Bilateral and symmetric C1-C2 dumbbell ganglioneuromas associated with neurofibromatosis type 1 causing severe spinal cord compression

Abstract

Background context: Ganglioneuromas are rarely located in the cervical region compressing the spinal cord. Only two cases of bilateral and symmetric dumbbell tumor have been reported previously.

Purpose: The purpose of the study was to present an additional case with bilateral and symmetric dumbbell ganglioneuromas of the cervical spine as part of multiple ganglioneuromas of the spine in a patient with neurofibromatosis type 1 (NF-1).

Study design: The study design was a case report.

Methods: A 15-year-old boy with NF-1 presented with a 6-month history of progressive tetraparesis. Magnetic resonance imaging showed voluminous bilateral and symmetric dumbbell masses at the C1-C2 level severely compressing the spinal cord. The spinal cord was also indented by a dumbbell mass at the left C3-C4 level. A systemic imaging survey of the patient showed numerous asymptomatic foraminal and extraforaminal tumors at all neuroforamina of the spine.

Results: The result was found to be surgical decompression of the spinal cord by subtotal resections of bilateral tumors at the C1-C2 level and unilateral tumor at the left C3-C4 level alleviated patient symptoms. Histopathological diagnosis was ganglioneuroma for all resected tumors.

Conclusion: Multiple ganglioneuromas, particularly bilateral and symmetric dumbbell tumors, are extremely rare but could be associated with NF-1.