Summary
The histopathological, clinical, and radiological findings in 25 patients (median age 20.5 years; range 1.7–64.2 years) with gangliogliomas were assessed to correlate degree of astrocytic anaplasia and proliferative potential with recurrence or survival. Most patients (64%) presented with seizures (median Karnofsky Performance Score 90%; range 70–100%). Computerized tomography and magnetic resonance imaging showed nonspecific abnormalities. Neoplastic ganglion cells were defined as heterotopic, irregularly grouped, or having more than one nucleus of bizarre shape or size. The astrocytic component was moderately anaplastic in 15 cases and highly anaplastic (HAA) in 10. Eight patients had gross total resection, 11 had subtotal resection, and six underwent biopsy. Ten patients (five gross total resection, three subtotal resection, two biopsy) had no further treatment, 15 underwent external irradiation, and five had adjuvant chemotherapy. Twenty-four patients are alive 15–394 weeks (median 203.5 weeks) postoperatively; one with ganglioglioma-HAA died at 65 weeks. No tumor recurred after gross total resection. Duration of preoperative symptoms < 1 year, greater anaplasia, and age > 30 years at diagnosis may have increased the risk of recurrence after subtotal resection or biopsy by four, three, and two times, respectively (not significant). Bromodeoxyuridine labeling index (BUdR LI) was < 1% in eight non-recurring tumors and 1.3% in another recurring twice (second recurrence LI = 1.6%). Most patients with ganglioglioma have a good prognosis. After gross total resection, only observation is required. After subtotal resection or biopsy, recurrence is possible. BUdR labeling may guide further therapy.
Similar content being viewed by others
References
Perkins OC: Ganglioglioma. Arch Pathol Lab Med 2: 11–17, 1926
Courville CB: Ganglioglioma. Tumor of the central nervous system: Review of the literature and report of two cases. Arch Neurol Psychiatry 24: 439–491, 1930
Courville CB: Gangliogliomas. A further report with special reference to those occurring in the temporal lobe. Arch Neurol Psychiatry 25: 309–326, 1931
Courville CB, Anderson FM: Neuro-gliogenic tumours of the central nervous system. Report of two additional cases of ganglioglioma of the brain. Bull Los Angeles Neurol Soc 6: 154–176, 1941
Russell DS, Rubinstein LJ: Pathology of Tumours of the Nervous System, 5th ed. Williams & Wilkins, Baltimore 289–306, 1989
Wolf A, Morton BF: Ganglion cell tumors of the central nervous system. N Y Neurol Inst Bull 6: 453–488, 1937
Zülch KJ: Histological Typing of Tumours of the Central Nervous System. International Classification of Tumours, No. 21. World Health Organization, Geneva 43–52, 1979
Zülch KJ, Wechsler W: Pathology and classification of gliomas. In: Krayenbuhl H, Maspes PE, Sweet WH (eds) Progress in Neurological Surgery. Vol 2. Year Book, Chicago 1–84, 1968
Demierre B, Stichnoth FA, Hori A: Intracerebral ganglioglioma. J Neurosurg 65: 177–182, 1986
Diepholder HM, Schwechheimer K, Mohadjer M, Knoth R, Volk B: A clinicopathologic and immunomorphologic study of 13 cases of ganglioglioma. Cancer 68: 2192–2201, 1991
Garrido E, Becker LF, Hoffman HJ, Hendrick BE, Humphreys R: Gangliogliomas in children. A clinicopathological study. Childs Brain 4: 339–346, 1978
Henry JM, Heffner RR, Earle KM: Gangliogiomas of CNS: A clinico-pathological study of 50 cases (Abstract). J Neuropathol Exp Neurol 37: 626, 1978
Johannsson JH, Rekate HL, Roessmann U: Gangliogliomas: Pathological and clinical correlation. J Neurosurg 54: 58–63, 1981
Kalyan-Raman UP, Olivero WC: Ganglioglioma: A correlative clinicopathological and radiological study of ten surgically treated cases with follow-up. Neurosurgery 20: 428–433, 1987
Silver JM, Rawlings CE III, Rossitch E Jr, Zeidman SM, Friedman AH: Ganglioglioma: A clinical study with longterm follow-up. Surg Neurol 35: 261–266, 1991
Sutton LN, Packer RJ, Rorke LB, Bruch DA, Schut L: Cerebral gangliogliomas during childhood. Neurosurgery 13: 124–128, 1983
Sutton LN, Packer RJ, Schut L: Gangliogliomas. In: Wilkins RH, Rengachary SS (eds) Neurosurgery Update I. McGraw-Hill, New York 461–463, 1990
Sutton LN, Packer RJ, Zimmerman RA, Bruce DA, Schut L: Cerebral gangliogliomas of childhood. In: Kageyama N, Takakura K, Epstein F, Hoffman HJ, Schut L (eds) Intracranial Tumors in Infancy and Childhood. Basic Research. Diagnosis and Treatment. Progress in Experimental Tumor Research Series. Vol 30. Karger, Basel 239–246, 1987
Ventureyra E, Herder S, Mallya BK, Keene D: Temporal lobe gangliogliomas in children. Childs Nerv Syst 2: 63–66, 1986
Allegra S, Serra P: Ganglioneuroma recidivante maligno del cervello. Riv Pat Nerv 71: 94–108, 1950
Bell WO, Packer RJ, Seigel KR, Rorke LB, Sutton LN, Bruce DA, Schut L: Leptomeningeal spread of intramedullary spinal cord tumors. Report of three cases. J Neurosurg 69: 295–300, 1988
Bevilacqua G, Sarnelli R: Ganglioglioma of the spinal cord. A case with a long survival. Acta Neuropathol (Berl) 48: 239–242, 1979
Bowles AP, Pantazis CG, Allen MB, Martinez J, Allsbrook WC: Ganglioglioma, a malignant tumor? Correlation with flow deoxyribonucleic acid cytometric analysis. Neurosurgery 23: 376–381, 1988
Hall WA, Yunis EJ, Albright AL: Anaplastic ganglioglioma in an infant: Case report and review of the literature. Neurosurgery 19: 1016–1020, 1986
Kitano M, Takayama S, Nagao T, Yoshimura O: Malignant ganglioglioma of the spinal cord. Acta Pathol Jpn 37: 1009–1018, 1987
Rodewald L, Miller DC, Sciorra L, Barabas G, Lee M: Central nervous system neoplasm in a young man with Martin-Bell syndrome-fra(X)-XLMR. Am J Med Genet 26: 7–12, 1987
Russell DS, Rubinstein LJ: Ganglioglioma: A case with long history and malignant evolution. J Neuropathol Exp Neurol 21: 185–193, 1962
Hoshino T, Prados M, Wilson CB, Cho KG, Lee K-S, Davis RL: Prognostic implications of the bromodeoxyuridine labeling index of human gliomas. J Neurosurg 71: 335–341, 1989
Hoshino T, Rodriguez LA, Cho KG, Lee K-S, Wilson CB, Edwards MS, Levin VA, Davis RL: Prognostic implications of the proliferative potential of low-grade astrocytomas. J Neurosurg 69: 839–842, 1988
Fletcher WA, Hoyt WF, Narahara MH: Congenital quadrantanopia with occipital lobe ganglioglioma. Neurology 38: 1892–1894, 1988
Miller DC, Koslow M, Budzilovich GN, Burstein DE: Synaptophysin: A sensitive and specific marker for ganglion cells in central nervous system neoplasms. Hum Pathol 21: 271–276, 1990
Burger PC, Fuller GN: Pathology: trends and pitfalls in histologic diagnosis, immunopathology, and applications of oncogene research. Neurol Clin 9: 249–271, 1991
Kaplan EL, Meier P: Non-parametric estimation from incomplete observations. J Am Stat Assoc 53: 457–481, 1958
Cox DR: Regression models and life tables. J R Stat Soc 34 (Series B): 187–220, 1972
EGRET Statistical Package. Statistics and Epidemiology Research Corporation, Seattle, 1990
Worcester WL: New formation of nerve cells in a cerebral tumor-neuroglioma. J Med Res 6: 59–63, 1901
Courville CB, Abbott KH: Malignant changes in cerebral ganglioglioma (Abstract). Excerpta Medica VIII 8: 896, 1955
Cavanagh JB: On certain small tumours encountered in the temporal lobe. Brain 81: 389–405, 1958
Gullotta F, Cervos Navarro J, Puig Serra J: Istologia, ultrastruttura e comportamento in vitro di un ganglioglioma cerebrale. Acta Neurol (Napoli) 25: 188–196, 1970
Lee JC, Glasauer FE: Ganglioglioma: Light and electron microscopic study. Neurochirurgia (Stuttg) 11: 160–170, 1968
Lichtenstein BW, Zeitlin H: Ganglioglioneuroma of the spinal cord associated with pseudosyringomyelia. Arch Neurol Psychiatry 37: 1356–1370, 1937
Bruton CJ: The Neuropathology of Temporal Lobe Epilepsy. Maudsley Monographs 31. Oxford University Press, Oxford, 1988
List CF, Dowman CE, Bagchi BK, Bebin J: Posterior hypothalamic hamartomas and gangliogliomas causing precocious puberty. Neurology 8: 164–174, 1958
Taylor DC, Falconer MA, Bruton CJ, Corsellis JAN: Focal dysplasia of the cerebral cortex in epilepsy. J Neurol Neurosurg Psychiatry 34: 369–387, 1971
VandenBerg SR, May EE, Rubinstein LJ, Herman MM, Perentes E, Vinores SA, Collins VP, Park TS: Desmoplastic supratentorial neuroepithelial tumors of infancy with divergent differentiation potential (‘desmoplastic infantile gangliogliomas’). J Neurosurg 66: 58–71, 1987
Daumas-Duport C, Scheithauer BW, Chodkiewicz J-P, Laws ER Jr, Vedrenne C: Dysembryoplastic neuroepithelial tumor: A surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases. Neurosurgery 23: 545–556, 1988
Loesel LS: Fine needle aspiration cytology of a cerebral ganglioglioma. Report of a case. Acta Cytol 32: 391–394, 1988
Davis RL, Nelson E: Unilateral ganglioglioma in a tuberosclerotic brain. J Neuropathol Exp Neurol 20: 571–581, 1961
Krouwer HGJ, Davis RL, Silver P, Prados M: Gemistocytic astrocytomas: A reappraisal. J Neurosurg 74: 399–406, 1991
Shaw EG, Daumas-Duport C, Scheithauer BW, Gilbertson DT, O'Fallon JR, Earle JD, Laws ER Jr, Okazaki H: Radiation therapy in the management of low-grade supratentorial astrocytomas. J Neurosurg 70: 853–861, 1989
Kros JM, Van Eden CG, Stefanko SZ, Waayer-Van Batenburg M, van der Kwast TH: Prognostic implications of glial fibrillary acidic protein containing cell types in oligodendrogliomas. Cancer 66: 1204–1212, 1990
Albright L, Byrd RP: Ganglioglioma of the entire spinal cord. Childs Brain 6: 274–280, 1980
Nelson J, Frost JL, Schochet SS: Sudden, unexpected death in a 5-year-old boy with an unusual primary intracranial neoplasm. Ganglioglioma of the medulla. Am J Forensic Med Pathol 8: 148–152, 1987
Richmond JG, Cunningham DL, Fuste FG, Erickson CC: Ganglioglioma of the brain stem. Am J Clin Pathol 45: 692–696, 1966
Jensen I, Klinken L: Temporal lobe epilepsy and neuropathology. Histological findings in resected temporal lobes correlated to surgical results and clinical aspects. Acta Neurol Scand 54: 391–414, 1976
Garcia CA, McGarry PA, Collada M: Ganglioglioma of the brain stem. Case report. J Neurosurg 60: 431–434, 1984
Hori A, Weiss R, Schaake T: Ganglioglioma containing osseous tissue and neurofibrillary tangles. Arch Pathol Lab Med 112: 653–655, 1988
Oberc-Greenwood MA, McKeever PE, Kornblith PL, Smith BH: A human ganglioglioma containing paired helical filaments. Hum Pathol 15: 834–838, 1984
Takahashi H, Ikuta F, Tsuchida T, Tanaka R: Ultrastructural alterations of neuronal cells in a brain stem ganglioglioma. Acta Neuropathol (Berl) 74: 307–312, 1987
Anderson FM, Adelstein LJ: Ganglion cell tumor (ganglioglioma) in the third ventricle. Operative removal with clinical recovery. Arch Surg 45: 129–139, 1942
Epstein N, Epstein F, Allen JC, Aleksic S: Intractable facial pain associated with a ganglioglioma of the cervicomedullary junction: Report of a case. Neurosurgery 10: 612–616, 1982
Fletcher DE, Bailey WH: Neurogliogenic tumor of the diencephalon with unusual characteristics. Report of a case with discussion of classification. J Neuropathol Exp Neurol 6: 128–138, 1947
Mørk SJ, Berg-Jensen M, Haugen A: Cerebellar ganglioglioma: Case report. J Neurosurg 51: 562–564, 1979
Hardison HH, Packer RJ, Rorke LB, Schut L, Sutton LN, Bruce DA: Outcome of 5 children with primary intramedullary spinal cord tumors. Childs Nerv Syst 3: 89–92, 1987
Probst A, Ulrich J, Zdrojewski B, Hirt H-R: Cerebellar ganglioglioma in a child. J Neuropathol Exp Neurol 38: 57–71, 1979
Molenaar WM, Jansson DS, Gould VE, Rorke LB, Franke WW, Lee VM-Y, Packer RJ, Trojanowski JQ: Molecular markers of primitive neuroectodermal tumors and other pediatric central nervous system tumors. Monoclonal antibodies to neuronal and glial antigens distinguish subsets of primitive neuroectodermal tumors. Lab Invest 6: 635–643, 1989
Roessmann U, Velasco ME, Gambetti P, Autilio-Gambetti L: Neuronal and astrocytic differentiation in human neuroepithelial neoplasms. An immunohistochemical study. J Neuropathol Exp Neurol 42: 113–121, 1983
Castillo M, Davis PC, Takei Y, Hoffmann JC: Intracranial ganglioglioma: MR, CT, and clinical findings in 18 patients. AJNR 11: 109–114, 1990
Cox JD, Zimmerman HM, Haughton VM: Microcystic ganglioglioma treated by partial removal and radiation therapy. Cancer 50: 473–477, 1982
Giangaspero F, Doglioni C, Rivano MT, Pileri S, Gerdes J, Stein H: Growth fraction in human brain tumors defined by the monoclonal antibody Ki-67. Acta Neuropathol (Berl) 74: 179–182, 1987
Shibuya M, Ito S, Miwa T, Davis RL, Wilson CB, Hoshino T: Proliferative potential of brain tumors. Analyses with Ki-67 and anti-DNA polymerase alpha monoclonal antibodies, bromodeoxyuridine labeling, and nucleolar organizer region counts. Cancer 71: 199–206, 1993
Zülch KJ: Brain Tumors. Their Biology and Pathology, 3rd ed. Springer-Verlag, Berlin 292–301, 1986
Dorne HL, O'Gorman AM, Melanson D: Computed tomography of intracranial gangliogliomas. AJNR 7: 281–285, 1986
Farwell JR, Dohrmann GJ, Flannery JT: Central nervous system tumors in children. Cancer 40: 3123–3132, 1977
Jellinger K, Machacek E: Rare intracranial tumours in infancy and childhood. In: Voth D, Gutjahr P, Langmaid C (eds) Tumours of the Central Nervous System in Infancy and Childhood. Springer-Verlag, Berlin 44–52, 1982
Zimmerman RA, Bilaniuk LT: Computed tomography of intracerebral gangliogliomas. J Comput Tomogr 3: 24–30, 1979
Brooks BS, King DW, Gammal TE, Meador K, Yaghmai F, Gay JN, Smith JR, Flanigin HF: MR imaging in patients with intractable complex partial epileptic seizures. AJNR 11: 93–99, 1990
Gaytan-Garcia S, Blume WT, Kaufmann JCE, Girvin JP: Gangliogliomas associated with complex partial seizures (Abstract). Can J Neurol Sci 16: 229, 1989
Spencer DD, Spencer SS, Mattson RH, Williamson PD: Intracerebral masses in patients with intractable partial epilepsy. Neurology 34: 432–436, 1984
Rossi E, Vaquero J, Martinez R, Garcia-Sola R, Bravo G: Intracranial gangliogliomas. Acta Neurochir (Wien) 71: 255–261, 1984
Rutledge SL, Snead OC III, Morawetz R, Chandra-Sekar B: Brain tumors presenting as a seizure disorder in infants. J Child Neurol 2: 214–219, 1987
Levesque MF, Meagher-Villemure K, Dubeau F, Villemure J-G, Melanson D, Quesney FL, Moumdjian R, Olivier A, Feindel W, Rasmussen T, Bertrand G: Cerebral gangliogliomas — Review of 24 cases (Abstract). Can J Neurol Sci 13: 284, 1986
Steegman AT, Winer B: Temporal lobe epilepsy resulting from ganglioglioma. Report of an unusual case in an adolescent boy. Neurology 11: 406–412, 1961
Nass R, Whelan MA: Gangliogliomas. Neuroradiology 22: 67–71, 1981
Bergin DJ, Johnson TE, Spencer WH, McCord CD: Ganglioglioma of the optic nerve. Am J Ophthalmol 105: 146–149, 1988
Chilton J, Caughron MR, Kepes JJ: Ganglioglioma of the optic nerve: Case report and review of the literature. Neurosurgery 26: 1042–1045, 1990
Cohen M: Primary intradural tumor of the orbital portion of the optic nerve. Arch Ophthalmol 48: 19–22, 1919
Gritzman MCD, Snyckers FD, Proctor NSF: Ganglioglioma of the optic nerve. A case report. S Afr Med J 63: 863–865, 1983
Hunt SJ, Johnson PC: Melanotic ganglioglioma of the pineal region. Acta Neuropathol (Berl) 79: 222–225, 1989
Feigin I, Budzilovich GN: Tumors of neurons and their precursors. J Neuropathol Exp Neurol 33: 483–506, 1974
Friedman WA, Vries JK, Quisling RG; Ganglioglioma of the medulla oblongata. Surg Neurol 12: 105–108, 1979
Mizuno J, Nishio S, Barrow DL, Davis PC, Tindall GT: Ganglioglioma of the cerebellum: Case report. Neurosurgery 21: 584–588, 1987
Herrmann H-D, Neuss M, Winkler D: Intramedullary spinal cord tumors resected with CO2 laser microsurgical technique: Recent experience in fifteen patients. Neurosurgery 22: 518–522, 1988
Miller G, Towfighi J, Page RB: Spinal cord ganglioglioma presenting as hydrocephalus. J Neurooncol 9: 147–152, 1990
Pialat J, Bancel B, Pierluca P, Pelissou I: Le gangliogliome intra-medullaire. Une observation, revue de la litterature. Ann Pathol 7: 41–46, 1987
Wald U, Levy PJ, Rappaport ZH, Michowitz SD, Schuger L, Shalit MN: Conus ganglioglioma in a 21-year-old boy. Case report. J Neurosurg 62: 142–144, 1985
Tampieri D, Moumdjian R, Melanson D, Ethier R: Intracerebral gangliogliomas in patients with partial complex seizures: CT and MR imaging findings. AJNR 12: 749–755, 1991
Katz MC, Kier EL, Schechter MM: The radiology of gangliogliomas and ganglioneuromas of the central nervous system. Neuroradiology 4: 69–73, 1972
Chamberlain MC, Press GA: Temporal lobe ganglioglioma in refractory epilepsy: CT and MR in three cases. J Neurooncol 9: 81–87, 1990
Modic MT, Beale SM: Magnetic resonance imaging of supratentorial neoplasms. In: Wilkins RH, Rengachary SS (eds) Neurosurgery Update I. McGraw-Hill, New York 12–29, 1990
Rommel T, Hamer J: Development of ganglioglioma in computed tomography. Neuroradiology 24: 237–239, 1983
Christensen E: Über Ganglienzellgeschwulste im Gehirn. Virchows Arch 300: 567–581, 1937
DiTullio MV, Fried H: ganglioglioma-neurogliogenic tumor involving the left frontal lobe: Case report. Neurosurgery 5: 371–375, 1979
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Krouwer, H.G.J., Davis, R.L., McDermott, M.W. et al. Gangliogliomas: a clinicopathological study of 25 cases and review of the literature. J Neuro-Oncol 17, 139–154 (1993). https://doi.org/10.1007/BF01050216
Issue Date:
DOI: https://doi.org/10.1007/BF01050216