Primary Neurolymphomatosis: A Literature Review.

Area of Science:

• Neurology
• Oncology
• Pathology

Background:

• Primary neurolymphomatosis (PNL) is a rare condition characterized by direct lymphomatous infiltration of the peripheral nervous system.
• Diagnosis is often delayed as the underlying hematological malignancy is not apparent at the onset of neurological symptoms.

Purpose of the Study:

• To comprehensively review published cases of PNL.
• To delineate the clinical features, diagnostic methods, progression, and treatment outcomes of PNL.

Main Methods:

• Extensive literature review of PNL cases.
• Extraction of demographic, clinical, paraclinical, treatment, and survival data.

Main Results:

• 301 cases analyzed (mean age 57.9 years, 61% male).
• Most common presentation: painless asymmetric neuropathy; other forms include radiculopathy and cranial nerve palsy.
• Diagnosis median 8 months, predominantly B-cell lymphoma (73%); 45% treatment response rate (chemotherapy, rituximab); 55% mortality.

Conclusions:

• PNL diagnosis is challenging and often delayed.
• B-cell lymphomas are the most frequent cause.
• Limited treatment efficacy and high mortality underscore the need for further research.