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Oncology of CNS Tumors pp 195–209Cite as

Glioneuronal Tumors

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Abstract

Neuronal and mixed neuronal–glial tumors are thought to arise from neuroepithelial cells. According to the 2007 WHO classification, this group of tumors comprises ganglioglioma and gangliocytoma, desmoplastic infantile astrocytoma (DIA) and ganglioglioma, dys-plastic cerebellar gangliocytoma (Lhermitte–Duclos disease), dysembryoplastic neuroepithelial tumor (DNT), central neurocytoma, cerebellar liponeurocytoma (CLN), paraganglioma of the cauda equina (PCE), and the more recently recognized subtypes papillary glion-euronal tumor, rosette-forming glioneural tumor of the fourth ventricle and extraventricular neurocytoma [31]. These tumors are composed of cells with a neuronal differentiation, sometimes accompanied by a second cellular component with a glial phenotype. In general, the cells of both lineages are well differentiated. Some of these tumors may be associated with cortical dysplasias. Neuronal and mixed neuronal–glial tumors usually carry a favorable prognosis.

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References

  1. Aker F V, Ozkara S Eren P, Peker O, Armagan S, Hakan T. (2005) Cerebellar liponeurocytoma/lipidized medulloblas-toma. J Neurooncol 71:53–59

    Article  PubMed  Google Scholar 

  2. Alkadhi H, Keller M, Brandner S, Yonekawa Y, Kollias SS. (2001) Neuroimaging of cerebellar liponeurocytoma. Case report. J Neurosurg 95:324–331

    Article  PubMed  CAS  Google Scholar 

  3. Amemiya S, Shibahara J, Aoki S, Takao H, Ohtomo K. (2008) Recently established entities of central nervous system tumors: review of radiological findings. J Comput Assist Tomogr 32:279–285

    Article  PubMed  Google Scholar 

  4. Anderson RC, Elder JB, Parsa AT, Issacson SR, Sisti MB. (2001) Radiosurgery for the treatment of recurrent central neurocytoma. Neurosurgery 48:1231–1237

    PubMed  CAS  Google Scholar 

  5. Bachli H, Avoledo P, Gratzl O, Tolnay M. (2003) Therapeutic strategies and management of desmoplastic infantile gan-glioglioma: two case reports and literature overview. Childs Nerv Syst 29:359–366

    Google Scholar 

  6. Becker AJ, Wiestler OD, Figarella-Branger D, Blümcke I. (2007) Ganglioglioma and gangliocytoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, 4th ed. IARC Press, Lyon, pp. 103–105

    Google Scholar 

  7. Bertalanffy A, Roesseler K, Koperek O, Gelpi E, Prayer D, Knosp E. (2005) Recurrent central neurocytomas. Cancer 104:135–142

    Article  PubMed  Google Scholar 

  8. Blümcke I, Wiestler OD. (2002) Gangliogliomas: an intriguing tumor entity associated with focal epilepsies. J Neuropathol Exp Neurol 61:575–584

    PubMed  Google Scholar 

  9. Brat DJ, Scheithauer BW, Eberhart CG, Burger PC. (2001) Extraventricular neurocytomas: pathologic features and clinical outcome. Am J Surg Pathol 25:1252–1260

    Article  PubMed  CAS  Google Scholar 

  10. Brat DJ, VanDenberg SR, Figarella-Branger D, Taratuto AL. (2007) Desmoplastic infantile astrocytoma and ganglio-glioma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, 4th ed. IARC Press, Lyon, pp. 96–98

    Google Scholar 

  11. Choi YH, Kim IO, Cheon JE, Kim WS, Yeon KM, Wang KC, Cho BK, Chi JG. (2001) Gangliocytoma of the spinal cord: a case report. Pediatr Radiol 31:377–380

    Article  PubMed  CAS  Google Scholar 

  12. Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, et al (1988) Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Neurosurgery 23:545–556

    Article  PubMed  CAS  Google Scholar 

  13. Daumas-Duport C, Pietsch T, Hawkins C, Shankar SK. (2007) Dysembryoplastic neuroepithelial tumour. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system. 4th ed. IARC Press, Lyon, pp. 99–102

    Google Scholar 

  14. Eberhart CG, Wiestler OD, Eng C. (2007) Cowden disease and dysplastic gangliocytoma of the cerebellum/Lhermitte-Duclos disease. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, 4th ed. IARC Press, Lyon, pp. 226–228

    Google Scholar 

  15. Figarella-Branger D, Söylemezoglu F, Burger PC. (2007) Central neurocytoma and extraventricular neurocytoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, 4th ed. IARC Press, Lyon, pp. 106–109

    Google Scholar 

  16. Geddes JF, Jansen GH, Robinson SF, Gomori E, Holton JL, Monson JP, Besser GM, Revesz T. (2000) “Gangliocytomas” of the pituitary: a heterogeneous group of lesions with differing histogenesis. Am J Surg Pathol 24:607–613

    Article  PubMed  CAS  Google Scholar 

  17. Gelabert-Gonzalez M. (2005) Paragangliomas of the lumbar region. J Neurosurg Spine 2:354–365

    Article  PubMed  Google Scholar 

  18. Hainfellner JA, Scheithauer BW, Gianspero, Rosenblum MK. (2007) Rosette-forming tumour of the fourth ventricle. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, 4th ed. IARC Press, Lyon, pp. 115–116

    Google Scholar 

  19. Hoving EW, Kros JM, Groninger E, den Dunnen WF. (2008) Desmoplastic infantile ganglioglioma with a malignant course. J Neurosurg Pediatrics 1:95–98

    Article  Google Scholar 

  20. Im SH, Chung CK, Cho BK, Lee SK. (2002) Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome. J Neurooncol 57:59–66

    Article  PubMed  Google Scholar 

  21. Jankinson MD, Bosma JJ, Du Plessis D, Ohgaki H, Kleihues P, Warnke P, Rainov NG. (2003) Cerebellar liponeurocytoma with an unusually aggressive clinical course: case report. Neurosurgery 53:1425–1427

    Article  Google Scholar 

  22. Johnson JHJ, Hariharan S, Berman J, Sutton LN, Rokke LB, Molloy P, Phillips PC. (1997) Clinical outcome of pediatric gangliogliomas: ninety-nine cases over 20 years. Pediatr Neurosurg 27:203–207

    Article  PubMed  Google Scholar 

  23. Kleihues P, Chimelli L, Giangaspero F, Ohgaki H. (2007) Cerebellar liponeurocytoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, 4th ed. IARC Press, Lyon, pp. 110–112

    Google Scholar 

  24. Komori T, Scheithauer BW, Anthony DC, Rosenblum MK, McLendon RE, Scott RM, Okazaki H, Kobayashi M. (1998) Papillary glioneuronal tumour: new variant of mixed neu-ronal–glial neoplasm. Am J Surg Pathol 22:1171–1183

    Article  PubMed  CAS  Google Scholar 

  25. Komori T, Scheithauer BW, Hirose T. (2002) A rosette-forming glioneuronal tumor of the fourth ventricle: infraten-torial form of dysembryoplastic neuroepithelial tumor?. Am J Surg Pathol 26:582–591

    Article  PubMed  Google Scholar 

  26. Koerbel A, Prevedello DM, Tatsui CE, Pellegrino L, Hanel RA, Bleggi-Torres LF, Araujo JC. (2003) Posterior fossa gangliocytoma with facial nerve invasion: case report. Arq Neuropsiquiatr 61:274–276

    Article  PubMed  Google Scholar 

  27. Kuchelmeister K, Nestler U, Siekmann R, Schachenmayr W. (2006) Liponeurocytoma of the left lateral ventricle – case report and review of the literature. Clin Neuropathol 25:86–94

    PubMed  CAS  Google Scholar 

  28. Kurosaki M, Saeger W, Lüdecke DK. (2002) Intrasellar gan-gliocytomas associated with acromegaly. Brain Tumor Pathol 19(2):63–7

    Article  PubMed  Google Scholar 

  29. Lagares A, Gomez PA, Lobato RD, Ricoy JR, Ramos A, de la Lama A. (2001) Ganglioglioma of the brainstem: report of three cases and review of the literature. Surg Neurol 56:315–322

    Article  PubMed  CAS  Google Scholar 

  30. Lok C, Viseux V, Avril MF, Richard MA, Gondry-Jouet C, Deramond H, Desfossez-Tribout C, Courtade S, Delaunay M, Piette F, Legars D, Dreno B, Saïag P, Longy M, Lorette G, Laroche L, Caux F. (2005) Cancerology Group of the French Society of Dermatology. Brain magnetic resonance imaging in patients with Cowden syndrome. Medicine (Baltimore) 84:129–136

    Article  Google Scholar 

  31. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) (2007) WHO classification of tumours of the central nervous system. Neuronal and mixed neuronal-glial tumours, 4th ed. IARC Press, Lyon, pp. 95–119

    Google Scholar 

  32. Luyken C, Blümcke H, Fimmers R, Urbach H, Elger CE, Wiestler OD, Schramm J. (2003) The spectrum of longterm epilepsy-associated tumors: long-term seizure and tumor outcome and neurosurgical aspects. Epilepsia 44:822–830

    Article  PubMed  Google Scholar 

  33. Luyken C, Blümcke I, Fimmers R, Urbach H, Wiestler OD, Schramm J. (2004) Supratentorial gangliogliomas: histo-pathological grading and tumor recurrence in 184 patients with a median follow-up of 8 years. Cancer 101:146–155

    Article  PubMed  Google Scholar 

  34. Majores M, von Lehe M, Fassunke J, Schramm J, Becker AJ, Simon M. (2008) Tumor recurrence and malignant progression of gangliogliomas. Cancer 113:3355–3363

    Article  PubMed  Google Scholar 

  35. Miliaras GC, Kyritsis AP, Polyzoidis KS. (2003) Cauda equina paraganglioma: a review. J Neurooncol 65:177–190

    Article  PubMed  Google Scholar 

  36. Nakazato Y, Figarella-Branger D, Becker AJ, Scheithauer BW, Rosenblum MK. (2007) Papillary glioneural tumour. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system. 4th ed. IARC Press, Lyon, pp. 113–114

    Google Scholar 

  37. Nolan MA, Sakuta R, Chuang N, Otsubo H, Rutka JT, Snead OC, Hawkins CE, Weiss SK. (2004) Dysembryoplastic neuroepithelial tumours in childhood: long-term outcome and prognostic features. Neurology 62:2270–2276

    Article  PubMed  CAS  Google Scholar 

  38. Nowak DA, Trost HA. (2002) Lhermitte-Duclos disease (dysplastic cerebellar gangliocytoma): a malformation, hamartoma or neoplasm? Acta Neurol Scand 105:137–145

    Article  PubMed  CAS  Google Scholar 

  39. Owler BK, Makeham JM, Shingde M, Besser M. (2005) Cerebellar liponeurocytoma. J Clin Neurosci 12:326–329

    Article  PubMed  Google Scholar 

  40. Park CK, Chung CK, Choe GY, Wang KC, Cho BK, Kim HJ. (2000) Intramedullary spinal cord ganglioglioma: a report of five cases. Acta Neurochir 142:547–552

    Article  CAS  Google Scholar 

  41. Pimentel J, Resende M, Vaz A, Reis AM, Campos A, Carvalho H, Honavar M. (2008) Rosette-forming glioneuronal tumor: pathology case report. Neurosurgery 62:E1162–1163

    Article  PubMed  Google Scholar 

  42. Puchner MJ, Lüdecke DK, Saeger W, Riedel M, Asa SL. (1995) Gangliocytomas of the sellar region–a review. Exp Clin Endocrinol Diabetes 103:129–149

    Article  PubMed  CAS  Google Scholar 

  43. Rades D, Fehlauer F. (2002) Treatment options for central neurocytoma. Neurology 59:1268–1270

    Article  PubMed  Google Scholar 

  44. Rades D, Fehlauer F, Schild SE. (2004) Treatment of atypical neurocytomas. Cancer 100:814–817

    Article  PubMed  Google Scholar 

  45. Rades D, Schild SE. (2006) Value of postoperative stereotac-tic radiosurgery and conventional radiotherapy for incompletely resected typical neurocytomas. Cancer 106:1140–1143

    Article  PubMed  Google Scholar 

  46. Robinson S, Cohen AR. (2006) Cowden disease and Lhermitte-Duclos disease: an update. Case report and review of the literature. Neurosurg Focus 20:E6

    Article  Google Scholar 

  47. Rosenblum MK. (2007) The 2007 WHO classification of nervous system tumors: newly recognized members of the mixed glioneuronal group. Brain Pathol 17:308–313

    Article  PubMed  Google Scholar 

  48. Rumana CS, Valadka AB. (1998) Radiation therapy and malignant degeneration of benign supratentorial ganglio-gliomas. Neurosurgery 42:1038–1043

    Article  PubMed  CAS  Google Scholar 

  49. Rushing EJ, Thompson LD, Mena H. (2003) Malignant transformation of a dysembryoplastic neuroepithelial tumor after radiation and chemotherapy. Ann Diagn Pathol 7:240–244

    Article  PubMed  Google Scholar 

  50. Scheithauer BW, Brandner S, Soffer D. (2007) Spinal paragan-glioma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system, 4th ed. IARC Press, Lyon, pp. 117–119

    Google Scholar 

  51. Selch MT, Goy BW, Lee SP, El-Sadin S, Kincaid P, Park SH, Withers HR. (1998) Gangliogliomas. Experience with 34 patients and review of the literature. Am J Clin Oncol 21:557–564

    Article  PubMed  CAS  Google Scholar 

  52. Shin JH, Lee HK, Khang SK, Kim DW, Jeong AK, Ahn KJ, Choi CG, Suh DC. (2002) Neuronal tumors of the central nervous system: radiologic findings and pathologic correlation. Radiographics 22:1177–1189

    PubMed  Google Scholar 

  53. Takei H, Dauser R, Su J, Chintagumpala M, Bhattacharjee MB, Jones J, Adesina AM. (2007) Anaplastic ganglioglioma arising from a Lhermitte-Duclos-like lesion. J Neurosurg 107:137–142

    PubMed  Google Scholar 

  54. Tamburrini G, Colosimo C Jr, Giangaspero F, Riccardi R, Di Rocco C. (2003) Desmoplastic infantile ganglioglioma. Childs Nerv Syst 19:292–297

    Article  PubMed  CAS  Google Scholar 

  55. Thines L, Lejeune JP, Ruchoux MM, Assaker R. (2006) Management of delayed intracranial and intraspinal metastases of intradural spinal paraganglioma. Acta Neurochir 148:63–66

    Article  CAS  Google Scholar 

  56. Trehan G, Bruge H, Vinchon M, Khalil C, Ruchoux MM, Dhellemmes P, Ares GS. (2004) MR imaging in the diagnosis of desmoplastic infantile tumor: retrospective study of six cases. AJNR Am J Neuroradiol 25:1028–1033

    PubMed  Google Scholar 

  57. VanDenberg SR. (1993) Desmoplastic infantile ganglio-glioma and desmoplastic cerebral astrocytoma of infancy. Brain Pathol 3:275–281

    Article  PubMed  CAS  Google Scholar 

  58. Vantomme N, Van Calenbergh F, Goffin J, Sciot R, Demaerel P, Plets C. (2001) Lhermitte-Duclos disease is a clinical manifestation of Cowden's syndrome. Surg Neurol 56:201–204

    Article  PubMed  CAS  Google Scholar 

  59. Vaquero J, Coca S. (2007) Atypical papillary glioneuronal tumor. J Neurooncol 83:319–323

    Article  PubMed  Google Scholar 

  60. Williams SR, Joos BW, Parker JC, Parker JR. (2008) Papillary glioneuronal tumor: a case report and review of the literature. Ann Clin Lab Sci 38:287–292

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Neurosurgery, University of Bonn Medical Center, Bonn, Germany

    Matthias Simon, Rudolf A. Kristof & Johannes Schramm

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  1. Matthias Simon
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  2. Rudolf A. Kristof
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Correspondence to Matthias Simon .

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Editors and Affiliations

  1. Universitätsklinikum München Klinikum Großhadern Neurochirurgische Klinik, Marchioninistr. 15, München, 81377, Germany

    Jörg-Christian Tonn

  2. Universitätsklinikum Hamburg Krankenhaus Eppendorf Klinik und Poliklinik für Neurochirurgie, Martinistr. 52, Neurologie, Hamburg, 20246, Germany

    Manfred Westphal

  3. Division of Neurosurgery, The Hospital for Sick Children, 555 University Avenue Suite 1502, Toronto, ON, M5G 1X8, Canada

    James T. Rutka

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Simon, M., Kristof, R.A., Schramm, J. (2010). Glioneuronal Tumors. In: Tonn, JC., Westphal, M., Rutka, J.T. (eds) Oncology of CNS Tumors. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-02874-8_11

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