Molecular Features and Prognostic Factors of Pleomorphic Xanthoastrocytoma: A Collaborative Investigation of the Tohoku Brain Tumor Study Group

Pleomorphic xanthoastrocytoma (PXA) is a rare glial tumor, however, its histological differentiation from high-grade gliomas is often difficult. Molecular characteristics may contribute to a better diagnostic discrimination. Prognostic factors of PXA are also important but few relevant reports have...

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Veröffentlicht in:Neurologia medico-chirurgica 2020, Vol.60(11), pp.543-552
Hauptverfasser: ONO, Takahiro, SASAJIMA, Toshio, SHIMIZU, Hiroaki, NATSUMEDA, Manabu, KANAMORI, Masayuki, ASANO, Kenichiro, BEPPU, Takaaki, MATSUDA, Kenichiro, ICHIKAWA, Masahiro, FUJII, Yukihiko, OHKUMA, Hiroki, OGASAWARA, Kuniaki, SONODA, Yukihiko, SAITO, Kiyoshi, NOBUSAWA, Sumihito, NAKAZATO, Yoichi, KITANAKA, Chifumi, KAYAMA, Takamasa, TOMINAGA, Teiji, For the Tohoku Brain Tumor Study Group
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container_end_page 552
container_issue 11
container_start_page 543
container_title Neurologia medico-chirurgica
container_volume 60
creator ONO, Takahiro
SASAJIMA, Toshio
SHIMIZU, Hiroaki
NATSUMEDA, Manabu
KANAMORI, Masayuki
ASANO, Kenichiro
BEPPU, Takaaki
MATSUDA, Kenichiro
ICHIKAWA, Masahiro
FUJII, Yukihiko
OHKUMA, Hiroki
OGASAWARA, Kuniaki
SONODA, Yukihiko
SAITO, Kiyoshi
NOBUSAWA, Sumihito
NAKAZATO, Yoichi
KITANAKA, Chifumi
KAYAMA, Takamasa
TOMINAGA, Teiji
For the Tohoku Brain Tumor Study Group
description Pleomorphic xanthoastrocytoma (PXA) is a rare glial tumor, however, its histological differentiation from high-grade gliomas is often difficult. Molecular characteristics may contribute to a better diagnostic discrimination. Prognostic factors of PXA are also important but few relevant reports have been published. This study investigated the molecular features and prognostic factors of PXAs. Seven university hospitals participated in this study by providing retrospective clinical data and tumor samples of PXA cases between 1993 and 2014. Tumor samples were analyzed for immunohistochemical (IHC) neuronal and glial markers along with Ki67. The status of the BRAF and TERT promoter (TERTp) mutation was also evaluated using the same samples, followed by feature extraction of PXA and survival analyses. In all, 19 primary cases (17 PXA and 2 anaplastic PXA) were included. IHC examination revealed the stable staining of nestin and the close association of synaptophysin to NFP. Of the PXA cases, 57% had the BRAF mutation and only 7% had the TERTp mutation. On univariate analysis, age (≥60 years), preoperative Karnofsky performance status (KPS) (≤80%), and marked peritumoral edema were significantly associated with progression-free survival (PFS). No independent factor was indicated by the multivariate analysis. In conclusion, PXA was characterized by positive nestin staining and a few TERTp mutations. The neuronal differential marker and BRAF status may help in diagnosis. Patient age, preoperative KPS, and marked perifocal edema were associated with PFS. The present study is limited because of small number of cases and its retrospective nature. Further clinical study is needed.
doi_str_mv 10.2176/nmc.oa.2020-0155
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Molecular characteristics may contribute to a better diagnostic discrimination. Prognostic factors of PXA are also important but few relevant reports have been published. This study investigated the molecular features and prognostic factors of PXAs. Seven university hospitals participated in this study by providing retrospective clinical data and tumor samples of PXA cases between 1993 and 2014. Tumor samples were analyzed for immunohistochemical (IHC) neuronal and glial markers along with Ki67. The status of the BRAF and TERT promoter (TERTp) mutation was also evaluated using the same samples, followed by feature extraction of PXA and survival analyses. In all, 19 primary cases (17 PXA and 2 anaplastic PXA) were included. IHC examination revealed the stable staining of nestin and the close association of synaptophysin to NFP. Of the PXA cases, 57% had the BRAF mutation and only 7% had the TERTp mutation. On univariate analysis, age (≥60 years), preoperative Karnofsky performance status (KPS) (≤80%), and marked peritumoral edema were significantly associated with progression-free survival (PFS). No independent factor was indicated by the multivariate analysis. In conclusion, PXA was characterized by positive nestin staining and a few TERTp mutations. The neuronal differential marker and BRAF status may help in diagnosis. Patient age, preoperative KPS, and marked perifocal edema were associated with PFS. The present study is limited because of small number of cases and its retrospective nature. 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Med. Chir.(Tokyo)</addtitle><description>Pleomorphic xanthoastrocytoma (PXA) is a rare glial tumor, however, its histological differentiation from high-grade gliomas is often difficult. Molecular characteristics may contribute to a better diagnostic discrimination. Prognostic factors of PXA are also important but few relevant reports have been published. This study investigated the molecular features and prognostic factors of PXAs. Seven university hospitals participated in this study by providing retrospective clinical data and tumor samples of PXA cases between 1993 and 2014. Tumor samples were analyzed for immunohistochemical (IHC) neuronal and glial markers along with Ki67. The status of the BRAF and TERT promoter (TERTp) mutation was also evaluated using the same samples, followed by feature extraction of PXA and survival analyses. In all, 19 primary cases (17 PXA and 2 anaplastic PXA) were included. IHC examination revealed the stable staining of nestin and the close association of synaptophysin to NFP. Of the PXA cases, 57% had the BRAF mutation and only 7% had the TERTp mutation. On univariate analysis, age (≥60 years), preoperative Karnofsky performance status (KPS) (≤80%), and marked peritumoral edema were significantly associated with progression-free survival (PFS). No independent factor was indicated by the multivariate analysis. In conclusion, PXA was characterized by positive nestin staining and a few TERTp mutations. The neuronal differential marker and BRAF status may help in diagnosis. Patient age, preoperative KPS, and marked perifocal edema were associated with PFS. The present study is limited because of small number of cases and its retrospective nature. Further clinical study is needed.</description><subject>Astrocytes</subject><subject>Brain cancer</subject><subject>Brain tumors</subject><subject>Central nervous system diseases</subject><subject>differential diagnosis</subject><subject>Edema</subject><subject>Medical prognosis</subject><subject>Multivariate analysis</subject><subject>Mutation</subject><subject>Nestin</subject><subject>neuronal differentiation</subject><subject>Neuronal-glial interactions</subject><subject>Original</subject><subject>pleomorphic xanthoastrocytoma</subject><subject>prognostic factors</subject><subject>Synaptophysin</subject><subject>TERT promoter 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The status of the BRAF and TERT promoter (TERTp) mutation was also evaluated using the same samples, followed by feature extraction of PXA and survival analyses. In all, 19 primary cases (17 PXA and 2 anaplastic PXA) were included. IHC examination revealed the stable staining of nestin and the close association of synaptophysin to NFP. Of the PXA cases, 57% had the BRAF mutation and only 7% had the TERTp mutation. On univariate analysis, age (≥60 years), preoperative Karnofsky performance status (KPS) (≤80%), and marked peritumoral edema were significantly associated with progression-free survival (PFS). No independent factor was indicated by the multivariate analysis. In conclusion, PXA was characterized by positive nestin staining and a few TERTp mutations. The neuronal differential marker and BRAF status may help in diagnosis. Patient age, preoperative KPS, and marked perifocal edema were associated with PFS. The present study is limited because of small number of cases and its retrospective nature. Further clinical study is needed.</abstract><cop>Japan</cop><pub>The Japan Neurosurgical Society</pub><pmid>33071274</pmid><doi>10.2176/nmc.oa.2020-0155</doi><tpages>10</tpages><oa>free_for_read</oa></addata></record>
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source J-STAGE Free; DOAJ Directory of Open Access Journals; PubMed Central Open Access; EZB-FREE-00999 freely available EZB journals; PubMed Central
subjects Astrocytes
Brain cancer
Brain tumors
Central nervous system diseases
differential diagnosis
Edema
Medical prognosis
Multivariate analysis
Mutation
Nestin
neuronal differentiation
Neuronal-glial interactions
Original
pleomorphic xanthoastrocytoma
prognostic factors
Synaptophysin
TERT promoter mutation
title Molecular Features and Prognostic Factors of Pleomorphic Xanthoastrocytoma: A Collaborative Investigation of the Tohoku Brain Tumor Study Group
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