Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
December 2021
Next Case of the Month Coming January 4...
Neurolymphomatosis
- Background:
- The patient underwent biopsy of the right brachial plexus, which revealed neurolymphomatosis.
- Neurolymphomatosis (NL) is a rare manifestation of non-Hodgkin lymphoma, characterized by secondary infiltration of the nervous system.
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NL is related to non-Hodgkin lymphoma in 90% of the cases and is associated with diffuse large B-cell lymphoma 75% of the time.
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NL most frequently involves peripheral nerves followed by spinal nerve roots, cranial nerves, and brachial and lumbar plexuses.
- Clinical Presentation:
- The typical presentation is progressive sensorimotor neuropathy and initially patients generally show a painful neuropathy of the affected nerve.
- Key Diagnostic Features:
- Nerve biopsy is considered the gold standard but it is invasive and carries a risk of permanent nerve damage. MRI and FDG-PET/CT are considered alternatives for diagnosing NL.
- MRI findings: Fusiform enlargement of nerves with or without nodularity, increased T2 signal intensity, and moderate-to-intense enhancement in the postcontrast images
- FDG-PET/CT findings: Intense linear or fusiform uptake along the nerves
- Contrast-enhanced CT can demonstrate similar findings to MRI but offers limited soft-tissue contrast.
- Differential Diagnoses:
- In the setting of lymphoma, the causes can be grouped under direct neoplastic involvement including neurolymphomatosis, treatment-related neuropathy (chemotherapy or radiation), autoimmune or paraneoplastic processes related to lymphoma or systemic diseases, infections, and finally conditions that are unrelated to lymphoma, including compressive neuropathy.
- The imaging characteristics of these various conditions can overlap; however, the MRI findings and degree of FDG uptake of NL can lead to the correct diagnosis in the proper clinical context.
- Treatment:
- ​Systemic chemotherapy, intrathecal chemotherapy, and radiotherapy
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