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

被引：5

作者：

Ono, Takahiro ^{[1
]}

Sasajima, Toshio ^{[1
]}

Shimizu, Hiroaki ^{[1
]}

Natsumeda, Manabu ^{[2
]}

Kanamori, Masayuki ^{[3
]}

Asano, Kenichiro ^{[4
]}

Beppu, Takaaki ^{[5
]}

Matsuda, Kenichiro ^{[6
]}

Ichikawa, Masahiro ^{[7
]}

Fujii, Yukihiko ^{[2
]}

Ohkuma, Hiroki ^{[4
]}

Ogasawara, Kuniaki ^{[5
]}

Sonoda, Yukihiko ^{[6
]}

Saito, Kiyoshi ^{[7
]}

Nobusawa, Sumihito ^{[8
]}

Nakazato, Yoichi ^{[9
]}

Kitanaka, Chifumi ^{[10
]}

Kayama, Takamasa ^{[11
]}

Tominaga, Teiji ^{[3
]}

机构：

[1] Akita Univ, Dept Neurosurg, Grad Sch Med, 1-1-1 Hondo, Akita, Akita 0108543, Japan

[2] Niigata Univ, Brain Res Inst, Dept Neurosurg, Niigata, Niigata, Japan

[3] Tohoku Univ, Dept Neurosurg, Grad Sch Med, Sendai, Miyagi, Japan

[4] Hirosaki Univ, Dept Neurosurg, Grad Sch Med, Hirosaki, Aomori, Japan

[5] Iwate Med Univ, Dept Neurosurg, Morioka, Iwate, Japan

[6] Yamagata Univ, Fac Med, Dept Neurosurg, Yamagata, Yamagata, Japan

[7] Fukushima Med Univ, Dept Neurosurg, Fukushima, Fukushima, Japan

[8] Gunma Univ, Dept Human Pathol, Grad Sch Med, Maebashi, Gumma, Japan

[9] Hidaka Hosp, Dept Pathol, Takasaki, Gumma, Japan

[10] Yamagata Univ, Dept Mol Canc Sci, Sch Med, Yamagata, Yamagata, Japan

[11] Yamagata Univ, Fac Med, Dept Adv Med, Yamagata, Yamagata, Japan

来源：

NEUROLOGIA MEDICO-CHIRURGICA | 2020年 / 60卷 / 11期

关键词：

differential diagnosis; neuronal differentiation; pleomorphic xanthoastrocytoma; prognostic factors; TERT promoter mutation; TERT PROMOTER MUTATIONS; NESTIN; IDH1;

D O I：

10.2176/nmc.oa.2020-0155

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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.

引用

页码：543 / 552

页数：10

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