of Korea.

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