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Cerebral lymphomatoid granulomatosis. A report of two cases, with disseminated necrotizing leukoencephalopathy in one

M A Verity, W L Wolfson

    Acta Neuropathologica
    |October 15, 1976
    PubMed

    Insights

    This study presents two cases of cerebral lymphomatoid granulomatosis, detailing neuropathologic findings and treatment responses. One patient developed intracranial hypertension, while the other showed signs of leukoencephalopathy after therapy.

    Area of Science:

    • Neuropathology
    • Oncology
    • Immunology

    Background:

    • Cerebral lymphomatoid granulomatosis (CLG) is a rare lymphoproliferative disorder with potential neurological sequelae.
    • Understanding the neuropathologic manifestations and treatment outcomes of CLG is crucial for patient management.

    Observation:

    • Case 1: A 30-year-old male with pulmonary lymphomatoid granulomatosis developed acute intracranial hypertension and coma following Prednisone therapy, with neuropathology revealing a necrotizing lesion in the left putamen.
    • Case 2: An 18-year-old female with pulmonary lymphomatoid granulomatosis presented with neurological deficits suggestive of a brain mass. Despite treatment with irradiation and chemotherapy, she relapsed and was found to have disseminated necrotizing leukoencephalopathy post-mortem.

    Findings:

    • Neuropathologic examination in Case 1 showed atypical lymphoplasmacytic infiltration surrounding a necrotizing hemorrhagic lesion.
    • Case 2's autopsy revealed parenchymatous degeneration consistent with disseminated necrotizing leukoencephalopathy, rather than active CLG, suggesting a treatment-related complication.

    Implications:

    • These cases highlight the diverse neuropathologic sequelae of lymphomatoid granulomatosis and its treatments.
    • The findings underscore the importance of considering treatment-induced neurotoxicity, such as disseminated necrotizing leukoencephalopathy, in patients with a history of lymphoproliferative disorders and chemotherapy.

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