Pediatric Mixed Glioneuronal Tumors in the Spinal Cord

  • Ravi RaghavanEmail author
Part of the Tumors of the Central Nervous System book series (TCNS, volume 6)


This chapter attempts to bring together what is known about an emerging group of mixed tumors in the pediatric spinal cord that display both astrocytic and neuronal elements, and are difficult to classify. Only a handful of cases have been adequately documented in the literature (Poliani et al., Neuropathology 29:574–578, 2009; Psarros et al., J Neurosurg 103(Suppl):184–190, 2005; Rickert et al., Acta Neuropathol 112:231–233, 2006; Scholz et al., Acta Neuropathol 117:591–593, 2009; Syed et al., Acta Neuropathol 111:53–55, 2006), and are generally known as mixed glioneuronal tumors (GNT). The family members of this group of neoplasms are discussed under three broad groups – spinal GNTs, spinal gangliogliomas, and unclassified entities. Emphasis is given to the unique ‘spinal GNTs’ in this chapter, and these are tentatively classified into two types, a more easily identifiable variety with distinct neuronal rosettes (‘Type 1’), and a less structured variant designated as ‘Type 2’. Bulk of the documented lesions fall into the Type 1 category, based on their initial presentation and histology. The ‘spinal gangliogliomas’ are discussed only briefly, being similar in pathology and behavior to their counterparts in other parts of the brain. The ‘unclassifiable’ group is an enigma, and awaits further multicenter collaborative studies for accurate categorization. The glioneuronal tumors in the spinal cord behave like low-grade neoplasms in most instances, with rare exceptions where prognosis can be unpredictable, and can lead to metastases, further morbidity, and grave outcomes.


Glioneuronal tumor Pediatric Spinal 



I am grateful to Professor Arie Perry, neuropathologist, Washington University Medical Center, St LouIs, MO, and Tarik Tihan, neuropathologist, University of San Francisco Medical Center, San Francisco, CA, for their valuable suggestions and help with this write-up.


  1. Anan M, Inoue R, Ishii K, Abe T, Fujiki M, Kobayashi H, Goya T, Nakazato Y (2009) A rosette-forming glioneuronal tumor of the spinal cord: the first case of a rosette-forming glioneuronal tumor originating from the spinal cord. Hum Pathol 40:898–901PubMedCrossRefGoogle Scholar
  2. Burger PC, Scheithauer BW, Vogel FS (2002) Surgical pathology of the nervous system and its coverings. Churchill Livingston, Philadelphia, pp 264–292Google Scholar
  3. Cenacchi G, Giangaspero F (2004) Emerging tumor entities and variants of CNS neoplasms. J Neuropathol Exp Neurol 63:185–192PubMedGoogle Scholar
  4. Costa J, Ruivo J, Miguéns J, Pimentel J (2006) Ganglioglioma of conus medullaris. Acta Neurochir (Wien) 148:977–980CrossRefGoogle Scholar
  5. Edgar MA, Rosenblum MK (2007) Mixed glioneuronal tumors: recently described entities. Arch Pathol Lab Med 131:228–233PubMedGoogle Scholar
  6. Harris BT, Horoupian DS (2000) Spinal cord glioneuronal tumor with rosetted neuropil islands and meningeal dissemination: a case report. Acta Neuropathol 100:575–579PubMedCrossRefGoogle Scholar
  7. Jallo GI, Freed D, Epstein FJ (2004) Spinal cord gangliogliomas: a review of 56 patients. J Neurooncol 68:71–77PubMedCrossRefGoogle Scholar
  8. Komori T, Scheithauer BW, Anthony DC, Rosenblum MK, McLendon RE, Scott RM, Okazaki H, Kobayashi M (1998) Papillary glioneuronal tumor: a new variant of mixed neuronal-glial neoplasm. Am J Surg Pathol 22:1171–1183PubMedCrossRefGoogle Scholar
  9. McLendon RE, Provenzale J (2002) Glioneuronal tumors of the central nervous system. Brain Tumor Pathol 19:51–58PubMedCrossRefGoogle Scholar
  10. Perilongo G, Gardiman M, Bisaglia L, Rigobello L, Calderone M, Battistella A, Burnelli R, Giangaspero F (2002) Spinal low-grade neoplasms with extensive leptomeningeal dissemination in children. Childs Nerv Syst 18:505–512PubMedCrossRefGoogle Scholar
  11. Perry A, Scheithauer BW, Macaulay RJB, Raffel C, Roth KA, Kros JM (2002) Oligodendrogliomas with neurocytic differentiation. A report of four cases with diagnostic and histogenetic implications. J Neuropathol Exp Neurol 61:947–955PubMedGoogle Scholar
  12. Phi JH, Park SH, Chung CK, Wang KC, Cho BK, Kim SK (2009) Atypical cell clusters expressing both neuronal and oligodendrocytic markers: novel histological pattern of glioneuronal tumors? Pathol Int 59:735–743PubMedCrossRefGoogle Scholar
  13. Poliani PL, Sperli D, Valentini S, Armentano A, Bercich L, Bonetti MF, Corriero G, Brisigotti M, Quattrone A, Lanza PL (2009) Spinal glioneuronal tumor with neuropil-like islands and meningeal dissemination: histopathological and radiological study of a pediatric case. Neuropathology 29:574–578PubMedCrossRefGoogle Scholar
  14. Psarros TG, Swift D, Mulne AF, Burns DK (2005) Neurocytoma-like neoplasm of the thoracic spine in a 15-month-old child presenting with diffuse lep­tomeningeal dissemination and communicating hydrocephalus. Case report. J Neurosurg 103(Suppl):184–190PubMedGoogle Scholar
  15. Rickert CH, Jasper M, Sepehrnia A, Jeibmann A (2006) Rosetted glioneuronal tumor of the spine: clinical, histological and cytogenetic data. Acta Neuropathol 112:231–233PubMedCrossRefGoogle Scholar
  16. Scholz M, Hoischen A, Radlwimmer B, Weber RG, Harders A, Reifenberger G, Riemenschneider MJ (2009) Rosetted glioneuronal tumor of the spine with overtly anaplastic histological features. Acta Neuropathol 117:591–593PubMedCrossRefGoogle Scholar
  17. Syed S, Rajaram V, Leonard JR, Perry A, Raghavan R (2006) Mixed glioneuronal tumors of the spinal cord in two children. Acta Neuropathol 111:53–55PubMedCrossRefGoogle Scholar
  18. Teo JG, Gultekin SH, Bilsky M, Gutin P, Rosenblum MK (1999) A distinctive glioneuronal tumor of the adult cerebrum with neuropil-like (including “rosetted”) islands: report of 4 cases. Am J Surg Pathol 23:502–510PubMedCrossRefGoogle Scholar
  19. Tihan T, Brat TJ, Goldwater PT, Burger PC (1999) Glioneuronal tumors with malignant histological features. J Neuropathol Exp Neurol 58:509CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media B.V. 2012

Authors and Affiliations

  1. 1.Anatomy & NeurosurgeryLoma Linda University Medical CenterLoma LindaUSA

Personalised recommendations