Difficulties of Differentiating Primary Histiocytic Sarcoma of the Central Nervous System from Glioblastoma in Older Patients: Methylation Analysis and Review of the Literature Article Swipe
YOU?
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· 2025
· Open Access
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· DOI: https://doi.org/10.2176/jns-nmc.2024-0335
Primary histiocytic sarcoma of the central nervous system is an extremely rare malignancy, with only 43 cases reported to date. It often presents diagnostic challenges due to its rarity and similarities with other central nervous system tumors, particularly glioblastoma. We report a case of primary central nervous system histiocytic sarcoma in a 72-year-old woman, one of the older patients among the reported cases. The patient presented with gait disturbance and left hemiplegia. Initial imaging and intraoperative findings suggested a high-grade glioma. However, comprehensive pathological examination, including immunohistochemistry, electron microscopy, and genetic analyses, led to the diagnosis of histiocytic sarcoma. Notably, the tumor exhibited CDKN2A homozygous deletion and a unique methylation profile distinct from gliomas. Despite treatment with surgery, radiation, and chemotherapy, the patient experienced meningeal dissemination 8 months post-surgery and died 11 months after diagnosis. This case highlights the importance of comprehensive diagnostic evaluation in challenging central nervous system tumors and provides valuable insights into the molecular characteristics of central nervous system histiocytic sarcoma, particularly regarding CDKN2A deletion and methylation patterns. Awareness of this rare entity is crucial for accurate diagnosis and appropriate management of central nervous system tumors.
Related Topics
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.2176/jns-nmc.2024-0335
- https://www.jstage.jst.go.jp/article/nmccrj/12/0/12_2024-0335/_pdf
- OA Status
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- References
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- Related Works
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- OpenAlex ID
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Raw OpenAlex JSON
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https://openalex.org/W4411768446Canonical identifier for this work in OpenAlex
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https://doi.org/10.2176/jns-nmc.2024-0335Digital Object Identifier
- Title
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Difficulties of Differentiating Primary Histiocytic Sarcoma of the Central Nervous System from Glioblastoma in Older Patients: Methylation Analysis and Review of the LiteratureWork title
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articleOpenAlex work type
- Language
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enPrimary language
- Publication year
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2025Year of publication
- Publication date
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2025-06-29Full publication date if available
- Authors
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Ryota TAOMOTO, Mikiko Aoki, Toshiyuki Enomoto, Noriko Hirao, Hiroshi Abe, Junji Shibahara, Koichi Ichimura, Kaishi Satomi, Makoto HamasakiList of authors in order
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https://doi.org/10.2176/jns-nmc.2024-0335Publisher landing page
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https://www.jstage.jst.go.jp/article/nmccrj/12/0/12_2024-0335/_pdfDirect link to full text PDF
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diamondOpen access status per OpenAlex
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https://www.jstage.jst.go.jp/article/nmccrj/12/0/12_2024-0335/_pdfDirect OA link when available
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Glioblastoma, Histiocytic sarcoma, Central nervous system, Medicine, Primary central nervous system lymphoma, Methylation, Oncology, Sarcoma, Pathology, Internal medicine, Cancer research, Histiocyte, Biology, Genetics, GeneTop concepts (fields/topics) attached by OpenAlex
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0Total citation count in OpenAlex
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44Number of works referenced by this work
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10Other works algorithmically related by OpenAlex
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