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Multifocal primary central nervous system angiosarcoma: illustrative case.

Satoshi Hirai1, Yoshifumi Tao1, Hirotake Nishimura2

  • 1Departments of1Neurosurgery and.

Journal of Neurosurgery. Case Lessons
|July 3, 2023
PubMed
Summary
This summary is machine-generated.

Primary central nervous system (CNS) angiosarcoma, a rare vascular tumor, can rapidly spread, causing multiple brain hemorrhages. This case highlights the importance of considering multifocal angiosarcoma in such presentations.

Keywords:
disseminationmalignant mesenchymal tumornoduleprimary central nervous system angiosarcomasubarachnoid space

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Area of Science:

  • Neuro-oncology
  • Pathology
  • Vascular Biology

Background:

  • Primary intracranial angiosarcoma is exceptionally rare.
  • Most reported cases of central nervous system (CNS) angiosarcoma are solitary.
  • Angiosarcoma originates from vascular endothelial cells, presenting as a malignant mesenchymal tumor.

Purpose of the Study:

  • To report a rare case of primary CNS angiosarcoma.
  • To describe its rapid progression and multifocal dissemination.
  • To emphasize diagnostic considerations for CNS angiosarcoma.

Main Methods:

  • Surgical removal of tumor nodules from the brain surface and hematoma.
  • Pathological examination of tissue samples.
  • Immunohistochemical analysis using vascular endothelial markers.

Main Results:

  • A patient developed multiple disseminated cerebral hemorrhagic lesions rapidly.
  • Surgical specimens revealed tumor nodules mixed with hematoma.
  • Pathology confirmed atypical vascular endothelial cells in the subarachnoid space.

Conclusions:

  • Multifocal angiosarcoma occurred on the brain surface and ventricles, suggesting cerebrospinal fluid dissemination.
  • Rapidly progressing multifocal angiosarcoma can lead to fatal outcomes.
  • Consider multifocal angiosarcoma in cases of multiple cerebral hemorrhages on the brain surface.