+ General Considerations
- Types: neuronoma, neurolemmoma, Schwannoma, malignant Schwannoma, neurofibroma, and neurofibrosarcoma
- Peripheral nerve sheath tumor is preferred Schwann cells are origin and all have similar biologic behaviour
- Fibrous, osteoid, chondroid, myxoid, and squamous patterns may be observed due to tumor differentiation
- Peripheral nerve sheath tumor commonly involve nerve roots of the brachial plexus
- Tumor progression occurs proximally and distally and may involve the spinal cord
- Metastatic disease is rare although pulmonary metastasis has been reported
+ Peripheral Nerve Sheath Tumor Classification
- Peripheral nerve sheath tumor classified according to level of involvement as:
- Peripheral: tumor involvement distal to brachial or lumbrosacral plexus
- Plexus: tumor involvement of nerves in brachial or lumbrosacral plexus and spinal nerves distal to intervertebral foramina
- Root: tumor involvement of ventral or dorsal nerve roots or tumor entering intervertebral foramina
+ Clinical Features
- Clinical signs are slowly progressive
- Lameness (77%) and muscle atrophy (93%) are common with pain and palpable mass other findings
+ Diagnosis
- Clinical signs and physical examination
- Advanced imaging using CT and MRI provides an indication of level of involvement and associated changes
- Myelography recommended for suspected spinal nerve root involvement
- EMG useful in delineating nerve root involvement
- CSF rarely useful
+ Treatment
Surgical treatment is recommended with technique depending on location:
- Local resection
- Amputation
- Laminectomy
- Combination of these procedures
+ Prognosis
- Prognosis depends on level of involvement and ability for complete surgical resection
- Excellent prognosis for peripheral classification of peripheral nerve sheath tumors due to ability for complete resection with no death related to tumor
- Poor prognosis for plexus and root peripheral nerve sheath tumors with death due to tumor in 78%
- No significant differences between plexus and root groups although trend for plexus tumors to survive longer