Primary holocord ganglioneuroblastoma

Case report

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The authors present a case of extensive primary intramedullary spinal CNS ganglioneuroblastoma (GNB) in a 23-year-old man. Central nervous system GNB is a poorly differentiated neuroepithelial tumor composed of neuroblasts and differentiated ganglion cells, and these lesions are extremely uncommon. Most previously reported primary intraaxial neuroblastic tumors were described in the brain. There has been only one other report of primary spinal cord CNS GNB published to date; the clinical course and prognosis for primary spinal cord tumors of this type are unknown. Similar tumor types demonstrate poor prognoses.

This 23-year-old man presented after 9 months of progressive myelopathy. Admission MR imaging showed an intraaxial enhancing mass extending from C-3 to the conus medullaris, with a holocord appearance in several areas. Due to the tumor size, operative intervention was initially limited to biopsy sampling. Chemotherapy resulted in histological maturation, but initial tumor regression was temporary. The patient suffered progressive quadriparesis, and neuroimaging demonstrated slow enlargement of the tumor and an associated syrinx. Nineteen months after diagnosis, the tumor was excised to gross-total resection in a 2-stage operation. One year following resection, the patient had no radiographic recurrence and was functional in a wheelchair with minimal paresis in the upper extremities. This case represents the most extensive example of primary spinal intramedullary CNS GNB reported to date. Holocord tumors present a significant challenge to the neurosurgeon, and resection bears substantial risk of morbidity. In spinal cord CNS GNB, chemotherapy followed by complete resection may be the most effective means of tumor control.

Abbreviations used in this paper: GNB = ganglioneuroblastoma; MEP = motor evoked potential; NB = neuroblastoma; PNET = primitive neuroectodermal tumor.

Article Information

Address correspondence to: James T. Kryzanski, M.D., Department of Neurosurgery, Tufts Medical Center, 800 Washington Street, Boston, Massachusetts 02111. email: jkryzanski@tuftsmedicalcenter.org.

Please include this information when citing this paper: published online July 8, 2011; DOI: 10.3171/2011.5.SPINE10563.

© AANS, except where prohibited by US copyright law.

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Figures

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    Admission MR images. Sagittal T2-weighted (A) and T1-weighted (B) MR images of the spine obtained after administration of contrast material. A uniformly T1-weighted, contrast-enhancing intramedullary infiltrative mass was demonstrated extending from the level of the C-3 vertebral body to the conus medullaris. There were 2 foci of syringomyelia at the cervicomedullary junction and proximal cervical cord. The caudal aspect of the tumor has a cystic appearance.

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    Photomicrographs showing the tumor's histopathological features. A: Photomicrograph of a smear showing small, round, blue cells and a mitotic figure (asterisk). B: Medium magnification of a section of the original tumor showing a combination of markedly hypercellular areas (upper area of panel) composed of neuroblasts and large dysplastic ganglion cells (asterisks, lower area of panel). H & E, original magnification × 400.

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    Photomicrographs showing the tumor's histopathological features. A: Section showing composite tissue; islands of neuropils with surrounding small cells that demonstrate neurocytic differentiation. B: Section showing a dense fibrillary background with numerous, densely eosinophilic, Rosenthal fibers. C: The islands of neuropils are highlighted by synaptophysin stain. D: The small, mature, neurocytic cells are stained with NeuN, a marker of neuronal differentiation. H & E (A and B), synaptophysin (C), and NeuN (D). Original magnification × 200 (A, C, and D) and × 400 (B).

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    Sagittal T2-weighted (A) and T1-weighted (B) MR images obtained at follow-up 13 months after tumor resection, after administration of contrast material. There is diffuse atrophy of the spinal cord extending from the C-5 level caudally throughout the thoracic spine. There has been interval resolution of the cervical syrinx, with only trace T2 signal remaining in the central canal at the C-4 level.

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