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[Large-cell intravascular malignant lymphoma].

B Croisile1, M Tommasi, A Jouvet

  • 1Service de Neurologie, Hôpital Neurologique P. Wertheimer, Lyon.

Revue Neurologique
|January 1, 1990
PubMed
Summary
This summary is machine-generated.

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Neoplastic angioendotheliosis, a rare disease of blood vessel proliferation, presented as subacute dementia and neurological deficits in a 57-year-old woman. Post-mortem analysis revealed widespread central nervous system lesions and neoplastic cells in multiple organs.

Area of Science:

  • Neurology
  • Oncology
  • Pathology

Background:

  • Neoplastic angioendotheliosis is a rare condition involving abnormal cell growth within blood vessels.
  • It typically manifests with dermatological and neurological symptoms.
  • This case highlights its severe, systemic, and fatal progression.

Observation:

  • A 57-year-old woman presented with 20 months of subacute dementia and transient neurological episodes.
  • Cerebrospinal fluid (CSF) protein levels were elevated, and MRI revealed white matter abnormalities.
  • Autopsy confirmed widespread ischemic lesions, particularly in the subcortical white matter.

Findings:

  • Microscopic examination showed significant proliferation of mononuclear cells within CNS blood vessels.
  • Immunohistochemistry confirmed these cells expressed leukocyte common antigen but not factor VIII-related antigen.

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  • Similar neoplastic cell infiltrates were found in the adrenal glands, liver, uterus, lungs, and kidneys.

    • Implications:

    • This case underscores the potential for neoplastic angioendotheliosis to cause severe neurological impairment and systemic disease.
    • The findings emphasize the importance of considering this rare condition in the differential diagnosis of unexplained dementia and neurological deficits.
    • Further research into the pathogenesis and treatment of neoplastic angioendotheliosis is warranted.