Intraneral perineurioma in children and adolescents

Abstract

Intraneral perineurioma (IP) is a rare and little-studied benign neoplasm of peripheral nerves in children and young adults. The clinical picture is usually a slowly progressive mononeuropathy, but plexuses with a predominance of motor deficits may also be involved. The sciatic nerve and its branches are most often affected. It is difficult to diagnose and requires a large amount of research to identify and confirm the diagnosis. Today, non-invasive neuroimaging methods such as magnetic resonance imaging (MRI) of nerve trunks and ultrasound of peripheral nerves are widely used for diagnosis, which are and allow getting ahead of the size and location of the neoplasm. MRI and ultrasound of the peripheral nerves reveal a focal fusiform enlargement of the nerve within one segment of the limb with an increase in the intensity of the MR signal from the IP. These neoplasms are considered rare, but recent advances in MRI and ultrasound diagnostics allow them to be detected at an early stage. MRI also helps distinguish IP from other peripheral nerve neoplasms. Confirmation of the diagnosis is based on histological examination of the altered nerve trunk. To date, there is no generally accepted strategy for the management of IP patients. Both conservative and surgical treatments are used. Neurolysis and nerve decompression improve neurological deficits in half of IP patients. IP does not recur after surgical treatment and does not become malignant during long-term follow-up of the patient.