Spinal ganglioneuroma with extension to the brachial plexus. The challenges for total surgical excision, a case report.

ABSTRACT

INTRODUCTION: Ganglioneuromas are benign neurogenic tumors that arise from the sympathetic ganglia. They are less aggressive compared to the more immature neuroblastomas and ganglioneuroblastomas but can grow to exert mass effect on surrounding tissues. PRESENTATION OF CASE A 7 years old girl who presented with progressive quadriplegia for 4 months. On examination, she had a right supra-clavicular mass with reduced power in the right hand than the left. Power in the lower limbs was also reduced with hyper-reflexia, clonus and Babinski positive. Laboratory investigations were unremarkable and Chest X-Ray showed a widened mediastinum. Magnetic Resonance Imaging (MRI) scan revealed an extra-medullary spinal tumor at C6/C7 extending laterally on the right through the C6/7 neuro-foramen to the para-spinal tissue and brachial plexus. A C6/7 laminectomy with Spinal cord decompression by partial resection of the tumor was done. Histology of the resected tissue showed ganglioneuroma. DISCUSSION: The presentation of Ganglioneuromas is usually asymptomatic until they are huge enough to exert mass effect on surrounding tissue. Most are located in the posterior mediastinum, retro-peritoneum and neck. Due to this, it may be very challenging to achieve total resection especially when they surround major vessels or nerves. A multi-disciplinary approach is needed for the best surgical outcomes but this is not always realized in our setting. CONCLUSION: In resource limited settings, more collaboration and training is needed to realize appropriate management of complex surgical conditions. Although complications are not uncommon, total surgical excision is necessary to prevent recurrence and progression of Ganglioneuromas.