ABSTRACT
This article reviews the pathology and management of peripheral nerve tumours, including a framework for investigation and decision-making. Most tumours are benign, including schwannomas and neurofibromas, but malignant peripheral nerve sheath tumours can occur. The risk of malignant change is remote for schwannomas but higher for neurofibromas, particularly in neurofibromatosis type 1. Magnetic resonance imaging is useful for defining the relationship of a swelling with adjacent nerves but is not definitive for tissue diagnosis. Increasing size, pain and neurological deficit suggest malignant change and TruCut needle biopsy is indicated, although there is a risk of sampling error. Excision biopsy preserving nerve function may be carried out for benign tumours to relieve symptoms. Malignant tumours require a multidisciplinary approach. Complete surgical excision with clear margins is the only curative treatment and may be supplemented with radiotherapy and chemotherapy. However, prognosis remains poor, particularly for patients with neurofibromatosis.
Subject(s)
Algorithms , Peripheral Nervous System Neoplasms , Humans , Magnetic Resonance Imaging , Nerve Sheath Neoplasms/diagnosis , Nerve Sheath Neoplasms/pathology , Nerve Sheath Neoplasms/surgery , Nerve Sheath Neoplasms/therapy , Neurilemmoma/diagnosis , Neurilemmoma/pathology , Neurilemmoma/surgery , Neurilemmoma/therapy , Neurofibroma/diagnosis , Neurofibroma/pathology , Neurofibroma/surgery , Neurofibroma/therapy , Peripheral Nervous System Neoplasms/diagnosis , Peripheral Nervous System Neoplasms/pathology , Peripheral Nervous System Neoplasms/surgery , Peripheral Nervous System Neoplasms/therapyABSTRACT
LEVEL OF EVIDENCE: III.
ABSTRACT
A prospective database study was undertaken to investigate the severity and progression of elbow and forearm contractures in patients with obstetric brachial plexus injury. One-hundred and fifty-nine patients, who had not undergone nerve repair (mean age 12 years at last follow-up) (56 Narakas Group 1, 66 Group 2, 27 Group 3, 10 Group 4) were studied. Mean fixed flexion of the elbow at last follow-up was 13° for Group 1, 15° for Group 2, 19° for Group 3 and 24° for Group 4. Severe contracture of 30° or more developed in 31 children (mean age 118 months), with poorer active shoulder abduction being a significant risk factor. Onset of contracture was before the age of 5 years but did not increase substantially beyond 12 years. Active and passive pronation and supination tended to decrease with increasing injury severity. Seventeen children developed severe restriction of passive pronation (supination contracture) (mean age 69 months), and 12 developed severe restriction of passive supination at a mean of 137 months.Level of evidence: III.
