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The following is a summary of “Clinical Presentation, Management, and Outcome in Neurolymphomatosis: A Systematic Review,” published in the August 2024 issue of Neurology by Kaulen et al.

Neurolymphomatosis (NL), a rare lymphoma involving the peripheral nervous system (PNS), poses diagnostic and therapeutic challenges due to overlapping symptoms, limited patient data, and lack of standardized treatment guidelines.

Researchers conducted a retrospective study to identify patient characteristics and prognostic factors associated with NL.

They searched PubMed and Scopus databases, adhering to PRISMA guidelines (2004 to 2023). Individual patient data on cases with a definitive NL diagnosis were included. Clinical, radiologic, pathologic, and outcome data were extracted. Survival analyses using log-rank tests and Cox proportional hazard models were performed.

The results showed that in 459 NL cases from 264 studies, primary NL occurred in 197 patients as the initial malignancy. At the same time, secondary NL, due to PNS relapse of known non-Hodgkin lymphoma, was seen in 262 cases after a median of 12 months. The NL was commonly presented as rapidly worsening, asymmetric, painful polyneuropathy. Infiltration affected peripheral nerves (56%), nerve roots (52%), plexus (33%), and cranial nerves (32%). The diagnosis was made at a median of 3 months after symptom onset, often with delays in primary NL. Diagnosis relied primarily on PNS biopsy or FDG-PET, with high diagnostic yields (>90%). Postmortem diagnoses were uncommon (3%). B-cell lymphomas accounted for 90% of cases. Tumor-directed therapy was given to 96% of patients, typically involving methotrexate or rituximab-based polychemotherapy. The median overall survival was 18 months. Favorable prognostic factors included primary NL without systemic disease (HR: 0.44; 95% CI 0.25–0.78; P=0.005), ECOG performance status <2 (HR: 0.30; 95% CI 0.18–0.52; P<0.0001), and rituximab-based treatment (HR: 0.46; 95% CI 0.28–0.73; P=0.001).

Investigators concluded that FDG-PET enhanced NL diagnosis, but diagnostic delays in primary cases persisted, while rituximab-based treatment improved outcomes, aiding early detection, prognostication, and treatment strategies for NL.

Source: neurology.org/doi/10.1212/WNL.0000000000209698