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Small B-cell lymphoma presenting as diffuse dural thickening with cranial neuropathies
Miguel Estevez1, Charleen Chu, Misha Pless
1Department of Neurology, University of Pittsburgh, PA, USA.
Journal of Neuro-Oncology
|September 21, 2002
Summary
A rare Epstein-Barr virus-negative lymphoma infiltrated the dura, causing severe neurological symptoms after liver transplant. Cranial radiation effectively treated this mucosa-associated lymphoid tissue (MALT) lymphoma.
Area of Science:
- Neuropathology
- Oncology
- Transplant Surgery
Background:
- Lymphoma can infiltrate the central nervous system, particularly in immunocompromised patients.
- Hepatitis C infection is a risk factor for certain non-Hodgkin's lymphomas.
- Pachymeningeal disease can present with diverse neurological deficits.
Observation:
- A patient developed diffuse pachymeningeal hypertrophy late after an orthotopic liver transplant.
- The lymphoma was Epstein-Barr virus-negative and immunophenotypically resembled MALT lymphoma.
- Neurological symptoms included scalp hyperesthesia, blindness, deafness, and ataxia.
Findings:
- Biopsy confirmed a small B-cell lymphoma with MALT-like immunophenotype infiltrating the dura.
- The lymphoma's rapid dural expansion caused significant neurological impairment.
- The patient's symptoms improved with cranial radiation therapy.
Implications:
- This case highlights a rare presentation of MALT lymphoma in the dura post-liver transplant.
- Early diagnosis and treatment, such as cranial radiation, are crucial for managing dural lymphoma.
- Understanding the association between hepatitis C and lymphoma may inform preventative strategies.