Gangliogliomas of the Spinal Cord: Neuroimaging Correlations with Pathology, Controversies in Pathological Diagnosis, and Prognosis

  • Douglas C. MillerEmail author
Part of the Tumors of the Central Nervous System book series (TCNS, volume 6)


Gangliogliomas of the spinal cord are not, in general, different from those in the brain, but there are special problems in their neuroradiological and pathological diagnosis. While often stated to be quite rare, the available literature suggests that these are from 10% to 25% of all intramedullary spinal cord neoplasms in children, and are less frequently found in adults. Their neuropathological diagnosis is highly dependent on adequate biopsies or resections, most often accomplished at centers with pediatric neurosurgeons accustomed to aggressive approaches to intramedullary spinal cord tumors. Neuroradiologically these are intrinsic cord masses which are on average longer, in terms of spinal segments, than other intramedullary tumor types; many are “holocord” tumors. The tumors usually enhance even though most are low grade. The neuropathological diagnosis as in the brain depends on finding a tumor with cells that include both glial cells, generally astrocytic, and large neurons or ganglion cells. Proof that neuron-like cells in a tumor are tumor ganglion cells involves abnormal H&E appearances and immunohistochemical stains, of which the most important is that for synaptophysin. The problems and controversies of interpretation of synaptophysin immunostains in the spinal cord, in view of somewhat similar patterns of immunopositivity on anterior horn cells and tumor ganglion cells, is illustrated and discussed. Other helpful techniques include stains for myelin and for axons. These are low grade tumors with a favorable oncologic prognosis if successfully gross totally excised.


Spinal Cord Ganglion Cell Spinal Cord Neuron Anterior Horn Cell Glioneuronal Tumor 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. Cheung YK, Fung CF, Chan FL, Leong LL (1991) MRI features of spinal ganglioglioma. Clin Imaging 15:109–112PubMedCrossRefGoogle Scholar
  2. Constantini S, Houten J, Miller DC, Freed D, Ozek MM, Rorke LB, Allen JC, Epstein FJ (1996) Intramedullary spinal cord tumors in children under the age of three years. J Neurosurg 85:1036–1043PubMedCrossRefGoogle Scholar
  3. Constantini S, Miller DC, Allen JC, Rorke LB, Freed D, Epstein FJ (2000) Radical excision of intramedullary spinal cord tumors: surgical morbidity and long-term follow-up evaluation in 164 children and young adults. J Neurosurg Spine 93:183–193CrossRefGoogle Scholar
  4. Epstein F (1986) Spinal cord astrocytomas of childhood. In: Symon I (ed) Advances and technical standards in neurosurgery, vol 13. Springer, Vienna, pp 135–169CrossRefGoogle Scholar
  5. Epstein F, Epstein N (1982) Surgical treatment of spinal cord astrocytomas of childhood: a series of 19 patients. J Neurosurg 57:685–689PubMedCrossRefGoogle Scholar
  6. Epstein FJ, Farmer JP, Freed D (1992) Adult intramedullary astrocytomas of the spinal cord. J Neurosurg 77:355–359PubMedCrossRefGoogle Scholar
  7. Hamburger C, Buttner A, Weis S (1997) Ganglioglioma of the spinal cord: report of two rare cases and review of the literature. Neurosurgery 41:1410–1416PubMedCrossRefGoogle Scholar
  8. Lotfinia I, Vahedi P (2009) Intramedullary cervical spinal cord ganglioglioma, review of the literature and therapeutic controversies. Spinal Cord 47:87–90PubMedCrossRefGoogle Scholar
  9. McGirt MJ, Chaichana AL, Atiba A, Attenello F, Woodworth GF, Jallo GI (2007) Neurologic outcome after resection of intramedullary spinal cord tumors in children. Childs Nerv Syst 24:93–97PubMedCrossRefGoogle Scholar
  10. Miller DC (2000) Surgical pathology of intramedullary spinal cord neoplasms. J Neuro Oncol 47:189–194CrossRefGoogle Scholar
  11. Miller DC (2009) Modern surgical neuropathology. Cambridge University Press, CambridgeGoogle Scholar
  12. Miller DC, Paullus WC (2010) Gangliogliomas and other low grade neuronal neoplasms of the central nervous system: diagnosis, treatment, and prognosis. In: Hayat E (ed) Tumors of the central nervous system. Springer, ViennaGoogle Scholar
  13. Miller DC, Koslow MK, Budzilovich GN, Burstein DE (1990) Synaptophysin: a sensitive and specific marker for ganglion cells in central nervous system tumors. Hum Pathol 21:271–276PubMedCrossRefGoogle Scholar
  14. Miller DC, Lang FF, Epstein FJ (1993) Central nervous system gangliogliomas, I: pathology. J Neurosurg 79:859–866PubMedCrossRefGoogle Scholar
  15. Park SH, Chi JG, Cho BK, Wang KC (1993) Spinal cord gangliogliomas in childhood. Pathol Res Pract 189:189–196PubMedCrossRefGoogle Scholar
  16. Park C-K, Chung C-K, Choe G-Y, Wang K-C, Cho B-K, Kim H-J (2000) Intramedullary spinal cord ganglioglioma: a report of five cases. Acta Neurochir 142:547–552CrossRefGoogle Scholar
  17. Patel U, Pinto RS, Miller DC, Handler MS, Rorke LB, Epstein FJ, Kricheff II (1998) MRI of spinal ganglioglioma. AJNR Am J Neuroradiol 19:879–887PubMedGoogle Scholar
  18. Quinn B (1998) Synaptophysin staining in normal brain: importance for diagnosis of ganglioglioma. Am J Surg Pathol 22:550–556PubMedCrossRefGoogle Scholar
  19. Satyarthee GD, Mehta VS, Vaishya S (2004) Ganglioglioma of the spinal cord: report of two cases and review of literature. J Clin Neurosci 11:199–203PubMedCrossRefGoogle Scholar
  20. Shrivastava RK, Epstein FJ, Perin NI, Post KD, Jallo GI (2005) Intramedullary spinal cord tumors in patients older than 50 years of age: management and outcome analysis. J Neurosurg Spine 2:249–255PubMedCrossRefGoogle Scholar
  21. Zhang PJ, Rosenblum MK (1996) Synaptophysin expression in the human spinal cord. Diagnostic implications of an immunohistochemical study. Am J Surg Pathol 20:273–276PubMedCrossRefGoogle Scholar
  22. Zimmerman H (1971) Introduction to tumors of the central nervous system. In: Minkler J (ed) Pathology of the nervous system, vol 2. McGraw-Hill, New York, pp 1947–1951Google Scholar

Copyright information

© Springer Science+Business Media B.V. 2012

Authors and Affiliations

  1. 1.Pathology and Anatomical SciencesThe University of Missouri School of MedicineColumbiaUSA

Personalised recommendations