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Neurolymphomatosis as primary presentation of extra-nodal NK/T-cell lymphoma, nasal type

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Neurolymphomatosis (NL) describes an infiltration of cranial and peripheral nerves by lymphoma cells, most frequently in non-Hodgkin B-cell lymphoma. This clinical entity is rare and poses a challenging diagnosis. We describe a case of a 64-year-old female patient with NL associated with extra-nodal NK/T-cell lymphoma (ENKTL), nasal type, presenting as a painful progressive mononeuropathy multiplex with an oral cavity lesion. ENKTL is usually associated with Epstein-Barr virus (EBV) infection and rarely affects the central and peripheral nervous system. Lumbar puncture, magnetic resonance imaging (MRI), nerve biopsy, and 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) help to establish the diagnosis. Thereby, NL should be considered in the differential diagnosis of painful progressive multiple neuropathies, even in patients without previous history of cancer.

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Linfoma Extranodal de Células T-NK Neurolinfomatose Lymphoma, Extranodal NK-T-Cell Neurolymphomatosis

Contexto Educativo

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Neurol Sci . 2024 Mar 12. doi: 10.1007/s10072-024-07445-4.

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Licença CC

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