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Perineurioma (localized hypertrophic neuropathy)
Archives of Pathology & Laboratory Medicine
|July 1, 1984
Summary
Localized hypertrophic neuropathy (LHN) is a benign peripheral nerve tumor of perineurial cell origin, not a Schwann cell disorder. This condition presents as a slowly progressive mononeuropathy with severe focal neurologic deficit.
Area of Science:
- Neuropathology
- Peripheral Nerve Disorders
- Oncology
Background:
- Localized hypertrophic neuropathy (LHN) is a rare condition characterized by nerve enlargement.
- Previous understanding suggested LHN might involve Schwann cell hyperplasia.
Observation:
- Three patients presented with painless, slowly progressive mononeuropathy over years, leading to severe focal neurologic deficits.
- Gross examination revealed fusiform enlargement of affected nerves.
- Histological analysis showed nerve fiber loss, fascicular disorganization, and proliferation of unique elongated cells with whorl formation.
Findings:
- The proliferating cells lacked S-100 protein immunoreactivity.
- Ultrastructural examination distinguished these cells from Schwann cells.
- These findings indicate LHN is a benign peripheral nerve tumor originating from perineurial cells.
Implications:
- LHN is reclassified as a perineurial cell tumor, distinct from Schwann cell-related neuropathies.
- The study suggests a potential divergence in the developmental origins of perineurial and Schwann's cells.
- This research refines the understanding of peripheral nerve tumors and neurodevelopmental origins.