Purpose: Tumors affecting peripheral nerves in children are rare. Accurate diagnosis ensures that management is appropriate and timely. A review of intrinsic nerve tumors was completed to differentiate common peripheral nerve lesions based on clinical characteristics and investigations.
Methods: A retrospective review was conducted for children (< 18 years old) diagnosed with an intrinsic tumor affecting peripheral nerve(s) or roots at the Children's Hospital of Eastern Ontario (CHEO) from 2009 to 2019.
Results: We report 14 children with perineurioma (N = 6), neurofibroma (N = 4), intraneural ganglion cyst (N = 2), or lipomatosis (N = 2). Mean age of symptom onset was 8.2 years (range 0.3 to 17.3 years). Presenting symptoms included muscle weakness (7/14), painless muscle wasting (2/14), contracture (1/14), pain (1/14), or the identification of a painless mass (3/14). Nerve conduction studies (NCS) or electromyography (EMG) were performed in 11/14 patients. MRI was useful at differentiating between these pediatric nerve tumors. Biopsies were performed in nine patients with additional surgical management pursued in four patients.
Conclusion: The rare nature of peripheral nerve tumors in children can pose diagnostic challenges. NCS/EMG are important to assist with localization, and MRI is useful to distinguish more benign tumors. Key MRI, clinical, and NCS features can in some cases guide management, potentially avoiding the need for invasive procedures.
Keywords: Ganglionic cyst; Lipomatosis; Magnetic resonance imaging; Nerve sheath neoplasms; Neurofibroma; Peripheral nerve.