Abstract
The treatment of visual pathway gliomas is controversial. The many retrospective studies reporting outcome data for patients with chiasmatic/hypothalamic gliomas are difficult to interpret for several reasons. First the natural history of these tumors is erratic with some reports suggesting that most visual pathway gliomas are hamartomas and follow an indolent course, and others reporting 10-year survival rates of close to 60%. Second, earlier studies did not clearly indicate which patients had neurofibromatosis type 1 (NF1) and recent evidence suggests that the natural history of optic gliomas is more favorable in patients with NFL Third the methods and accuracy of diagnosis have changed dramatically and patients diagnosed before and after the[/p] advent of CT/MR imaging have often been included in the same series.
While surgical resection is usually not a viable option for definitive treatment of these tumors, recent studies have shown favorable results after subtotal resection in selected patients. The efficacy of radiotherapy has not been unequivocally demonstrated and treatment-related morbidity has become a major concern, in particular, adverse effects on cognition and growth. Chemotherapy has been advanced as an viable alternative to avoid or delay the adverse affects of RT, but the long-term outcome benefits and adverse effects of treatment are just being defined. Despite the limitations of currently available information, sufficient data are now available to rational management quotelines for the majority of children with chiasmatic/hypothalamic gliomas.
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References
Dutton JJ: Gliomas of the anterior visual pathway. Survey of Ophthalmology 38: 427–451, 1994
Packer RJ, Bilaniuk LT, Cohen BH, Braffman BH, Obringer AC, Zimmerman RA, Siegel KR, Sutton LN, Savino PJ, Zackai EH, Meadows AT: Intracranial visual pathway gliomas in children with neurofibromatosis. Neurofibromatosis 1: 212–222, 1988
Meleu O: Ophthalmic manifestations of brain tumors. In: Peyman GA, Sanders DR, Goldberg MF (eds) Principles and Practices of Ophthalmology. Philadelphia, WB Saunders Company. 1980, pp 2012–2015
Glaser JS: Gliomas of the anterior visual pathways in childhood; rationale for conservative management. In: Brockhurst RJ, Borouchoff Sa, Hutchinson BT (eds) Controversy in Ophthalmology, Philadelphia, W.B. Saunders Company. 1977, pp 897–906
Wong JYC, Uhl V, Wara WM, Sheline GE: Optic gliomas. Cancer 60: 1847–1855, 1987
Rush JA, Younge BR, Campbell RJ, MacCarty CS: Optic glioma: long term follow up of 85 histopathologically verified cases. Ophthalmol 89: 1213–1219, 1982
Horwich A, Bloom HJG: Optic gliomas: radiation therapy and prognosis. Int J Radial Oncol Biol Phys 11: 1067–1079, 1985
Miller NR, Iliff WJ, Green WR: Evaluation and management of gliomas of the anterior visual pathways. Brain 97: 743–754, 1974
Tenny RT, Laws ER, Younge BR, Rush JA: The neurosurgical management of optic gliomas. J Neurosurg 57: 452–458, 1982
Macfadzean RM, Brewin TB, Doyle D, Grossart K: Glioma of the optic chiasm and its management. Trans Ophthalmol Soc UK 103: 199–207, 1983
Alvord EC, Lofton S: Gliomas of the optic nerve or chiasm. J Neurosurg 68: 85–98, 1988
Chang CH, Wood EH: The value of radiation therapy for gliomas of the anterior visual pathway. In: Brockhurst RJ, Borouchoff SA, Hutchinson BT (eds) Controversy in Ophthalmology, Philadelphia, WB Saunders Company. 1977, pp 878–886
DeSousa Al, Kalsbeck JE, Mealey J, Ellis FD, Muller J: Optic chiasmatic glioma in children. Am J Ophthalmol 87: 376–381, 1979
Packer RJ, Savino PJ, Bilaniuk LT, Zimmerman RA, Schatz NJ, Rosenstock JG, Nelson DS, Jarrett PD, Bruce DA, Schut L: Chiasmatic gliomas of childhood: a reappraisal of natural history and effectiveness of cranial irradiation. Child's Brain 10: 393–403, 1983
Jenkin D, Angyalfi S, Becker L, Berry M, Buncic R, Chan H, Doherty M, Drake J, Greenberg M, Hendrick B, Hoffman H, Humphreys R, Weitzman S: Optic glioma in children: surveillance, resection or irradiation. Int J Radial Oncol Biol Phys 25: 215–225, 1993
Davis FA: Primary tumors of the optic nerve. Arch Ophthalmol 23: 735–821, 957–1022, 1940
Marshall D: Glioma of the optic nerve as a manifestation of von Recklinghausen's disease. Am J Ophthalmol 37: 15–36, 1954
Lloyd LA: Gliomas of the optic nerve and chiasm in childhood. Trans Am Ophthalmol Soc 52: 488–535, 1973
Imes RK, Hoyt WF: Childhood chiasmal gliomas: an update on the fate of patients in the 1969 San Francisco study. Br J Ophthalmol 70: 179–182, 1986
Nishio S, Takeshita I, Fujiwara S, Fukui M: Optico-hypotha-lamic glioma: an analysis of 16 cases. Child's Nerv Syst 9: 334–338, 1993
Petronio J, Edwards MSB, Prados M, Freyberger S, Rabbitt J, Silver P, Levin V: Management of chiasmal and hypothalamic gliomas of infancy and childhood with chemotherapy. J Neurosurg 74: 701–708, 1991
Brauner R, Malandry F, Rappaport R, Zucker JM, Kalifa C, Pierre-Kahn A, Bataini P, Dufier JL: Growth and endocrine disorders in optic glioma. Eur J Pediatr 149: 825–828, 1990
Oxenhandler DC, Sayers MP: The dilemma of childhood optic gliomas. J Neurosurg 48: 34–41, 1978
Riccardi VM: Neurofibromatosis. In: Gomez MR (ed) Neurocutaneous Diseases: A Practical Approach. Boston, Butterworth. 1987, pp 11–29
Lewis RA, Gerson LP, Axelson KA, Riccardi VM, Whitford RP: Von Recklinghausen neurofibromatosis II. Incidence of optic gliomata. Ophthalmol 91: 929–935, 1984
Listernick R, Charrow J, Greenwald MJ, Esterly NB: Optic gliomas in children with neurofibromatosis type 1. J Pediatr 114: 788–792, 1989
Listernick R, Charrow J, Greenwald M, Mets M: Natural history of optic pathway tumors in children with neurofibromatosis type 1: A longitudinal study. J Pediatr 125: 63–66, 1994
Russell DS, Rubinstein LJ: Gliomas of the optic nerve and chiasms. In: Pathology of Tumours of the Nervous System. 5th edition. Baltimore, Williams & Wilkins. 1989. pp 370–376
Fletcher WA, Imes RK, Hoyt WE Chiasmal gliomas: appearance and long-term changes demonstrated by computerized tomography. J Neurosurg 65: 154–159, 1986
Verhoeff FH: Tumors of the optic nerve. In: Penfield W (ed) Cytology and Cellular Pathology of the Nervous System. New York, Hoeber. 1932, pp 1028–1939
Miller NR: Tumors of neuroectodermal origin. In: Walsh's and Hoyt's Clinical Neuro-ophthalmology. 4th edition, volume 3, chapter 45:1988, pp 1243-1324
Civitello LA, Packer RJ, Rorke LB, Siegel K, Sutton LN, Schut L: Leptomeningeal dissemination of low-grade gliomas in childhood. Neurology 38: 562–566, 1988
Bruggers CS, Friedman HS, Phillips PC, Wiener MD, Hockenberger B, Oakes WJ, Buckley EG: Leptomeningeal dissemination of optic pathway gliomas in three children. Am J Ophthalmol 111: 719–723, 1991
Packer RJ, Siegel KR, Sutton LN, Litmann P, Bruce DA, Schut L: Leptomeningeal dissemination of primary central nervous system tumors of childhood. Ann Neurol 18: 217–221, 1985
Glaser JS, Hoyt WF, Corbett J: Visual morbidity with chiasmal glioma. Arch Ophthalmol 85: 3–12, 1971
Davis PC, Hoffman JC, Weidenheim KM: Large hypothalamic and optic chiasm gliomas in infants: difficulties in distinction. AJNR 5: 579–585, 1984
Wren MWG: Significance of the so-called J-shaped sella in the diagnosis of intracranial aneurysm. Br J Ophthalmol 53: 307–309, 1969
Hoyt WF, Baghdassarian SA: Optic gliomas of childhood: natural history and rationale for conservative management. Br J Ophthalmol 53: 793–798, 1969
Martin P, Cushing H: Primary gliomas of the chiasm and optic nerves in their intracranial portion. Arch Ophthalmol 52: 209–241, 1923
Danoff BF, Kramer S, Thompson N: The radiotherapeutic management of optic nerve gliomas in children. Int J Radiat Oncol Biol Phys 6: 45–50, 1980
Brown EW, Riccardi VM, Mawad M, Handel S, Goldman A, Bryan RN: MR imaging of optic pathways in patients with neurofibromatosis. AJNR 8: 1031–1036, 1987
Bynke H, Kagström E, Tjernström K: Aspects on the treatment of gliomas of the anterior visual pathway. Acta Ophthalmol 55: 269–280, 1977
Flickenger JC, Torres C, Deutsch M: Management of low grade gliomas of the optic nerve. Cancer 61: 635–642, 1988
Iraci G, Gerosa M, Tomazzoli L, Padatschor K, Fiore DL, Javicoli R, Secchi AG: Gliomas of the optic nerve and chiasm. A clinical review. Child's Brain 8: 326–349, 1981
Duffner PK, Cohen ME, Anderson SW, Voorhess ML, MccGillivray ME, Panahon A, Brecher ML: Long-term effects of treatment on endocrine function in children with brain tumors. Ann Neurol 14: 528–532, 1983
Liu GT, Lessel S: Spontaneous visual improvement in chiasmal gliomas. Am J Ophthalmol 114: 193–201, 1992
Brzowski AE, Bazan C, Mumma JV, Ryan SG: Spontaneous regression of optic glioma in a patient with neurofibromatosis. Neurology 42: 679–681, 1992
Borit A, Richardson EP: The biological and clinical behaviour of pilocytic astrocytomas of the optic pathways. Brain 105: 161–187, 1982
Packer RJ, Sutton LN, Bilaniuk LT, Radcliffe J, Rosenstock JG, Siegel KR, Bunin GR, Savino PJ, Bruce DA, Schut L: Treatment of chiasmatic/hypothalamic gliomas of childhood with chemotherapy: An update. Ann Neurol 23: 79–85, 1988
Dosoretz DE, Blitzer PH, Wang CC, Linggood RM: Management of glioma of the optic nerve and/or chiasm. Cancer 45: 1467–1471, 1980
North American study group for optic glioma: Tumor spread in unilateral optic glioma; Study report number 2. Neurofibromatosis 2: 195–203, 1989
Wright JE, McDonald WI, Call NB: Management of optic nerve gliomas. Br J Ophthalmol 64: 545–552, 1980
Eggers H, Jakobiec FA, Jones IS: Optic nerve gliomas. In: Duane TD, Jaeger EA (eds) Clinical Ophthalmology. New York, Harper and Row. Volume 2, chapter 42: 1976, pp 1–17
Wisoff JH, Abbott R, Epstein F: Surgical management of exophytic chiasmatic-hypothalamic tumors of childhood. J Neurosurg 73: 661–667, 1990
Baram TZ, Moser RP, Van Eyes J: Surgical management of progressive visual loss in optic gliomas of childhood (Abstract). Ann Neurol 20: 398, 1986
Capo H, Kupersmith MJ: Efficacy and complications of radiotherapy of anterior visual pathway tumors. Neurol Clinics 9: 179–203, 1991
Traveras JM, Mount LA, Wood EH: The value of radiation therapy in the management of glioma of the optic nerves and chiasm. Radiology 66: 518–528, 1956
Bamford FN, Jones PM, Pearson D, Ribeiro GG, Shalet M, Beardwell CG: Residual disabilities in children treated for intracranial space-occupying lesions. Cancer 37: 1149–1151, 1976
Packer RJ, Sutton LN, Atkins TE, Radcliffe J, Bunin GR, D'Angio G, Siegel KR, Schut L: A prospective study of cognitive function in children receiving whole brain radiotherapy and chemotherapy: 2 year results. J Neurosurg 70: 707–713, 1989
Duffner PK, Cohen ME, Thomas PRM, Lansky SB: The long-term effects of cranial irradiation on the central nervous system. Cancer 56: 1841–1846, 1985
Danoff BF, Cowchock S, Marquette C, Mulgrew L, Kramer S: Assessment of the long-term effects of primary radiation therapy for brain tumors in children. Cancer 49: 1580–1586, 1982
Taphoorn MJB, Schiphorst AK, Snoek FJ, Lindeboom J, Wolbers JG, Karim ABMF, Huijgens PC, Heimans JJ: Cognitive functions and quality of live in patients with low-grade gliomas: the impact of radiotherapy. Ann Neurol 36: 48–54, 1994
Ogilvy-Stuart AL, Clayton PE, Shalet SM: Cranial irradiation and early puberty. J Clin Endocrinol Metab 78: 1282–1286. 1994
Fuks Z, Glatstein E, Marsa GW Bagshaw MA, Kaplan HS: Long-term effects of external radiation on the pituitary and thyroid glands. Cancer 37: 1152–1161, 1976
Rosenstock JG, Evans AE, Schut L: Response to vincristine of recurrent brain tumors in children. J Neurosurg 45: 135–140, 1976
Rosenstock JG, Packer RJ, Bilaniuk L, Bruce DA, Radcliffe JL, Savino P: Chiasmatic optic gliomas treated with chemotherapy. J Neurosurg 63: 862–866, 1985
67.Sutton LN, Molloy P, Sernyak H, Goldwein J, Phillips P, Rorke L, Moshang T, Lange B, Packer RJ: Long-term outcome of hypothalamic/chiasmatic astrocytomas in children treated with conservative surgery. In press
Edwards MS, Levin VA, Wilson CB: Brain tumor chemotherapy: An evaluation of agents in current use for phase II and III trials. Cancer Treat Rep 64: 1179–1205, 1980
Sumer T, Freeman AI, Cohen M, Bremer A, Thomas PRM, Sinks LF: Chemotherapy in recurrent noncystic low grade astrocytomas of the cerebrum in children. J Surg Oncol 10: 45–54, 1978
Lefkowitz IB, Packer RJ, Sutton LN, Siegel KR, Bruce DA, Evans AE, Schut L: Results of the treatment of children with recurrent gliomas with lomustine and vincristine. Cancer 61: 896–902, 1988
Pons M, Finlay JL, Walker RW, Pucetti D, Packer RJ, McElwain M: Chemotherapy with vincristine (VCR) and etoposide (VP-16) in children with low grade astrocytoma. J Neuro-Oncol 14: 151–158, 1992
Friedman HS, Krischner JP, Burger P, Oakes WJ, Hockenberger B, Weiner MD, Falleta JM, Norris D, Ragab AH, Mahoney DH, Whitehead MV, Kun LE: Treatment of children with progressive of recurrent brain tumors with carboplatin or iproplatin: A pediatric oncology group randomized phase II study. J Clin Oncol 10: 249–256, 1992
Packer RJ, Lange B, Ater J, Nicholson HS, Allen J, Walker R, Prados M, Jakacki R, Reaman G, Needler MN, Phillips PC, Ryan J, Boyett JM, Geyer R, Finlay J: Carboplatin and vincristine for recurrent and newly diagnosed low-grade gliomas of childhood. J Clin Oncol 11: 850–856, 1993
Packer RJ, Allen JC, Phillips P, Jakacki R, Ater JL, Geyer JR, Finlay J, Nicholson HS, Kurczynski E, Boyett J, and the Low Grade Glioma Study Group, Washington DC: Efficacy of chemotherapy for children with newly diagnosed progressive low-grade gliomas (Abstract). Ann Neurol 36: 496, 1994
Aarabi B, Long DM, Miller NR: Enlarging optic chiasmal glioma with stable visual acuity. Surg Neurol 10: 175–177, 1978
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Garvey, M., Packer, R.J. An integrated approach to the treatment of chiasmatic-hypothalamic gliomas. J Neuro-Oncol 28, 167–183 (1996). https://doi.org/10.1007/BF00250197
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DOI: https://doi.org/10.1007/BF00250197