Presentation
Multiple cranial neuropathies.
Patient Data
CASE OF THE MONTH: This case was selected as the Case of the Month for April 2024.
There is enlarged infiltration of the fifth cranial nerve bilaterally, leading to obliteration of Meckel cave, cavernous sinus, and foramen ovale bilaterally, more prominent on the left. The lesions are isointense to brain parenchyma on T1-weighted and T2-weighted images and demonstrate restricted diffusion and vivid contrast enhancement. A similar lesion in the bilateral cranial nerves IX and X extends to the jugular foramina.
There is vivid contrast enhancement of the meatal portion of both facial nerves.
No brain lesions were observed. CT of the thorax and abdomen (not shown) were normal.
There is enlargement and vivid contrast enhancement along the C5, C8, and T1 nerve roots with lateral recess obliteration, more prominent on the right.
Histopathology
Microscopic description: the test sample reveals large cells with bright or enlarged nuclei, centrally located within the nucleus. Numerous mitotic cells are present, interspersed with nerve ganglion cells. The specimen is stained immunohistochemically with markers CD3(+) for small cells, CD20(+) for B cells, Ki67 at 40%, Synaptophysin(-), and Chromogranin A(-).
Conclusion: B-cell lymphoma, possibly suggestive of neurolymphomatosis.
Case Discussion
Neurolymphomatosis occurs when non-Hodgkin lymphoma invades cranial nerves, peripheral nerve roots, plexuses, or nerves themselves.
Magnetic resonance imaging plays a crucial role as the most sensitive and specific noninvasive diagnostic tool. Nerves, roots, and plexuses show enlargement and enhancement after the administration of gadolinium.
The radiographic differential diagnosis includes acute or chronic inflammatory radiculoneuropathies and peripheral nerve sheath tumors.
Biopsy of involved peripheral nervous system structures remains the diagnostic gold standard.
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