Cervical Symmetric Dumbbell Ganglioneuromas Causing Severe Paresis
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.\nMagnetic resonance imaging (MRI) demonstrated bilateral asymmetric masses at the C1/2 level. T1-weighted images exhibited hypointensity. T2-weighted images revealed heterogeneous hyperintensity (). Contrast-enhanced T1-weighted images showed enhancement in the tumor parenchyma (). Computed tomography myelogram showed spinal cord compression bilaterally at C1/2. Because the progression of the paralysis was rapid and the patient hoped for a palliative surgical procedure, we performed emergency operation to remove the intracanalar tumor and decompress the spinal cord.\nAfter the posterior arch at C1 and cranial portion of the C2 lamina was removed and the dura was exposed (), extradural bilateral tumor masses at C1/2 were identifiable (). Tumors surface were smooth with capsules. Firstly the extradural tumor was partially resected and the debulking effect obtained. Bilateral C2 dorsal roots were swollen, with cancerization and connected intradural portions. After durotomy, bilateral symmetric tumors originating from bilateral C2 dorsal root were identified (). These were partially adherent to the dura. The intradural portions of the tumors were totally resected piece by piece. Consequently, C2 bilateral dorsal roots were sacrificed. Subtotal resection for the bilateral extradural portions was then carried out. It was confirmed that that intracanalar portions were totally resected and the emergency operation was thus terminated, because radical resection for the extracanalar portions, which would have required dissection of the tumor from vertebral arteries, was not deemed appropriate given the patient's relatively poor general condition. Finally, we performed duraplasty with artificial dura mater to extend the intradural space.\nHistopathological 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 (). The tumors were thus diagnosed as ganglioneuromas.\nAfter 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 (). He was followed up with MRI at three month intervals. Two years postoperatively, there was no recurrence intracanally and no increase in the foraminal portions. Taking into account his poor general condition, including cancerous ascites due to the peritoneal dissemination of past gastric cancer, we are not planning to perform an additional operation as long as his improvement in paresis is maintained.