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Year : 2001  |  Volume : 11  |  Issue : 4  |  Page : 220-221
Cervical ganglioneuroma


3-6-779/6,ST No 14, Himayat Nagar, Hyderabad-500029, India

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How to cite this article:
Moorthy N, Bhaskar G, Venkatakumar N, Sharada V. Cervical ganglioneuroma. Indian J Radiol Imaging 2001;11:220-1

How to cite this URL:
Moorthy N, Bhaskar G, Venkatakumar N, Sharada V. Cervical ganglioneuroma. Indian J Radiol Imaging [serial online] 2001 [cited 2019 Nov 13];11:220-1. Available from: http://www.ijri.org/text.asp?2001/11/4/220/28410
Dear Sir

Ganglioneuromas belong to a rare group of nerve sheath tumors that constitute the intradural extramedullary spinal masses. They are benign spinal tumors that occur either in the spinal cord or peripheral nerve roots. Between 35-45 % of the patients with nerve sheath tumors have neurofibromatosis.

A nineteen-year-old man presented with quadriparesis, inability to walk and bladder disturbance of three months duration. On clinical examination he had 3/5-grade motor power in the upper limbs. The rest of the CNS examination was normal. He has all cutaneous markers of neurofibromatosis. Routine investigations including plain radiography of cervical spine and skull were normal. MRI of the cervical spine and skull were normal. MRI of the cervical spine was performed on the following protocol: FSE T2 and SE T1 in sagittal and GRE T2 and SE T1 AXIAL planes. Multiple dumbbell shaped bilateral extramedullary lesions were seen at C2and C3 levels. The lesions were hypointense on T1 WI and hyperintense on T2 WI images in relation to the cord intensity. The lesions were seen extending outside the spinal canal through the neural foramina. The size of the lesions varied from 1.2x 1.3 x1.2 cm. The cord was compressed on either side at C2 and C3 level. There was no signal change in the cord. The rest of the cord and nerve roots were normal. There were no changes in the cervical vertebrae or intervertebral discs. Paravertebral soft tissues were normal. On surgery, three lobulated well capsulated masses that were purely extradural were excised. The biopsy of the tumor showed nerve fibres, cellular areas consisting of elongated nuclei arranged in short fascicles and diffuse sheets. A number of mature ganglion cells were found.

Nerve sheath tumors can be intradural (70-75%), extradural (15%) or extradural - intradural dumbbell masses (15%). The distribution of the nerve sheath tumors throughout the spine is uniform with a slight lumbar predominance, multiple lesions are reported in 1-55% of patients and are common in neurofibromatosis.

Pain and radiculopathy are the frequent presenting symptoms. Ganglioneuroma is a mature form of neurogenic tumor beside the adrenal. These tumors arise from parasympathetic ganglia along the spine particularly the thorax. They occur in a range of age groups with approximately 50% occurring in patients over 20 years of age. Mostly extradural, ganglioneuromas have an intraspinal component causing neurological symptoms either by cord compression or by involvement of cauda equina.

Plain film findings include pedicle erosion, enlarged neural foramina and paraspinal soft tissue masses. Myelography may show features of intradural or extradural masses.

Depending on the site of the tumor if the lesion is present in a child, and calcification is seen in the paraspinal mass, the diagnosis of ganglioneuroma should always be considered. On CT, ganglioneuromas appear as hypo or slightly hyperdense masses. These tumors have lower attenuation than skeletal muscle due to high amount of lipid within the tumor, interstitial fluid and areas of cystic degeneration. Nearly 75% of these tumors are isointense compared to spinal cord on T1 WI images, 25% are hypointense. Most of them are hyperintense on T2WI images [1] . The non-homogeneous appearance and low to intermediate intensity on T1W1 image and high signal intensity on T2W1 images correspond to areas of cystic degeneration, hemorrhage or necrotic degeneration. All nerve sheath tumors enhance following contrast administration [2] . Ganglioneuroma show gradually increasing enhancement on dynamic MR images. MR allows excellent visualization of the extent of involvement into the neural canal. The ability of MR to show the tumor position in several planes helps in planning surgical removal. Ganglioneuromas histologically show mature ganglion cells, Schwann cells, and nerve fibres and have a distinct whorled pattern [3] . The extradural nerve sheath tumors have to be differentiated from ependymoma, vertebral chordoma, both typically slow growing and extending through and expanding the neural foramina. Approximately 5% of spinal meningiomas are both intra & extradural. Some of the conditions that resemble nerve sheath tumors include conjoined nerve roots, nerve sleeve cysts, ganglion cysts, and foraminal or extraforaminal disc herniations. Some of the Ganglioneuromas (25%) are not truly benign but contain poorly differentiated components including ganglioneuroblastomas, neuroblastomas, pheochromocytoma etc. If a ganglioneuroma has atypical CT or MR features coexistence of a malignant component should be considered ganglionic [2] . Ganglion tumors tend to have an elongated shape and are oriented vertically along the direction of the sympathetic chain, in contradiction to nerve sheath tumors, which have more rounded shape [4] . They also tend to be larger and more frequently have areas of calcification. Recent reports describe rare presentations like ganglioneuroma of adrenal gland associated with pheochromocytoma, rare sites like mandible, trigeminal nerve, retropharyngeal region and cervical dumbell ganglioneuroma in an 18 month-old-child [5].

 
   References Top

1.Li MH, Holtas, Larsson EM. MR imaging of intradural extramedullary tumors. Acta Radiol. 1992; 33: 207-212.   Back to cited text no. 1    
2.Ichikawa T, Ohtomo K, Araki T. Ganglioneuroma : computed tomography and magnetic resonance features Br J Radiol. 1996; 69: 114-21  Back to cited text no. 2    
3.Osborn AG. Tumors, cysts, tumor like lesions of the spine and spinal cord. In: Diagnostic neuroradiology. St. Louis: Mosby, p. 895-899   Back to cited text no. 3    
4.Reed JC, Hallet KK, Feigin DS. Neural tumors of the thorax ; subject review from the AFIP. Radiology. 1978; 126: 9-17.  Back to cited text no. 4    
5.Maggi G, Dorato P, Trischitta V, Varone A, Civetta F. Cervical dumbbell ganglioneuroma in an eighteen month old child: a case report. J.neurosurgery sci. 1995; 39: 257-60  Back to cited text no. 5    

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Correspondence Address:
NLN Moorthy
3-6-779/6,ST No 14, Himayat Nagar, Hyderabad-500029
India
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