Primary neurolymphomatosis diagnosis and treatment: A retrospective study

authors

• Lagarde Stanislas
• Tabouret E.
• Matta M.
• Franques J.
• Attarian S.
• Pouget J.
• Maues de Paula A.
• Figarella-Branger D.
• Dory-Lautrec P.
• Chinot O.
• Barrié M.

keywords

• MRI
• Electroneuromyography
• Peripheral nervous system
• Chemotherapy
• Lymphoma
• Neurolymphomatosis

abstract

Background: To discuss the therapeutic approach for primary neurolymphomatosis. Methods: We report all primary neurolymphomatosis cases referred to our institution, with descriptions of clinical, radiological, electrophysiological, histological features and long-term follow-up. We treated all patients with a combination of high-dose methotrexate and alkylating agents. Results: Five patients were diagnosed with histologically confirmed primary neurolymphomatosis. The majority of them presented with painful asymmetric sensory–motor neuropathy. Magnetic resonance imaging was abnormal in 4 of 5 patients, as shown with gadolinium enhancements. Electroneuromyography revealed denervation in all 4 cases with contributive examinations. All our patients received a chemotherapy combination of high-dose methotrexate and alkylating agent. Median progression-free survival was 8 months (2 complete responses and 2 partial responses), and overall survival was 24 months. Conclusions: Primary neurolymphomatosis is rare and polymorphic; it represents a difficult diagnosis of neuropathy. In our cohort, treatment with a chemotherapy combination with high-dose methotrexate showed encouraging results.

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