Overview
Intraneural perineurioma is a benign tumor characterized by proliferation of perineurial cells within peripheral nerves, leading to localized hypertrophic neuropathy and progressive mononeuropathy 1. Histologically distinct from Schwann cell tumors, it results in nerve fiber loss and disorganized fascicular patterns without S-100 protein immunoreactivity 1.Diagnosis
Clinical Presentation: Painless, slowly progressive mononeuropathy over years, causing severe focal neurologic deficits 1.
Physical Examination: Reveals fusiform enlargement of affected nerves 1.
Imaging: MRI may show nerve enlargement but lacks specific diagnostic features 1.
Histology: Essential for diagnosis; characterized by elongated cells with whorl formation, negative for S-100 protein, and distinct ultrastructural features 1.
Electroneurography (ENG): Often shows axonal damage and slowed conduction velocities 1.
Biopsy: Definitive; confirms perineurial cell proliferation and exclusion of Schwann cell origin 1.Management
Surgical Excision: Primary treatment, aiming for complete resection to prevent further nerve damage 1.
Symptomatic Treatment: Pain management and physical therapy to support functional recovery 1.
Observation: In cases where surgery is not feasible, close monitoring for progression 1.Special Populations
Pediatrics: Limited data; management principles similar to adults but with consideration for growth and development 1.
Elderly: Surgical risks may be higher; individualized assessment required 1.
Comorbidities: Presence of comorbidities may influence surgical risk stratification and recovery 1.Key Recommendations
Confirm Diagnosis via Histological Examination: Essential for distinguishing intraneural perineurioma from other nerve lesions (Evidence: Strong 1).
Surgical Excision is Recommended for Treatment: Aim for complete removal to halt progression and preserve nerve function (Evidence: Moderate 1).
Close Monitoring in Non-Surgical Candidates: Regular follow-up necessary to assess for disease progression (Evidence: Expert opinion 1).References
1 Bilbao JM, Khoury NJ, Hudson AR, Briggs SJ. Perineurioma (localized hypertrophic neuropathy). Archives of pathology & laboratory medicine 1984. link