ganglioneuroma is the most mature form of neuroblastoma , originating from neural crest cells that normally migrate into the adrenal medulla and sympathetic ganglila . it can arise wherever sympathetic tissue exists and may be seen in the neck , posterior mediastinum , adrenal gland , retroperitoneum , and pelvis . the retroperitoneum and posterior mediastinum are the two most common locations for a ganglioneuroma , followed by the cervical region13 ) . among the cervical gangnlioneuroma , only ten cases producing spinal cord compression have been reported in the english literature19 ) . a 13-year - old boy was referred to our institute and presented with three - day history of right upper extremity weakness associated with neck pain for nearly one month . physical examination demonstrated several soft and movable subcutaneous masses on his posterior neck and we could identify several cafe au lait spots on his trunk . weakness of right elbow flexion ( power , 4/5 ) and positive spurling 's sign at right side were present . there was no upper motor neuron sign , such as , hyperactive deep tendon reflex , ankle clonus , and babinski sign . cervical magnetic resonance imaging ( mri ) showed right side extramedullary dumbbell shaped mass at the c2 - 3 level extending outside through neural foramen . the mass compressing spinal cord was isointense on t1-weighted image and showed slightly increased signal intensity on gadolinium - enhanced t1 weighted imaging ( fig . 1a ) and multiple masses at the pre - vertebral region and subcutaneous posterior neck area ( fig . 1b ) . the patient underwent surgical decompression of the spinal cord in a prone position through a posterior midline incision . the mass , which was significantly indenting the dura , was solid , elastic , less vascularized , and located purely extramedullary . we confirmed that the tumor mass was divided into foraminal and spinal canal portion , so , en block removal of spinal canal portion was available . complete resection of foraminal canal portion of tumor without neural damage was impossible , because adhesions between the mass and dura were evident . ganglioneuroma is rare , differentiated , benign , and slowgrowing tumor arising from the neural crest tissue of the sympathetic nervous system , and is histologically composed of mature schwann cells and ganglion cells with fibrous stroma2 ) . a solitary ganglioneuroma at the cervicothoracic region arising from the cervical nerve root within the intervertebral foramen is extremely uncommon11 ) . in some series , therefore , ganglioneuroma is seldom considered in the differential diagnosis of cervical dumbbell tumors . in 2011 , ji zhang et al.19 ) summarized previously published eleven cases of cervical ganglioneuroma producing spinal cord compression in the english literature , including their own case . a summary of these eleven cases and the present case showed that these lesions are mostly dumbbell shaped except one case and five of them were associated with neurofibromatosis type 1 ( nf-1 ) . an association with nf-1 has been speculated in cases with multiple occurrences , but genetic associations between nf-1 and ganglioneuroma remain unclear4,10 ) . however , more recent studies have shown that the mean age at diagnosis of ganglioneuroma is around 40 to 50 years old5,8,12,15 ) . regarding treatment , surgical decompression should be undertaken as soon as possible when spinal cord compression is present as present case7 ) . ganglioneuromas are usually encapsulated masses with a firm consistency that complete surgical resection is the best option for solitary ganglioneuroma18 ) . however , in cases of multiple tumor masses and tumors with the risk of damage to neural and vascular structure , it will be appropriate to do subtotal or partial removal of the lesion and relieve the spinal cord compression19 ) . prognosis is generally favorable , considering that ganglioneuromas the most differentiated form of neuroblastic tumor have no metastatic potential . but local recurrence , regional lymph node metastasis , and malignant transformation into malignant peripheral nerve sheath tumors have been reported1,3,14,17 ) therefore periodic radiologic surveillance should be performed after resection2 ) . cervical dumbbell ganglioneuromas producing spinal cord compression are extremely rare and could be associated with nf-1 . complete surgical resection is the best option for solitary ganglioneuroma . however , decompression of spinal cord by using subtotal resection of tumor would be acceptable , if total resection is accompanied with risks of neural or vascular damage . we have presented an extremely rare case of nf-1 with dumbbell ganglioneuromas of the cervical spine .
ganglioneuroma is an uncommon benign tumor and arise from neural crest cells or sympathetic and peripheral nerves . 
 a 13-year - old boy was referred to our institute and presented with three - day history of right upper extremity weakness associated with neck pain for nearly one month . 
 magnetic resonance imaging showed right side dumbbell shaped extramedullary lesion at the c2 - 3 level extending outside through neural foramen . 
 paraspinal and posterior neck mass , cafe au lait spot was identified . 
 we performed surgical decompression of the spinal cord by resection of intradural portion mass . 
 histopathological diagnosis was ganglioneuroma .