Real-world experience with management of spinal ganglioneuroma: long-term follow-up observations of 31 cases.

INTRODUCTION: Spinal ganglioneuroma (GN) is the most benign neoplasm of neuroblastic origin. There is little knowledge about spinal GN because of sporadic cases reported. The objective of this study is to describe the clinical manifestations and long-term follow-up outcomes of spinal GN after consecutive treatment. METHODS: The clinical and follow-up data of 31 patients with spinal GN receiving consecutive treatment in our institute are retrospectively analyzed. RESULTS: The mean age of the 31 patients was 40.39 ± 14.8 years. They were diagnosed with spinal GN and received surgical treatment in our institution between February 2012 and August 2019. Of them, 22 (71%) patients presented preoperative neurological symptoms. The mean duration from symptom onset to surgery was 19.76 ± 49.59 months. Eighteen patients received complete surgical resection and 13 patients received subtotal excision. In addition, radicotomy was performed simultaneously after sophisticated consideration in 19 patients. The follow-up period averaged 64.13 ± 22.67 months. The preoperative neurological dysfunction was improved significantly during the follow-up period in all cases. No local recurrence or malignant transformation has occurred so far. CONCLUSION: Spinal GN is a rare but benign neoplasm, and the origin of spinal GN remains controversial. With our experience, we propose that the origin of spinal GN from dorsal root ganglion should be equally considered as its counterpart of sympathetic ganglion. Complete resection is the preferred surgical strategy to avoid malignant events and recurrence. And the close postoperative follow-up observations are warranted.

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

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.

Spinal Ganglioneuroma: A Systematic Review of the Literature.

OBJECTIVE: Spinal ganglioneuromas (GNs) are rare benign tumors that often manifest as symptoms related to the compression of neural elements. The preferred treatment for affected patients is surgical resection, which typically improves symptoms and accompanies a low likelihood of tumor recurrence. We conducted a systematic review of reports of GNs involving the spinal cord and nerve roots, examining their clinical presentation, surgical management, and outcomes. METHODS: Using the keywords "ganglioneuroma" and "spinal," we conducted a systematic database review of MEDLINE (PubMed), Scopus, and Embase, querying studies reporting cases of spinal GNs. Patients' demographics, location of the tumors, clinical features, and surgical outcomes were extracted from eligible articles. RESULTS: A total of 93 spinal GN cases in 52 case reports/series met our criteria. Data analysis revealed a general male predominance, though thoracic spinal GNs were seen more in females. The mean age of patients with cervical, thoracic, thoracolumbar, and lumbar spinal GNs were 41.28, 27.65, 15.61, and 38.73 years, respectively. Multiple-level GNs were mostly seen in male patients or individuals with neurofibromatosis type 1. In all but 1 case, recurrence and reoperation were not reported in the short-term (months) and long-term (2-10 years) follow-up. CONCLUSIONS: We found unique epidemiologic characteristics for patients with GNs of different spinal regions. The treatment of choice is achieving gross total resection, but given the eloquency of the lesions, achieving decompression via subtotal resection can also be associated with improved outcomes. To date, no global postoperative surveillance protocol exists, considering the low recurrence rate and relevant cost-benefit ratios.

Spinal Ganglioneuroma.

An otherwise healthy 57-year-old man presented with intermittent low back pain and was incidentally found to have a left-sided paraspinal mass invading the spinal canal and causing spinal cord compression. He underwent a T11-12 hemilaminectomy, facetectomy, and instrumented fusion for a gross total resection with a good clinical outcome. Pathology revealed the lesion to be a ganglioneuroma. Ganglioneuroma is a rare and interesting pathology. These tumors are benign peripheral neuroblastic tumors derived from the neural crest and found along the entire neuroaxis. Tumors come to clinical attention if they cause symptomatic compression of neural structures or are found incidentally on imaging. Additionally, as these tumors share a common lineage with pheochromocytomas, systemic symptoms can be observed resulting from secretion of vasoactive peptides. The pathologic diagnosis of ganglioneuroma is predominantly based on morphology.

Giant intrathoracic ganglioneuroma with scoliosis treated by one-stage posterior resection and scoliosis correction: a case report.

Spinal ganglioneuroma occurs mostly in the thoracic spine causing various manifestations including scoliosis that can be misdiagnosed as idiopathic scoliosis. Few reports exist in the literature on the diagnosis and management of scoliosis secondary to huge ganglioneuroma and usually staged treatment is preferred. In this report, we present a 17-year-old female patient presented with back pain, lower limbs numbness, spinal deformity, and shortness of breath. Plain X-rays showed a 50° right thoracic scoliotic curve. MRI and chest CT revealed a huge extra pulmonary mass shifting the mediastinum with intra spinal extension through the left neural foramina compressing the spinal cord. Percutaneous US guided needle biopsy confirmed the diagnosis of ganglioneuroma. One-stage posterior instrumented correction of scoliosis, spinal cord decompression, and excision of the whole mass from the mediastinum and the spine through posterior approach was done for the patient with smooth postoperative recovery. Chest CT scan was done 2 years after surgery and excluded any local recurrence.