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Table of Contents    
Year : 2012  |  Volume : 60  |  Issue : 3  |  Page : 348-350

A cervicothoracic intramedullary ganglioneuroblastoma presenting with acute quadriparesis

1 Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, Whitefield, Bangalore, India
2 Department of Pathology, Sri Sathya Sai Institute of Higher Medical Sciences, Whitefield, Bangalore, India

Date of Submission01-Apr-2012
Date of Decision15-Apr-2012
Date of Acceptance14-May-2012
Date of Web Publication14-Jul-2012

Correspondence Address:
Sumit Thakar
Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, Whitefield, Bangalore
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.98539

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How to cite this article:
Thakar S, Ghosal N, Hegde AS. A cervicothoracic intramedullary ganglioneuroblastoma presenting with acute quadriparesis. Neurol India 2012;60:348-50

How to cite this URL:
Thakar S, Ghosal N, Hegde AS. A cervicothoracic intramedullary ganglioneuroblastoma presenting with acute quadriparesis. Neurol India [serial online] 2012 [cited 2021 Dec 5];60:348-50. Available from:


Primary intra-axial central nervous system (CNS) neuroblastic tumors are rare and constitute a subset of primitive neuroectodermal tumors (PNETs), [1],[2],[3] with a variable prognosis. [4] The ganglioneuroblastoma (GNB) constitutes ~10% of these tumors. The sites of predilection include the mediastinum and retroperitoneum. In the spinal canal, this aggressive tumor typically presents as a dumb-bell shaped extradural lesion. Previously, the intramedullary occurrence of a spinal GNB has been reported only thrice. [5],[6],[7]

A 30-year-old man presented with a history of a sudden onset of weakness of all four limbs, numbness in the lower limbs, and bladder incontinence. His general examination was normal. His neurological examination revealed intact cranial nerve function, spastic weakness, quadreparesis (grade 2 - 3), impaired posterior column sensations in the lower limbs, and exaggerated deep tendon reflexes. Magnetic resonance imaging (MRI) of the spine revealed a homogenously contrast-enhancing, intramedullary mass, from C6 to T6 [Figure 1], with associated syringes at both ends: a larger one proximally, extending up to the cervicomedullary junction, and a smaller one distally. There was no evidence of intra-tumoral hemorrhage. Based on these findings, a clinicoradiological diagnosis of acute dissection of an intramedullary tumor-associated syringomyelia was made. He underwent C5 to T7 laminotomy and total excision of the tumor. Intraoperatively, after a midline myelotomy of the swollen cord, a grayish, vascular and suckable tumor was encountered. It had a good plane with the normal cord in most areas. A gross total resection of what seemed to be an intramedullary ependymoma was achieved. Histological examination [Figure 2] revealed a tumor composed of predominantly large cells with ganglionic differentiation dispersed in a densely fibrillar glial stroma with Rosenthal fibers and eosinophilic granular bodies. Vascular hyalinization was prominent. There were foci of perivascular inflammation and microcystic change. There was another component of the tumor that consisted of sheets of small round cells suggesting neuroblastic differentiation. This component had foci of mitotic activity and punctate necrosis. Neurofilament positivity was seen in the cells with ganglionic differentiation, while synaptohpysin was positive in the neuroblastic component. These findings were suggestive of a GNB (World Health Organization grade IV). Postoperatively, the patient's strength improved to grade 4 in all the limbs, after a transient deterioration. He was referred to an oncologist for adjuvant radiation therapy and chemotherapy with ifosfamide and etoposide. At a follow-up visit, three months following surgery, an MRI confirmed no residue or recurrence and evidence of resolving syringomyelia [Figure 3].
Figure 1: (a) T2 weighted MR image, sagittal section of the cervicothoracic spine, showing an intramedullary lesion extending downward from C6. A syrinx is noted extending proximally up to the cervicomedullary junction (b) T2 weighted image (WI), sagittal section of the thoracic spine, showing the lower extent of the lesion and a small, distally located syrinx (c) Post-gadolinium T1 WI, sagittal section of the cervicothoracic spine, showing heterogeneous contrast uptake in the lesion (d) Post-gadolinium T1 WI, axial section at T2, showing the enhancing lesion in an enlarged cord

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Figure 2: Histopathological examination demonstrating: (a) small round cells with hyperchromatic nuclei, scant cytoplasm, cells showing ganglionic differentiation (curved arrow) with (b) clustering of ganglionic cells in a fibrillar glial stroma; (c) High power view of the neuroblastoma component showing mitosis with (Inset) synaptophysin positivity; (d) Neurofilament positivity seen in the cells with ganglionic differentiation. [Hematoxylin and Eosin:(a) (b) (c) ×400] [Avidin Biotin Complex Immunoperoxidase: (c-Inset) ×100; (d) ×400]

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Figure 3: (a) Postoperative T2 weighted MR image, sagittal section of the cervicothoracic spine, showing resolving syringomyelia (b) No contrast enhancing residue / recurrence noted in the contrast T1 WI, sagittal section through the cervicothoracic spine

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An acute presentation of a spinal intramedullary tumor is commonly ascribed to intra-tumoral hemorrhage or ischemia. Literature review reveals one previous such case [8] where an acute dissection of a tumor-associated syrinx was responsible for an abrupt onset of symptoms. Amplification of various pathogenetic factors, both from within the cord and from without, have been proposed for such an occurrence. In our patient, it is conceivable that the rapidly growing malignant tumor rendered fluctuancy to the hydrodynamics of the cord and syrinx. Mild or transient changes in posture or spinal venous pressures in such a pathophysiological setting would have induced acute dissection of the syrinx. The clinicoradiological presentation of an intramedullary GNB is non-specific, and the diagnosis is based entirely on an immunohistological examination. The tumor behavior carries a high-risk label, like for other CNS PNETs arising outside the posterior fossa. Treatment of a spinal GNB includes resection followed by adjuvant radiotherapy (local treatment and / or craniospinal radiation) and chemotherapy, the optimal dose of which is undetermined at present. Close follow-up is thus prudent, even in radically resected cases like ours.

  References Top

1.Bennett JP Jr, Rubinstein LJ. The biological behavior of primary cerebral neuroblastoma: A reappraisal of the clinical course in a series of 70 cases. Ann Neurol 1984;16:21-7.  Back to cited text no. 1
2.Berger MS, Edwards MS, Wara WM, Levin VA, Wilson CB. Primary cerebral neuroblastoma. Long-term follow-up review and therapeutic guidelines. J Neurosurg 1983;59:418-23.  Back to cited text no. 2
3.Horten BC, Rubinstein LJ. Primary cerebral neuroblastoma. A clinicopathological study of 35 cases. Brain 1976;99:735-56.  Back to cited text no. 3
4.Shimada H, Ambros IM, Dehner LP, Hata J, Joshi VV, Roald B, et al. The international neuroblastoma pathology classification (the Shimada system). Cancer 1999;86:364-72.  Back to cited text no. 4
5.Miele WR, Pfannl R, Kryzanski JT. Primary holocord ganglioneuroblastoma: Case report. J Neurosurg Spine 2011;15:457-63.  Back to cited text no. 5
6.Sibilla L, Martelli A, Farina L, Uggetti C, Zappoli F, Sessa F, et al. Ganglioneuroblastoma of the spinal cord. AJNR Am J Neuroradiol 1995;16:875-7.  Back to cited text no. 6
7.Singh SK, Srivastava C, Ojha BK, Chandra A, Husain N. Ganglioneuroblastoma of the conus: A rare and aggressive tumor. Neurol India 2010;58:966-8.  Back to cited text no. 7
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8.Amato VG, Assietti R, Morosi M, Arienta C. Acute brainstem dissection of syringomyelia associated with cervical intramedullary neurinoma. Neurosurg Rev 2005;28:163-7.  Back to cited text no. 8


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