Clinical Presentation, Management, and Outcome in Neurolymphomatosis: A Systematic Review

Area of Science:

• Neurology
• Oncology
• Pathology

Background:

• Neurolymphomatosis (NL) involves lymphoma infiltration of the peripheral nervous system (PNS), presenting diagnostic and therapeutic challenges.
• Limited cohort data hinders understanding of prognostic factors and consensus therapy for NL.
• This study aimed to characterize NL and identify prognostic factors.

Approach:

• A systematic literature review (2004-2023) followed PRISMA guidelines, analyzing individual patient data from 264 studies.
• Clinical, radiologic, and pathologic data were extracted for 459 Neurolymphomatosis cases.
• Univariable and multivariable survival analyses were conducted using log-rank tests and Cox proportional hazard models.

Key Points:

• Neurolymphomatosis predominantly manifests as rapidly progressive, asymmetric painful polyneuropathy, affecting peripheral nerves, nerve roots, plexus, and cranial nerves.
• Diagnosis, often delayed, relies on PNS biopsy or FDG-PET with high yields (>90%); most cases are B-cell lymphomas.
• Favorable prognostic factors include primary NL without systemic disease, good performance status (ECOG <2), and rituximab-based therapy.

Conclusions:

• Advances in neuroimaging, especially FDG-PET, improve Neurolymphomatosis diagnosis, though delays persist in primary cases.
• Rituximab-based therapy is associated with improved outcomes in Neurolymphomatosis patients.
• These findings aid clinicians in early Neurolymphomatosis recognition, prognostic assessment, and treatment strategies.