Primary neurolymphomatosis is the direct infiltration of lymphomatous neoplastic cells into nerve roots and/or peripheral nerves. A 67-year-old man had a 24-month history of progressive and severe left lower limb neuropathic pain, ipsilateral ankle dorsiflexion weakness and gait disturbance. Gadolinium-enhanced MRI showed thickening and enhancement of the cauda equina, L5, S1 and S2 nerve roots. Fluorodeoxyglucose positron emission tomography showed concordant hypermetabolism. L5 nerve root biopsy confirmed diffuse large B-cell lymphoma. One cycle of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP) chemotherapy resulted in remission, but this was not sustained. Primary neurolymphomatosis is rare and diagnostically challenging, and often the diagnosis is delayed. While biopsy is the gold standard for diagnosis, neuroimaging helps to characterise lesions and to determine the feasibility of biopsy.
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http://dx.doi.org/10.1136/pn-2023-003977 | DOI Listing |
Ann Indian Acad Neurol
November 2024
Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
BJC Rep
April 2024
Beth Israel Deaconess Medical Center & Harvard Medical School, Boston, MA, USA.
Background: Neurolymphomatosis is a lymphoid malignancy of the peripheral nervous system and its natural history is poorly understood.
Methods: We performed PubMed search and extracted clinical data for Kaplan-Meier statistics to determine outcome parameters over time. Kruskal-Wallis test was performed to compare prognostic factors.
Case Rep Oncol
October 2024
Division of Hematology, Department of Medicine, Juntendo University School of Medicine, Tokyo, Japan.
Am J Hematol
December 2024
Department of Neurology, Division of Neuro-Oncology, Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, Massachusetts, USA.
Lymphomatous infiltration of the peripheral nervous system (PNS), termed neurolymphomatosis, represents a distinct extranodal non-Hodgkin lymphoma variant with dismal outcome. CD19-directed chimeric antigen receptor (CD19-CAR) T-cell therapy has emerged as a safe and effective treatment for B-cell lymphomas. We aimed to assess toxicity and efficacy of CD19-CAR T-cells in neurolymphomatosis.
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