Ganglioglioma of the cervicothoracic spinal cord in a patient with neurofibromatosis type 1: A case report.

Abstract

Background: Gangliogliomas are rare tumors of the central nervous system. They are usually located intracranially and rarely in the spinal cord. There is no clear correlation between this tumor and neurofibromatosis type 1 (NF1) with only four cases described. The aim of this article is to describe one more case and add data to the literature regarding this rare association.Case Description: An 8-year-old boy with NF1 presented progressive asymmetrical paraparesis (Grade 4 medical research council scale on the right leg and Grade 3 on the left leg). The cervicothoracic spinal magnetic resonance imaging demonstrated an intramedullary lesion from C4 to T4 vertebrae. The patient underwent a microsurgical resection. A partial resection was performed due to a drop in the motor evoked potential signal amplitude during dissection. Pathology report revealed a ganglioglioma (World Health Organization Grade I). Postoperatively, the patient evolved with worsening of the paraparesis. A few weeks later, he has improved his preoperative functional neurological state (better strength and gait). Adjuvant radiotherapy was not used. The patient is being followed up at the neurosurgery outpatient clinic.Conclusion: This is another case of spinal ganglioglioma associated with NF1. The tumor must be included in the differential diagnosis of patients with NF1 and spinal lesions. Complete microsurgical resection remains the standard treatment for spinal gangliogliomas, however, in this specific case, it was decided to leave a portion of the tumor to prevent neurological damage. The prognosis and treatment of this condition associated with NF1 remains to be determined.