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.

Keywords: Ganglioneuroma, Cervical spine, Dumbbell tumor, Neurofibromatosis type 1, Symmetric