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Primary central nervous system angiosarcoma: two case reports.

James R Hackney1, Cheryl Ann Palmer, Kristen O Riley

  • 1Division of Neuropathology, University of Alabama at Birmingham, PD6A 175, Birmingham, AL, 35294, USA. capalmer@uab.edu.

Journal of Medical Case Reports
|August 23, 2012
PubMed
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Primary brain angiosarcoma is rare. Complete surgical removal is standard care, with radiation and chemotherapy offering additional benefits for these uncommon brain tumors.

Area of Science:

  • Neuro-oncology
  • Vascular Neoplasms
  • Rare Cancers

Background:

  • Primary brain angiosarcoma is an exceptionally rare malignant vascular tumor.
  • Only 15 adult cases reported in the past 25 years, highlighting its rarity.

Purpose of the Study:

  • To present two detailed cases of primary brain angiosarcoma.
  • To review existing literature for diagnostic and treatment strategies.
  • To propose a rational approach for managing this rare condition.

Main Methods:

  • Detailed clinical case presentations of two patients with primary brain angiosarcoma.
  • Comprehensive characterization using advanced imaging, histopathology, and immunohistochemistry.
  • Systematic literature review on diagnosis and treatment.

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Main Results:

  • Case 1: A 35-year-old female with an extra-axial retro-orbital mass.
  • Case 2: A 47-year-old male with a sphenoid wing mass presenting with visual and cognitive deficits.
  • Literature review identified key diagnostic and therapeutic considerations.

Conclusions:

  • Gross total resection is the established standard of care for primary brain angiosarcoma.
  • Adjuvant therapies, including radiation and chemotherapy, are increasingly vital.
  • Multimodal treatment approaches are essential for managing these rare tumors.