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Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models

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Malignant cell infiltration in the peripheral nervous system.

W Grisold¹, Chiara Briani, Andrea Vass

¹Department of Neurology, Kaiser-Franz-Josef Hospital, Austrian Cluster for Tissue Regeneration and Ludwig Boltzmann Institute for Experimental and Clinical Traumatology, Vienna, Austria.

Handbook of Clinical Neurology

|August 13, 2013

Summary

Malignancies rarely affect the peripheral nervous system through neoplastic nerve lesions. Diagnosis depends on tumor type, involvement patterns, and pain, with retrograde spread indicating a poor prognosis.

Keywords:

brachial plexus infiltration cranial nerve invasion dorsal root ganglia extracranial nerve lesions intravascular spread meningeal carcinomatosis neoplastic nerve infiltration nerve metastasis nerve root neurolymphomatosis

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Area of Science:

Neurology
Oncology
Pathology

Background:

The peripheral nervous system (PNS) can be affected by malignancies through various mechanisms.
Neoplastic lesions of peripheral nerves, including compression, invasion, and infiltration, are uncommon.
These lesions are most frequently observed in leukemia (neuroleukemiosis) and lymphoma (neurolymphomatosis).

Observation:

Neoplastic infiltration of peripheral nerves by solid tumors is rare, occurring even less frequently in sarcomas than other cancers.
Characteristic topographical sites for neoplastic infiltration include the skull base, head and neck region, and brachial and lumbosacral plexuses.
Malignant invasion can affect cranial nerves, particularly those of the face, with potential centripetal spread, and can also involve autonomic nerves and ganglia.

Findings:

The retrograde spread of malignancy within nerves is a significant indicator of a poor prognosis.
Clinical diagnosis relies on identifying the specific tumor type, the pattern of nerve involvement, and frequently, the presence of pain.

Implications:

Understanding these rare but serious manifestations of malignancy in the PNS is crucial for accurate diagnosis and prognosis.
This knowledge aids in developing targeted diagnostic and therapeutic strategies for patients with neuro-oncological conditions.
Further research into the mechanisms and clinical outcomes of neoplastic neuropathies can improve patient management and outcomes.