Bilateral and symmetric C1–C2 dumbbell ganglioneuromas associated with neurofibromatosis type 1 causing severe spinal cord compression
Received 30 July 2009; received in revised form 15 January 2010; accepted 27 January 2010.
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.
Department of Orthopedic Surgery, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita 010-8543, Japan
Corresponding author. Department of Orthopedic Surgery, Akita University Graduate School of Medicine, 1-1-1 Hondo, Akita 010-8543, Japan. Tel.: (+81) 18-884-6148; fax: (+81) 18-836-2617.
ABSTRACT