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LETTER TO EDITOR
Year : 2013  |  Volume : 2  |  Issue : 4  |  Page : 226

Dumbbell-shaped neurofibroma of the upper thoracic spine: A case report


1 Department of Neurosurgery, MGIMS, Sevagram, Wardha (M.S.), India
2 Department of Orthopaedics and Traumatology, MGIMS, Sevagram, Wardha (M.S.), India
3 Department of Otorhinolaryngology, MGIMS, Sevagram, Wardha (M.S.), India

Date of Web Publication17-Oct-2013

Correspondence Address:
Manoj Kumar
Department of Orthopaedics and Traumatology, MGIMS, Sevagram, Wardha (M.S.)
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-330X.119925

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How to cite this article:
Kumar S A, Kumar M, Malgonde M. Dumbbell-shaped neurofibroma of the upper thoracic spine: A case report. South Asian J Cancer 2013;2:226

How to cite this URL:
Kumar S A, Kumar M, Malgonde M. Dumbbell-shaped neurofibroma of the upper thoracic spine: A case report. South Asian J Cancer [serial online] 2013 [cited 2019 Dec 14];2:226. Available from: http://journal.sajc.org/text.asp?2013/2/4/226/119925

Dear Editor,

Dumbbell-shaped thoracic neurofibroma represents a distinct type of tumor and can involve both the spinal canal and the posterior thoracic cavity. Dumbbell tumors are those with an intraspinal and paraspinal component, connected through a frequently enlarged and eroded intervertebral foramen. Most dumbbell tumors are located in the thoracic spine. The extraspinal tumor extension is usually larger than the intraspinal tumor part, but the intraspinal tumor component commonly causes the typical symptoms of local pain and symptoms from spinal cord compression in the thoracic spine. The differential diagnosis of dumbbell shaped foraminal masses include neurogenic tumors (schwannoma, neurofibroma) and meningiomas. Asymptomatic cases require no further treatment, but symptomatic cases justify surgical treatment in the form of total removal of neurofibroma. Posterior laminectomy with unilateral facetectomy allows single-stage resection of dumbbell neurofibromas with significant intraspinal and paraspinal components. Here, we report the case of 34-year-old female patient with dumbbell neurofibroma in the upper thoracic region.

A 34-year-old female was admitted to the hospital with complaints of pain over left scapular region and tingling over both lower limbs since 2 years and progressive weakness of both lower limbs for 3 months. She was bedridden for the past 30 days. Clinical examination did not show any neurocutaneous markers. Neurological examination revealed spastic paraparesis, more pronounced distally, hypoesthesia below D3 dermatome and hyperactive deep tendon reflexes.

T1-weighted magnetic resonance imaging (MRI) of the dorsal spine showed isointense signal and hyperintense signal on T2-weighted images, with patchy necrotic area. The intradural component was located in the left lateral thecal sac at D3 level, extending from D2-3 to D3-4 level with large extraforaminal component in the posterior mediastinum seen traversing through widened D2-3 neural foramina. There was scalloping of the left lateral posterior D3 vertebral body, adjacent pedicle and posterior end of left rib. There was compression and displacement of the cord toward the right side at D3 level by intradural component of mass [Figure 1].
Figure 1: T1-weighted and T2-weighted magnetic resonance imaging of the thoracic dumbbell neurofibroma with extension to posterior mediastinum, the lesion being isointense on T1 sequence and hyperintense on T2 sequence

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The cord showed T2 hyperintense signal at the same level suggestive of cord edema. The extraforaminal component of the mass was extending from D2 vertebral level up to mid D5 vertebral level. The lesion was measuring 7.4 cm × 6.3 cm × 6.1 cm in size [Figure 2].
Figure 2: T1-weighted and T2-weighted magnetic resonance imaging of the thoracic dumbbell neurofibroma with extension to posterior mediastinum, the lesion being isointense on T1 sequence and hyperintense on T2 sequence

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The patient was operated using a posterior approach. Laminectomy of D2-D3 vertebrae with near total excision of dumbbell tumor from intraspinal region and posterior mediastinum was done. Capsule of the tumor in the posterior mediastinum was left behind as it was adherent to pleura. Since the case was considered as a neurofibroma, there was a possibility of compression of spinal cord by the intradural component, but no intradural extramedullary tumor extension was noted after opening the dura during surgery. Histopathological examination of resected specimen confirmed the diagnosis of neurofibroma.

Post-operatively physiotherapy and rehabilitation was given. The patient made an uneventful recovery with subsequent improvement in her neurological status. At the 2 month follow-up neurological examination showed 4+/5 power in both lower limbs and pain over shoulder was completely relieved. The patient wass able to walk without support and could perform routine activities.

A neurofibroma in the spinal canal, invading the peripheral segment of the nerve by extending out of the intervertebral foramen and presenting itself with a dumbbell tumor is quite uncommon. Of the paraspinal neurofibromas, 72% were with intradural extramedullary localization, whereas 14% were with extradural, 13% were with dumbbell formation and 1% was with intramedullary localizations. [2],[9] The extraspinal part is usually larger than the intraspinal part in dumbbell neurofiboma. [7] Tumor may attain massive dimensions, can be round to oval, lobulated and show cystic degeneration.

Dumbbell formation is important due to the attachment of the extramedullary part to the surrounding tissues. Local compression of the spinal cord results in the clinical findings. Common presentations include radicular pain and dysesthesia along with progressive motor weakness. [3],[5]

Direct radiographs are sufficient to establish the diagnosis in 50%of the cases. The most frequent findings on direct radiograph are pedicle erosion and vertebral body scalloping. Regular expansion of the interpedicular distance and intervertebral foramen may directly indicate the presence of the dumbbell tumor. MRI is highly sensitive and specific in detecting the disease, determining the accompanying pathologies and following the development of complications. Dumbbell neurofibromas appear as iso- or hyperintense to the spinal cord on T1-weighted images and give hyperintense signal on T2-weighted images. Regular enhancement is seen upon gadolinium administration in dumbbell neurofibroma. [1],[8]

Asymptomatic cases require no treatment while symptomatic cases need surgical intervention. Majority of the nerve fibers are entrapped by tumoral tissue in dumbbell neurofibroma cases as was the case in our patient as well. It is impossible to remove the tumor without sacrificing the nerve root and aggressive surgery may result in severe neurological deficits. [3],[4] Thus, partial resection should be preferred in dumbbell neurofibroma cases that cause compression of the spinal cord. As the aim of partial resection is to relieve the symptoms, the extent of surgical treatment is based upon the clinical picture of the patient. Decompressive surgery is a partial resection that carries the risk of recurrence and repeat surgery may be needed. [3],[5]

Dumbbell tumors with significant dissemination into the paraspinal region may require complex spinal exposure. Although two-stage operations may be performed to manage the intraspinal and paraspinal components separately, a single-stage procedure is preferable. [6],[10],[11],[12],[13],[14] Our patient presented with myelopathic findings and therefore decompressive excision of the tumor was planned. In our patient, near total excision of foraminal and extradural component including extension into the posterior mediastinum carried out using a single stage posterior approach with posterolateral extension. Tumor capsule was left intact in the mediastinum because it was adherent to pleura. Intraoperatively, dural exploration was performed for the intradural extramedullary component, but no tumor extension was found. Prognosis is excellent after the surgical resection. Recurrence is very rare subsequent to total excision. Recurrence after 3 years was noted in one of 66 paraspinal neurofibroma patients who were treated by Levy et al. [3]

The most significant feature of dumbbell neurofibromas is the adhesion of the tumor to the surrounding environment by enlarging the foramen and projecting outward from the spinal canal. [3],[5] Dumbbell neurofibroma may present clinically as paraparesis or paraplegia (compressive myelopathy) even without intradural extramedullary extension as noted in our case. The goal of surgery is total removal of the tumor. Although a variety of surgical approaches for these lesions is available, most thoracic spine dumbbell tumors can be effectively managed with a single-stage posterior approach with posterolateral extension (single posterior midline approach with laminectomy and costotransversectomy), which is easy to perform, secure and less invasive and was well-tolerated by the patient. [3],[7],[13],[14]

 
  References Top

1.Khong PL, Goh WH, Wong VC, Fung CW, Ooi GC. MR imaging of spinal tumors in children with neurofibromatosis 1. AJR Am J Roentgenol 2003;180:413-7.  Back to cited text no. 1
    
2.Lee M, Rezai AR, Freed D, Epstein FJ. Intramedullary spinal cord tumors in neurofibromatosis. Neurosurgery 1996;38:32-7.  Back to cited text no. 2
    
3.Levy WJ, Latchaw J, Hahn JF, Sawhny B, Bay J, Dohn DF. Spinal neurofibromas: A report of 66 cases and a comparison with meningiomas. Neurosurgery 1986;18:331-4.  Back to cited text no. 3
    
4.McCormick PC. Anatomic principles of intradural spinal surgery. Clin Neurosurg 1994;41:204-23.  Back to cited text no. 4
    
5.McCormick PC, Post KD, Stein BM. Intradural extramedullary tumors in adults. Neurosurg Clin N Am 1990;1:591-608.  Back to cited text no. 5
    
6.Schultheiss R, Gullotta G. Resection of relevant nerve roots in surgery of spinal neurinomas without persisting neurological deficit. Acta Neurochir (Wien) 1993;122:91-6.  Back to cited text no. 6
    
7.Seppälä MT, Haltia MJ, Sankila RJ, Jääskeläinen JE, Heiskanen O. Long-term outcome after removal of spinal neurofibroma. J Neurosurg 1995;82:572-7.  Back to cited text no. 7
    
8.Shu HH, Mirowitz SA, Wippold FJ 2 nd . Neurofibromatosis: MR imaging findings involving the head and spine. AJR Am J Roentgenol 1993;160:159-64.  Back to cited text no. 8
    
9.Yagi T, Ohata K, Haque M, Hakuba A. Intramedullary spinal cord tumour associated with neurofibromatosis type 1. Acta Neurochir (Wien) 1997;139:1055-60.  Back to cited text no. 9
    
10.Sarica FB, Tufan K, Çekinmez M, Erdoðan B, ªen O. Dumbbell-shaped neurofibroma of the upper cervical spine: A case report. J Nerv Sys Surg 2008;13:190-4.  Back to cited text no. 10
    
11.Citow JS, Macdonald RL, Ferguson MK. Combined laminectomy and thoracoscopic resection of a dumbbell neurofibroma: Technical case report. Neurosurgery 1999;45:1263-5.  Back to cited text no. 11
    
12.Agrawal A, Srivastava S, Joharapurkar SR, Gharde P, Ubeja G. Single stage complete excision of large thoracic dumbbell schwannoma by modified posterior approach. Surg Neurol 2008;70:432-6.  Back to cited text no. 12
    
13.Payer M, Radovanovic I, Jost G. Resection of thoracic dumbbell neurinomas: Single postero-lateral approach or combined posterior and transthoracic approach? J Clin Neurosci 2006;13:690-3.  Back to cited text no. 13
    
14.J. Gonzalez-Cruz, A. Nanda: Dumbbell Shaped Thoracic Spine Cavernous Hemangioma: A Case Report and Review of the Literature. The Internet Journal of Neurosurgery. 2006;3.  Back to cited text no. 14
    


    Figures

  [Figure 1], [Figure 2]


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