Intramedullary spinal cord ganglioglioma: Case report and comparative literature review

Abstract

Introduction: Intramedullary spinal cord gangliogliomas (ISCGGs) account for 35-40% of all intramedullary tumors in children. These tumors have a different algorithm for treatment and prognosis than other medullary tumors, such as astrocytomas and spinal ependymomas. The objective of the study was to review the literature and examine an approach to diagnosing and treating this tumor based on a case report of ISCGG diagnosed at our center.

Method: An exhaustive review of cases of ISCGG published via the PubMed-NCBI platform between 1911 and 2018 was performed, and each patient's epidemiological characteristics, tumor location, symptoms, radiological diagnosis and treatment were appended.

Results: A total of 167 cases of ISCGG were found, including our own. In our sample, 52% of patients were female and the most commonly affected age group was 0-9 years of age (35% of patients). Motor deficit has been found to be the main symptom in a larger proportion of adults patients versus pediatric patients. On X-ray, this tumor shows greater hyperintensity and contrast uptake than astrocytomas and ependymomas, as well as a higher percentage of intratumoral cysts. The BRAF^V600E mutation is less common in spinal as opposed to supratentorial gangliogliomas. Surgery with complete resection is the treatment of choice. Only 19% of the patients in the sample received radiotherapy, and only 9% received chemotherapy as their only line of treatment.

Conclusions: ISCGGs are common in the pediatric population and require strong suspicion for proper diagnosis and treatment, as the risk of recurrence of ISCGGs is 3 times greater than that of supratentorial gangliogliomas.

Keywords: Ganglioglioma espinal; Ganglioglioma intramedular; Intramedullary ganglioglioma; Spinal cord ganglioglioma; Spinal tumor; Tumor espinal.