Primary neurolymphomatosis diagnosis and treatment: a retrospective study.
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.
Service de Neuro-Oncologie, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France. Electronic address: stanislas.lagarde@ap-hm.fr.
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Lagarde
Service de Neuro-Oncologie, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France.
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Tabouret
Service de Neuro-Oncologie, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France.
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Matta
Centre de Référence des Maladies Neuromusculaires et de la SLA, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France.
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Franques
Centre de Référence des Maladies Neuromusculaires et de la SLA, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France.
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Attarian
Centre de Référence des Maladies Neuromusculaires et de la SLA, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France.
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Pouget
Service d'anatomie pathologique et de neuropathologie, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France.
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Maues De Paula
Service d'anatomie pathologique et de neuropathologie, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France.
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Figarella-Branger
Service de neuroradiologie diagnostique et interventionnelle, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France.
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Dory-Lautrec
Service de Neuro-Oncologie, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France.
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Chinot
Service de Neuro-Oncologie, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue St-Pierre, 13005 Marseille, France.
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Barrié
D000368:Aged; D000477:Alkylating Agents / Q000627:therapeutic use; D000964:Antimetabolites, Antineoplastic / Q000627:therapeutic use; D018572:Disease-Free Survival; D004576:Electromyography; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D059906:Neuroimaging; D010524:Peripheral Nervous System Neoplasms / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D012189:Retrospective Studies
D000477:Alkylating Agents; D000964:Antimetabolites, Antineoplastic; D008727:Methotrexate
Chemotherapy; Electroneuromyography; Lymphoma; MRI; Neurolymphomatosis; Peripheral nervous system