Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 59, Num. 3, 2011, pp. 465-466

Neurology India, Vol. 59, No. 3, May-June, 2011, pp. 465-466

Letter to Editor

Giant cervicothoracic ganglioneuroma

Ji Zhang, Jin Li, Rajendra Shrestha, Shu Jiang

Department of Neurosurgery, West China Hospital, Sichuan University, 37 Guo Xue Xiang, Wu Hou District, Chengdu, China

Correspondence Address: Ji Zhang Department of Neurosurgery, West China Hospital, Sichuan University, 37 Guo Xue Xiang, Wu Hou District, Chengdu China zhangjiweijhui@yahoo.com.cn

Date of Submission: 23-Jan-2011
Date of Decision: 16-Feb-2011
Date of Acceptance: 21-Feb-2011

Code Number: ni11133

PMID: 21743186

DOI: 10.4103/0028-3886.82735

Sir,

A 24-year-old woman presented with a 15-year history of left shoulder swelling associated with left upper extremity numbness and pain for eight years. She had felt warmness around the swelling region for three years. These symptoms had gradually worsened over the past few months. Physical examination revealed a well-demarcated, soft and movable mass. Neck movement and all cervical nerves were intact. There was no family history of neurofibromatosis. Computed tomography (CT) scan disclosed pedicular erosion and enlargement of left C6-C7 intervertebral foramina [Figure - 1]. Cervicothoracic magnetic resonance imaging (MRI) revealed a large extramedullary asymmetric dumbbell lesion (dimensions: 91 × 72 × 83 mm) extending outside through the neural foramen of C6-7 into the left paravertebral region of the sternocleidomastoideus, scalenus and supraclavicular fossa [Figure - 2]a. The spinal cord was severely compressed by intraspinal growth of the tumor, and the enhancement was not apparent [Figure - 2]b . Removal of the tumor was approached between the sternocleidomastoideus and the scalenus, combined with a posterior midline C7 hemilaminectomy. During the operation, the lesion was identified as originating from the C7 nerve root and extending to the left paravertebral region through the C6-7 intervertebral foramen. The tumor was totally resected and the C7 nerve root was preserved. Postoperative MRI confirmed complete surgical excision [Figure - 3], and three-dimension CT showed a left C7 vertebral plate defect due to the operation [Figure - 4]. Histopathological features [Figure - 5] were consistent with the diagnosis of ganglioneuroma. The patient recovered well after surgery, and her limb numbness and pain was gradually relieved without any dysfunction of neck movement.

Ganglioneuromas are rare, slow-growing, benign tumors and arise from neural crest cells or sympathetic and peripheral nerves. A solitary ganglioneuroma at the cervicothoracic region arising from the cervical nerve root within the intervertebral foramen is extremely uncommon. ^[1] We could review only ten published cases in the English literature [Table - 1]. ^{[2],[3],[4],[5],[6],[7],[8],[9],[10],[11]} These cases were frequently dumbbell-shaped and more than half of them extended into the spinal canal through one or more intervertebral foramina. Giant ganglioneuroma as seen in our patient has not been reported in the cervical region. Regarding treatment, complete excision is the best option. ^[10] In our patient, despite the large tumor size, complete excision was achieved with favorable results via combined approaches. The long-term prognosis of these tumors is excellent after total tumor excision. ^[12] However, if total dissection of the tumor is associated with the risk of damage to neural and vascular structures, it will be appropriate to do partial decompression of the lesion and relieve the spinal cord compression. Radiotherapy was not taken, given its benign biological course and malignant transformation of the glial component. ^[13]

References

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2.	Bhand AA. Ganglioneuroma of the cervical spine. J Coll Physicians Surg Pak 2005;15:114-6.  Back to cited text no. 2  [PUBMED]
3.	Kyoshima K, Sakai K, Kanaji M, Oikawa S, Kobayashi S, Sato A, et al. Symmetric dumbbell ganglioneuromas of bilateral C2 and C3 roots with intradural extension associated with von Recklinghausen's disease: Case report. Surg Neurol 2004;61:468-73.   Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.	Shephard RH, Sutton D. Dumb-bell ganglioneuromata of the spine with a report of four cases. Br J Surg 1958;45:305-17.  Back to cited text no. 4  [PUBMED]
5.	Sinclair JE, Yang YH. Ganglioneuromata of the spine associated with von Recklinghausen's disease. J Neurosurg 1961;18:115-9.  Back to cited text no. 5
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7.	Maggi G, Dorato P, Trischitta V, Varone A, Civetta F. Cervical dumbbell ganglioneuroma in an eighteen month old child. A case report. J Neurosurg Sci 1995;39:257-60.  Back to cited text no. 7  [PUBMED]
8.	Radulovi DV, Branislav D, Skender-Gazibara MK, Igor NM. Cervical dumbbell ganglioneuroma producing spinal cord compression. Neurol India 2005;53:370-1.  Back to cited text no. 8  [PUBMED]
9.	Tei R, Morimoto T, Miyamoto K, Aketa S, Shimokawara T, Shin Y, et al. Intradural extramedullary ganglioneuroma associated with multiple hamartoma syndrome. Neurol Med Chir (Tokyo) 2007;47:513-5.   Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.	Ugarriza LF, Cabezudo JM, Ramirez JM, Lorenzana LM, Porras LF. Bilateral and symmetric C1-C2 dumbbell ganglioneuromas producing severe spinal cord compression. Surg Neurol 2001;55:228-31.  Back to cited text no. 10  [PUBMED]  [FULLTEXT]
11.	Miyakoshi N, Hongo M, Kasukawa Y, Misawa A, Shimada Y. Bilateral and symmetric C1-C2 dumbbell ganglioneuromas associated with neurofibromatosis type 1 causing severe spinal cord compression. The Spine Journal 2010;10:e11-5.   Back to cited text no. 11
12.	Han PP, Dickman CA. Thoracoscopic resection of thoracic neurogenic tumors. J Neurosurg 2002;96:304-8  Back to cited text no. 12
13.	Califano L, Zupi A, Mangone GM, Long F. Cervical ganglioneuroma: report of a case. Otolaryngol Head Neck Surg 2001;124:115-6.  Back to cited text no. 13  [PUBMED]  [FULLTEXT]

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