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Pseudotumor associated with CNS lymphoma

H L Hudson1, D R Hinton, D I Friedman

  • 1Department of Ophthalmology, University of Southern California, Los Angeles, Riverside, USA.

Journal of Neuro-Ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society
|September 1, 1996
PubMed
Summary
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A patient initially treated for orbital pseudotumor experienced a recurrence, which was diagnosed as central nervous system (CNS) lymphoma. This case highlights the importance of considering CNS lymphoma in recurrent orbital inflammatory conditions.

Area of Science:

  • Neuro-oncology
  • Ophthalmology
  • Neuropathology

Background:

  • Orbital pseudotumor is a rare idiopathic inflammatory condition that can affect the orbit and surrounding structures.
  • Intracranial extension of orbital pseudotumor is uncommon and can mimic other intracranial pathologies.
  • High-dose corticosteroids are a standard treatment for orbital pseudotumor.

Observation:

  • A 42-year-old immunocompetent woman presented with orbital pseudotumor and presumed intracranial extension into the temporal lobe.
  • Initial treatment with high-dose corticosteroids resulted in clinical and radiological improvement.
  • Five months later, the patient developed worsening headaches and recurrence of the temporal lobe lesion.

Findings:

  • Biopsy of the recurrent temporal lobe lesion revealed a central nervous system (CNS) lymphoma.

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  • Histopathology showed severe reactive gliosis adjacent to a subarachnoid lymphocytic infiltrate.
  • The findings suggest a misdiagnosis of orbital pseudotumor initially, with the underlying pathology being CNS lymphoma.

    • Implications:

    • This case underscores the critical need for thorough diagnostic evaluation in cases of suspected orbital pseudotumor with intracranial involvement.
    • Recurrence or lack of sustained response to corticosteroids in such cases should prompt consideration of alternative diagnoses, including CNS lymphoma.
    • Accurate and timely diagnosis of CNS lymphoma is crucial for appropriate treatment and improved patient outcomes.