The natural history of neurolymphomatosis

Neurolymphomatosis is a lymphoid malignancy of the peripheral nervous system and its natural history is poorly understood.

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.

Our search identified 559 patients and their median age was 61 years. Median overall survival (OS) was 12.0 (range 10.0-15.0) months. Diffuse large B-cell lymphoma was the most frequent histology, involving the brachial plexus, cranial nerves, and sciatic nerve. None had molecular profiling. There was a progressive lengthening of OS in successive decades, from 0.5 (95% CI 0.0-0.8) to 26.4 (95% CI 18.0-34.8) months between 1951 and 2022 (r² = 0.0528, p < 0.00001). Time from first treatment (treatment 1) to progression increased from 2.0 to 36.0 (95% CI 6.5-50.7) months (r² = 0.0961, p = 0.00236). Time from symptom onset to diagnosis remained unchanged (r² = 0.0000556, p = 0.939). Patients were most frequently treated with methotrexate, rituximab, and/or radiation either alone or in combination. Primary neurolymphomatosis had a better prognosis than secondary neurolymphomatosis. No OS difference was noted between B- and T-cell disease, but low-grade B-cell performed better than Burkitt's lymphoma.

Better outcome for patients with neurolymphomatosis is noted over time. But timely diagnosis remains a major problem that needs improvement.