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.