Cervical Dumbbell Ganglioneuroma Causing Quadriplegia

Abstract

Ganglioneuroma is a rare benign tumour that originates from the ganglion cells of the sympathetic nervous system. They are rare in cervical spine region and only 8 % of ganglioneuromas occur in the neck. The common sites of occurrence are in the posterior mediastinum, retroperitoneum and adrenal medulla, and as such, a cervical occurrence presenting with quadriplegia is a reportable event. We present a 26-year old young male with a two-year history of neck pain and progressive quadriplegia. He later became wheelchair-bound. Musculoskeletal examination revealed multiple generalized nodular skin swellings with café au lait macules. Magnetic resonance imaging showed a huge dumbbell tumour of the first two cervical vertebrae, to the right side of the spinal canal causing significant spinal cord compression. He had surgical intervention, aimed at complete tumour resection, postoperatively, power of the limbs improved to normal. Histological examination was consistent with ganglioneuroma. We present this report because the occurrence of ganglioneuroma is rare, secondly a cervical presentation is unusual and thirdly it presented as a rare cause of quadriplegia.

Large posterior mediastinal ganglioneuroma with intradural cervical spine extension: A rare case report and review of literature

“…The thoracic cavity (60%–80%, posterior mediastinum), the abdominal cavity (10%–15%, adrenal gland, retroperitoneum, pelvic, sacral, and coccygeal sympathetic ganglia, and the organ of Zuckerkandl), and the cervical area (5%) are the most common locations for GN [ 9 ]. The spinal canal contributes to approximately 5–8% of all GN [ 5 ]. On radiological imaging, a paraspinal ganglioneuroma can also extend through the neural foramen into the spinal canal, producing a dumbbell shape [ 10 ].…”

“…These tumors are typically asymptomatic and develop to a considerable size; however, depending on the location of the tumor, symptoms may occur from loco-regional compression [ 4 ]. Although there have been reports of ganglioneuromas with spinal extension, intradural extramedullary ganglioneuromas are exceedingly rare [ 5 ]. Here, we describe a 4-year-old boy who had a large ganglioneuroma having an intradural extramedullary extension with a large posterior mediastinal component compressing and displacing the surrounding structures.…”

Resection of Bilateral Symmetrical Multiple Level Cervical Ganglioneuroma in a 43-Year-Old Man, a Probable Case of Neurofibromatosis Type-1: Report of a Case and Review of Literature

“…Ganglioneuromas (GNs) are rare benign tumors of the sympathic nervous system with neural crest cell origin that usually are diagnosed in women and in the age below 20 [1][2][3]. As to the sites, GN is found more commonly in the posterior mediastinum, retroperitoneal space, adrenal medulla, and neck [1].…”

“…In this regard, cases of cervical spine GNs presenting with radiculopathy and/or myelopathy are quite rare [3,4]. Literature review indicates that there is an association between this tumor, multiple endocrine neoplasms (MEN) [5,6], and Von Recklinghausen's disease (NF-1) [2][3][4]. Although numerous bilateral symmetrical single-level cervi-cal spine GNs have been reported [2][3][4][7][8][9][10][11][12], multiple level cervical symmetrical dumbbell GNs have been only reported only twice in literatures yet [8,11].…”

“…Literature review indicates that there is an association between this tumor, multiple endocrine neoplasms (MEN) [5,6], and Von Recklinghausen's disease (NF-1) [2][3][4]. Although numerous bilateral symmetrical single-level cervi-cal spine GNs have been reported [2][3][4][7][8][9][10][11][12], multiple level cervical symmetrical dumbbell GNs have been only reported only twice in literatures yet [8,11]. Our present case is a probable case of Von Recklinghausen's disease (NF-1), with numerous skin dimples since childhood, who presented with cervical pain and myelopathy signs and symptoms.…”