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Congenital primary cerebral angiosarcoma. Case report.

Y Suzuki1, Y K Yoshida, R Shirane

  • 1Department of Neurosurgery, Tohoku University School of Medicine, Sendai, Japan. Yasuhiro@nsg.med.tohoku.ac.jp

Journal of Neurosurgery
|March 4, 2000
PubMed
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A rare congenital primary cerebral angiosarcoma was diagnosed in an infant presenting with vomiting. Surgical removal was successful, with no recurrence observed at 11 months post-operation.

Area of Science:

  • Pediatric Oncology
  • Neuro-oncology
  • Vascular Tumors

Background:

  • Central nervous system (CNS) angiosarcomas are exceptionally rare.
  • Congenital primary cerebral angiosarcomas are even rarer, with limited case reports in neonates.

Observation:

  • A 30-day-old infant presented with projectile vomiting and a tense anterior fontanelle, indicative of increased intracranial pressure.
  • A left frontotemporal tumor was identified as the cause of the infant's symptoms.
  • The tumor was highly vascular, well-circumscribed, and amenable to total surgical excision.

Findings:

  • Histopathological examination confirmed a malignant angiosarcoma.
  • Immunohistochemical analysis revealed diffuse positivity for endothelium-specific antigens (factor VIII-related antigen, CD31, CD34).

Related Experiment Videos

  • Magnetic resonance (MR) imaging demonstrated characteristic findings for this rare tumor type.

    • Implications:

    • This case highlights the importance of considering rare CNS tumors in infants with neurological symptoms.
    • Successful surgical resection offers a favorable prognosis for congenital primary cerebral angiosarcoma.
    • Further research into the specific characteristics and management of these rare pediatric brain tumors is warranted.