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An autopsy case of primary gliosarcoma with multiple extracranial metastases: pathology after administration of bevacizumab and genetic profile

Sato, Yoshiki Deguchi, Shoichi Norose, Tomoko Oishi, Takuma Mitsuya, Koichi Sugino, Takashi Akiyama, Yasuto Nagashima, Takeshi Urakami, Kenichi Shimoda, Yuji Ohshima, Keiichi Hayashi, Nakamasa Yamaguchi, Ken 名古屋大学

2023.11

概要

Gliosarcoma (GS) is a rare primary malignant brain tumor corresponding to World Health
Organization grade 4.1 It accounts for less than 0.5% of all intracranial tumors and is estimated
to comprise 1.8%–2.4% of glioblastoma, IDH-wild-type (GB) cases.2-4 In terms of the histopathology of GS, as one of the morphological variants of GB, it features a biphasic pattern with
gliomatous and sarcomatous components.4 Metastasis of GS has been reported to occur at a rate
as high as 11%, which is much higher than that of GB.3,5,6 A few reports on the pathology of the
extracranial metastasis of GS have shown that metastatic lesions had a sarcomatous component
alone or a mixture of gliomatous and sarcomatous ones.5,6 Therefore, it is considered that GS
tends to disseminate hematogenously due to its mesenchymal sarcomatous component.
GS has been treated in the same manner as GB.3-5 Bevacizumab is frequently used for the
recurrence of GB. However, the efficacy of bevacizumab for GS or its metastatic lesions is
still poorly understood. The pathology after the administration of bevacizumab is also unclear,
especially in metastatic specimens.
Recent genetic studies have demonstrated that GS shares gene alterations that also function
as drivers of GB. It has been reported that somatic mutations, such as in TP53 and PTEN, are
found in both gliomatous and sarcomatous components.7-9 Germline mutations associated with
GB, such as in TP53 and NF1, have been reported,10 but few with GS.
In this report, we review an autopsy case of GS with multiple extracranial metastases treated
by bevacizumab, including the results of genetic analysis. This study was approved by the ethics
committee of Shizuoka Cancer Center (J2021-56-2021-2-3). ...

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参考文献

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