Tumors of the Central Nervous System, Volume 9 pp 155-165 | Cite as
Gangliogliomas and Other Low Grade Neuronal Neoplasms of the Central Nervous System: Diagnosis, Treatment, and Prognosis
Abstract
Glioneuronal tumors of the brain and spinal cord are relatively uncommon but highly interesting neoplasms, whose very existence provides support for current stem cell theories regarding the origins of central nervous system tumors. This chapter describes the clinical presentations, neuroradiological features, and diagnostic pathological characteristics of gangliogliomas, parenchymal neurocytic tumors, and other intra-parenchymal (not intraventricular) neuronal neoplasms of the CNS, together with summaries of current treatment options. Gangliogliomas are tumors composed of mixtures of glial cells, usually astrocytoma cells, with large neoplastic neurons (ganglion cells); the majority are slow-growing, indolent neoplasms which respond best to neurosurgical extirpation. Pleiomorphic Xanthoastrocytomas, subependymal giant cell tumors, and some anaplastic large cell gliomas all may exhibit co-expression of glial and neuronal antigens in the same cells. Parenchymal neurocytic tumors mostly resemble oligodendrogliomas, but like their intraventricular counterparts have immunoreactivity for synaptophysin, neurofilament protein, and other neuronal antigens. These are more controversial entities, with a range of appearances (some have ganglion cells, some lack astrocytic elements whereas in others they are present, and many have anaplastic changes such as vascular hyperplasia, necrosis, and high mitotic rates).
Keywords
Ganglion Cell Total Excision Glioneuronal Tumor Subependymal Giant Cell Astrocytoma Mixed GliomaReferences
- Adachi Y, Yagishita A (2008) Gangliogliomas: characteristic imaging findings and role in temporal lobe epilepsy. Neuroradiology 50:829–834PubMedCrossRefGoogle Scholar
- 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. IARC Press, Lyon, pp 103–105Google Scholar
- Blümcke I, Wiestler OD (2002) Gangliogliomas: an intriguing tumor entity associated with focal epilepsies. J Neuropathol Exp Neurol 61:575–584PubMedGoogle Scholar
- Brat DJ, Scheithauer BW, Eberhart CG, Burger PC (2001) Extraventricular neurocytomas. Pathologic features and clinical outcome. Am J Surg Pathol 25:1252–1260PubMedCrossRefGoogle Scholar
- Castillo M, Davis PC, Hoffman JC (1990) Intracranial ganglioglioma. Am J Roentgenol 154:607–612Google Scholar
- Chou S, Varikatt W, Dexter M, Ng T (2010) Extraventricular neurocytoma with atypical features and ganglionic differentiation. J Clin Neurosci 17:920–922PubMedCrossRefGoogle Scholar
- El Khashab M, Gargan L, Margraf L, Koral K, Nejat F, Swift D, Weprin B, Bowers DC (2009) Predictors of tumor progression among children with gangliogliomas. J Neurosurg Pediatr 3:461–466PubMedCrossRefGoogle Scholar
- Figarella-Branger D, Soylemezoglu 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. IARC Press, Lyon, pp 106–109Google Scholar
- Giangasapero F, Cenacchi G, Losi L, Cerasoli S, Bisceglia M, Burger PC (1997) Extraventricular neoplasms with neurocytoma features: a clinicopathological study of 11 cases. Am J Surg Pathol 21:206–212CrossRefGoogle Scholar
- Giannini C, Paulus W, Louis DN, Liberski P (2007) Pleomorphic xanthoastrocytoma. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system. IARC Press, Lyon, pp 22–24Google Scholar
- Guilioni M, Gardella E, Rubboli G, Zucchelli M, Bernardi B, Roncaroli F, Tassinari CA, Calbucci F (2006) Lesionectomy in epileptogenic gangliogliomas: seizure outcome and surgical results. J Clin Neurosci 13:529–535CrossRefGoogle Scholar
- Guilioni M, Rubboli G, Marucci G, Martinoni M, Volpi L, Michelucci R, Marliani AF, Bisulli F, Tinuper P, Castana L, Sartori I, Calbucci F (2009) Seizure outcome of epilepsy surgery in focal epilepsies associated with temporomesial glioneuronal tumors: lesionectomy compared with tailored resection. J Neurosurg 11:1275–1282Google Scholar
- Hainfellner JA, Scheithauer BW, Giangaspero F, Rosenblum MK (2007) Rosette-forming glioneuronal tumour of the fourth ventricle. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system. IARC Press, Lyon, pp 115–116Google Scholar
- Hassoun J, Gambarelli D, Grisoli F, Pellet W, Salamon G, Pellissier JF, Toga M (1982) Central Neurocytoma. An electron- microscopic study of two cases. Acta Neuropathol 56:151–156PubMedCrossRefGoogle Scholar
- Im S, Chung CK, Cho B, Wang K, Yu I, Song IC, Cheon GJ, Lee DS, Chi JG (2002) Intracranial ganglioglioma: preoperative characteristics and oncologic outcome after surgery. J Neuro Oncol 59:173–183CrossRefGoogle Scholar
- Im SH, Chung CK, Kim SK, Cho BK, Kim MK, Chi JG (2004) Pleomorphic xanthoastrocytoma: a developmental glioneuronal tumor with prominent glioproliferative changes. J Neuro Oncol 66:17–27CrossRefGoogle Scholar
- Jaffey PB, Mundt AJ, Baunoch DA, Armstrong DL, Hamilton WJ, Zagaja VG, Grossman RG, Wollman RL (1996) The clinical significance of extracellular matrix in gangliogliomas. J Neuropathol Exp Neurol 55:1246–1252PubMedCrossRefGoogle Scholar
- Kepes JJ, Rubinstein LJ, Eng LF (1979) A study of 12 cases. Cancer 44:1839–1852PubMedCrossRefGoogle Scholar
- 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. IARC Press, Lyon, pp 110–112Google Scholar
- Lang FF, Epstein FJ, Ransohoff J, Allen JC, Wisoff J, Abbott IR, Miller DC (1993) Central nervous system gangliogliomas. Part 2: clinical outcome. J Neurosurg 79:867–873PubMedCrossRefGoogle Scholar
- Liauw SL, Byer JE, Yachnis AT, Amdur RJ, Mendenhall WM (2007) Radiotherapy after subtotally resected or recurrent ganglioglioma. Int J Radiat Oncol 67:244–247CrossRefGoogle Scholar
- Luyken C, Blümcke I, Fimmers R, Urbach H, Wiestler OD, Schramm J (2004) Supratentorial ganglioglioma: histopathologic grading and tumor recurrence in 184 patients with a median follow-up of 8 years. Cancer 101:146–155PubMedCrossRefGoogle Scholar
- Majores M, Von Lehe M, Fassunke J, Schramm J, Becker AJ, Simon M (2008) Tumor recurrence and malignant progression of ganglioglioma. Cancer 113:3355–3363PubMedCrossRefGoogle Scholar
- Miller DC (2009) Modern surgical neuropathology. Cambridge University Press, New YorkGoogle Scholar
- Miller DC, Koslow MK, Budzilovich GN, Burstein DE (1990) Synaptophysin: a sensitive and specific marker for ganglion cells in central nervous system neoplasms. Hum Pathol 21:271–276PubMedCrossRefGoogle Scholar
- Miller DC, Lang FF, Epstein FJ (1993) Central nervous system gangliogliomas. Part I: pathology. J Neurosurg 79:859–866PubMedCrossRefGoogle Scholar
- Nakazato Y, Figarella–Branger D, Becker AJ, Scheithauer BW, Rosenblum M (2007) Papillary glioneuronal tumour. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (eds) WHO classification of tumours of the central nervous system. IARC Press, Lyon, pp 113–114Google Scholar
- Ng H-K, Ko HCW, Tse CCH (1994) Immunohistochemical and ultrastructural studies of oligodendrogliomas revealed features of neuronal differentiation. Int J Surg Pathol 2:47–56CrossRefGoogle Scholar
- Nishio S, Takeshita I, Fukui M (1990) Primary cerebral ganglioneurocytoma in an adult. Cancer 66:358–362PubMedCrossRefGoogle Scholar
- Ogiwara H, Nordli DR, DiPatri AJ, Alden TD, Bowman RM, Tomita T (2010) Pediatric epileptoginic gangliogliomas: seizure outcome and surgical results. J Neurosurg Pediatr 5:271–276PubMedCrossRefGoogle Scholar
- Perry A, Scheithauer BW, Macaulay RJB, Raffel C, Roth KA, Kros JM (2002) Oligodendrogliomas with neurocytic differentiation. A report of 4 cases with diagnostic and histogenetic implications. J Neuropathol Exp Neurol 61:947–955PubMedGoogle Scholar
- Perry A, Burton SS, Fuller GN, Robinson CA, Palmer CA, Resch L, Bigio EH, Gujrati M, Rosenblum MK (2010) Oligodendroglial neoplasms with ganglioglioma-like maturation: a diagnostic pitfall. Acta Neuropathol 120(2):237–252. doi: 10.1007/s00401-010-0695-9 PubMedCrossRefGoogle Scholar
- Powell SZ, Yachnis AT, Rorke LB, Rojiani AM, Eskin TA (1996) Divergent differentiation in pleomorphic xanthoastrocytoma: evidence for a neuronal element and possible relationship to ganglion cell tumors. Am J Surg Pathol 20:80–85PubMedCrossRefGoogle Scholar
- Rades D, Zwick L, Lepper J, Bonsanto M, Tronnier V, Dunst J, Schild SE (2010) The role of postoperative radiotherapy for the treatment of ganglioglioma. Cancer 116:432–442PubMedCrossRefGoogle Scholar
- Rodriguez FJ, Scheithauer BW, Port JD (2005) Unusual malignant glioneuronal tumors of the cerebrum of adults: a clinicopathologic study of 3 cases. Acta Neuropathol 112:727–737CrossRefGoogle Scholar
- Russell DS, Rubenstein LJ (1989) Pathology of tumours of the nervous system, 5th edn. Williams & Wilkins, BaltimoreGoogle Scholar
- Sharma MC, Deb P, Sharma S, Sarkar C (2006) Neurocytoma: a comprehensive review. Neurosurg Rev 29:270–285PubMedCrossRefGoogle Scholar
- Varlet P, Soni D, Miquel C, Roux F-X, Meder J-F, Chneiweiss H, Daumas-Duport C (2004) New variants of malignant glioneuronal tumors: a clinicopathological study of 40 cases. Neurosurgery 55:1377–1392PubMedCrossRefGoogle Scholar
- Zentner J, Wolf HK, Ostertun B, Hufnagel A, Campos MG, Solymosi L, Schramm J (1994) Gangliogliomas: clinical radiological and histopathological findings in 51 patients. J Neurol Neurosurg Psychiatr 57:1497–1502PubMedCrossRefGoogle Scholar