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Primary intracranial epithelioid angiosarcoma--case report

T Fuse1, T Takagi, M Hirose

  • 1Department of Neurosurgery, Nagoya City Higashi General Hospital.

Neurologia Medico-Chirurgica
|June 1, 1995
PubMed
Summary
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This case study details a rare epithelioid angiosarcoma in the brain. The tumor expressed epithelial markers, presenting unique diagnostic challenges and a poor prognosis for intracranial vascular tumors.

Area of Science:

  • Neuro-oncology
  • Pathology
  • Vascular Tumors

Background:

  • Primary intracranial tumors are rare, with epithelioid angiosarcomas being exceptionally uncommon.
  • Epithelioid angiosarcomas are aggressive vascular neoplasms that can occur in various locations, including the central nervous system.

Observation:

  • A 39-year-old male presented with left-hand weakness due to a right parietal lobe lesion.
  • Neuroimaging showed a well-defined, intensely enhancing lesion with surrounding edema.
  • Histopathological examination revealed an epithelioid vascular tumor with positive staining for cytokeratin, vimentin, Ulex europaeus agglutinin, and CD31.

Findings:

  • The tumor exhibited aggressive growth, necessitating multiple surgical resections.
  • Immunohistochemical analysis confirmed the expression of epithelial markers (cytokeratin, vimentin) alongside vascular markers (Ulex europaeus agglutinin, CD31).

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  • This represents the first reported case of an intracranial epithelioid angiosarcoma expressing epithelial markers.
  • Implications:

    • The expression of epithelial markers in this intracranial epithelioid angiosarcoma highlights diagnostic complexities.
    • This case underscores the aggressive nature and poor prognosis associated with intracranial epithelioid angiosarcomas.
    • Further research into the molecular mechanisms and therapeutic strategies for such rare tumors is warranted.