Abstract
Ganglioneuroma （GN） is a rare benign neural tumor that is thought to arise from the ganglia of the sympathetic chain or parasympathetic nervous system. A 3-year-old boy presented with tumors in the adrenal gland and the right orbit, and was diagnosed with neuroblastoma （NB） stage Ⅳ After chemotherapy, the tumors were resected and the pathological diagnoses of ganglioneuroblastoma （GNB） in the adrenal gland and GN in the orbit were made. The tumor relapsed at the intracranial dura mater 21 years after the initial diagnosis and was diagnosed as GN based on a biopsy sample. The GN in the mandible was detected in the left mandible 24 years later and in the right mandible 27 years later, and was resected. Histopathologically, the tumor consisted of mature ganglion cells. Furthermore, immunohistochemistry showed that the tumor cells were immunoreactive for vimentin, S-100, neurofilament, glial fibrillary acidic protein （GFAP）, and synaptophysin, while they were non-reactive for α-smooth muscle actin （α-SMA） and cytokeratin AE1/AE3. The Ki-67 labeling index （LI） was 1％. Finally, this led to a diagnosis of GN. This case is extremely rare in that GN was found in the bilateral mandible. This is a very rare case of metastatic NB of the adrenal gland that developed in the bilateral mandible after a long period.
