We report an extremely rare case with bilateral and symmetric dumbbell ganglioneuromas of the cervical spine in an elderly patient. A 72-year-old man came by ambulance to our hospital due to progressive incomplete paraplegia. Magnetic resonance imaging demonstrated bilateral symmetric dumbbell tumors at the C1/2 level. We performed total resection of the intracanalar tumor, aiming at complete decompression of the spinal cord, and partial and subtotal resection of foraminal outside portions. Histopathological examination of the surgical specimen indicated the tumor cells to be spindle cells with the presence of ganglion cells and no cellular pleomorphism, suggesting a diagnosis of ganglioneuroma. Although the surgery was not curative, the postoperative course was uneventful and provided a satisfactory outcome. This is the fourth known case of cervical ganglioneuromas of the bilateral symmetric dumbbell type.
Ganglioneuromas are rare, slow-growing benign tumors that usually develop from the sympathetic nervous system, of which 60% occur in children and young adults [
A 72-year-old man came by ambulance to our hospital due to progressive incomplete paraplegia. One month previously, he had felt left upper extremity numbness. He could not stand alone or walk because of sudden onset lightheadedness and reduced muscular strength in both legs. Manual muscle test of the right side arm and leg (below C2) was graded as 4- and that of the left side arm and leg (below C2) was graded as 4. He showed bladder dysfunction. He had incomplete sensory loss below C3. Tonus was increased in both legs and deep tendon reflexes were hyperactive. In addition, he had been diagnosed with gastric cancer and had undergone total gastrectomy and splenectomy, resulting in peritoneal dissemination.
Magnetic resonance imaging (MRI) demonstrated bilateral asymmetric masses at the C1/2 level. T1-weighted images exhibited hypointensity. T2-weighted images revealed heterogeneous hyperintensity (
After the posterior arch at C1 and cranial portion of the C2 lamina was removed and the dura was exposed (
Histopathological examination of the surgical specimen indicated the tumor cells to be spindle cells with the presence of ganglion cells and no cellular pleomorphism. Ganglion cells were dissipated throughout the tumor (
After the operation, the patient's paraplegia improved gradually and he became able to walk. MRI after operation showed that bilateral foraminal portions were left intact and the intracanal tumor was completely resected, with sufficient spinal cord decompression (
This case is the fourth known example of cervical ganglioneuromas of bilateral symmetric dumbbell type. The previous three cases are as follows. Ugarriza et al. [
In this case, the intradural portion of the tumor, as well as the extradural portion, was causing spinal cord compression. Therefore, resection of the intradural portion via durotomy, accompanied with debulking procedure for the extradural portions, was the chosen method. Taking into account the origin of the ganglioneuroma, the tumor arises from sympathetic nerve and there is thus the possibility of the dumbbell tumor becoming extended to the intracanal. However, in such cases, extradural extension is usual and intradural extension is very rare because the origin of these tumors is related to the sensory root ganglion and extended from the foramen outside [
Radilographically, ganglioneuromas showed a well defined, oval shape. Calcification of around 30% was seen on computed tomography scan. MR signal intensity was mainly low on T1-weighted images and high on T2-weighted images. Pathologically, ganglioneuromas are white, firm and encapsulated, slow-growing tumors. Histologically, these tumors contain large ganglion cells and areas of smaller lymphocyte-like cells within a matrix of fibrous and Schwann cells. Additionally, multinucleate cells with a well-defined nucleolus in each nucleus are commonly found. A diagnosis of ganglioneuroma is based on the absence of necrosis or immature ganglion cells [
For the treatment of symptomatic solitary ganglioneuromas, there is general agreement that total resection is essential. However, for multiple spinal ganglioneuromas in which complete resection at all sites is impossible, subtotal or partial resection can be an acceptable choice [
We described an extremely rare case with bilateral and symmetric dumbbell ganglioneuromas of the cervical spine in an elderly patient. We performed total resection of the intracanalar tumor, and partial and subtotal resection of the foraminal outside portions, aiming at complete decompression of the spinal cord.
No potential conflict of interest relevant to this article was reported.
Preoperative magnetic resonance images. T2-weighted sagittal images.
Intraoperative findings. After the posterior arch at C1 and cranial portion of the C2 lamina were removed and the dura was exposed
Histopathological examination. The tumor cells were found to be spindle cells with no cellular pleomorphism and presence of ganglion cells. Ganglion cells were dissipating throughout the tumor (H&E, ×100).
Postoperative magnetic resonance images. Although bilateral foraminal portions were left, the intracanal tumor was resected completely and spinal cord decompression was sufficient.