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. 2008 Jan 28;7(2):799–806. doi: 10.1111/j.1750-3639.1997.tb01065.x

Optic Chiasmatic‐Hypothalamic Glioma

James T Rutka 1,✉, Laurence E Becker 1, Harold J Hoffman 1
PMCID: PMC8098322  PMID: 9161730

Abstract

Optic chiasmatic‐hypothalamic gliomas (OCHGs) have been considered benign tumors and self‐limiting in growth potential because of their histological appearance. Unfortunately, most clinical series have reported significant morbidity and mortality especially with the more extensive, posteriorly positioned tumors. The biological behavior of OCHGs is age‐dependent, with patients younger than five years and older than 20 years typically having tumors that exhibit aggressive growth. There are no specific pathological features to help differentiate the clinical behavior of such tumors. The emergence of modern imaging techniques, including magnetic resonance imaging (MRI), has facilitated the monitoring of the natural history of the disease and the determination of the effects of therapy. Most patients with OCHGs survive for many years. While the natural history of an OCHG for any individual may be indeterminate, enough data are now available from large series to make recommendations for treatment. Our current treatment policy for patients with OCHGs in the context of NF‐I without visual failure is a conservative one involving CSF shunting for hydrocephalus if present and medical therapy for endocrinologic dysfunction. Patients with or without NF‐I with visual deterioration or progressive neurological deficits and a rapidly expanding suprasellar mass lesion are treated surgically. After tumor resection, patients whose vision is significantly compromised or who show progression of their disease on serial neuroimaging scans receive chemotherapy. If chemotherapy proves ineffective in disease stabilization, then considerations of radiation therapy are given to children over five years old.

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References

  • 1. Albright AL, Sciabassi RJ (1985) Cavitron ultrasonic surgical aspirator and visual evoked potential monitoring for chiasmal gliomas in children. Report of two cases. J Neurosurg 63: 38–40. [DOI] [PubMed] [Google Scholar]
  • 2. Alvord EC Jr, Lofton S. (1988) Gliomas of the optic nerve or chiasm. Outcome by patients' age, tumor site, and treatment. J Neurosurg 68: 85–98. [DOI] [PubMed] [Google Scholar]
  • 3. Bakardjiev AI, Barnes PD, Goumnerova LC, Black PM, Scott RM, Pomeroy SL, Billett A., Loeffler JS, Tarbell NJ (1996) Magnetic Resonance Imaging changes after stereotactic radiation therapy for childhood low grade astrocytoma. Cancer 76: 864–874. [DOI] [PubMed] [Google Scholar]
  • 4. Beyer RA, Paden P., Sobel D., Flynn FG (1986) Moyamoya pattern of vascular occlusion after radiotherapy for glioma of the optic chiasm. Neurology 36: 1173–8. [DOI] [PubMed] [Google Scholar]
  • 5. Chong BW, Newton TH (1993) Hypothalamic and pituitary pathology radiologic. Clinics of North America 31: 5: 1147–83. [PubMed] [Google Scholar]
  • 6. Chutorian AM, Schwartz JF, Evans RA, Carter S. (1964) Optic gliomas in children. Neurology 14: 83–95. [DOI] [PubMed] [Google Scholar]
  • 7. Cohen DH, Steinberg M., Buchwald R. (1974) Suprasellar germinomas: diagnostic confusion with optic gliomas. Case report. J Neurosurg 41: 490–3. [DOI] [PubMed] [Google Scholar]
  • 8. Flickinger JC, Torres C., Deutsch M. (1988) Management of low grace gliomas of the optic nerve and chiasm. Cancer 61: 635–642. [DOI] [PubMed] [Google Scholar]
  • 9. Gajjar A., Bhargava R., Jenkins JJ, Heideman R., Sanford RA, Langston JW, Walter AW, Kuttesch JF, Muhlbauer M., Kun LE (1995) Low‐grade astrocytoma with neuraxis dissemination at diagnosis J Neurosurg 83: 67–71. [DOI] [PubMed] [Google Scholar]
  • 10. Glaser JS, Hoyt WF, Corbett J. (1971) Visual morbidity with chiasmal glioma. Long‐term studies of visual fields in untreated and irradiated cases. Arch Ophthalmol 85: 3–12. [DOI] [PubMed] [Google Scholar]
  • 11. Goud RJ, Hilal DK, Chutorian AM (1987) Efficacy of radiotherapy in optic gliomas Pediatr Neurol 3: 29–32. [DOI] [PubMed] [Google Scholar]
  • 12. Hoffman HJ (1983) Optic pathway gliomas. In: Brain tumors in the young, Amador LV (eds.), Charles C Thomas: Springfield IL , pp. 622–33. [Google Scholar]
  • 13. Hoffman HJ (1996) Optic and hypothalamic gliomas. Techniques in Neurosurgery 2: 194–203. [Google Scholar]
  • 14. Hoffman HJ, Humphreys RP, Drake JM, Rutka JT (1993) Optic pathway/hypothalamic gliomas: a dilemma in management. Pediatr Neurosurg 19: 186–95. [DOI] [PubMed] [Google Scholar]
  • 15. Hoyt WF, Baghdassarian SA (1969) Optic glioma of childhood. Natural history and rationale for conservative management. Br J Ophthalmol 53: 793–8. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 16. Imes RK, Hoyt WF (1986) Childhood chiasmal gliomas: update on the fate of patients in the 1969 San Francisco study. Br J Ophthalmol 70: 179–82. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 17. Janss AJ, Grundy R., Cnaan A., Savino PJ, Packer RJ, Nackai EH, Goldwein JW, Sutton LN, Radcliffe J., Molloy PT (1995) Optic pathway and hypothalamic glioma in children younger than age 5 years with a 6‐year follow‐up. Cancer 75: 1051–59. [DOI] [PubMed] [Google Scholar]
  • 18. Kestle JRW, Hoffman HJ, Mock AR (1993) Moyamoya phenomenon after radiation for optic glioma. J Neurosurg 79: 32–5. [DOI] [PubMed] [Google Scholar]
  • 19. Kovalic JJ, Grigsby PW, Shephard MJ, Fineburg BB, Thomas PR (1990) Radiation therapy for gliomas of the optic nerve and chiasm. Int J Radiat Oncol Biol Phys 18: 927–932. [DOI] [PubMed] [Google Scholar]
  • 20. Mamelak AN, Pracos MD, Obana WG, Cogen PH, Ecwards MS (1994) Treatment options and prognosis for multicentric juvenile pilocytic astrocytoma. J Neurosurg 81:24–30. [DOI] [PubMed] [Google Scholar]
  • 21. Martin P., Cushing H. (1923) Primary gliomas of the chi asm and optic nerve in their intracranial portion. Arch Ophthalmol 52: 209. [Google Scholar]
  • 22. Matson DD (1969) Neurosurgery of Infancy and Childhood, Charles C Thomas: Springfield , IL , USA . [Google Scholar]
  • 23. McCallough DC, Johnson DL (1989) Optic nerve gliomas and other tumors involving the optic nerve and chiasm. In: Pediatic Neurosurgery, MeLaurin RE, (ed.), pp. 391–397, WB Saunders: Philadelphia , PA , USA . [Google Scholar]
  • 24. Moghrabi A., Friedman HS, Burger PC, Tien R., Oakes WJ (1993) Carboplatin treatment of progressive optic pathway gliomas to delay radiotherapy. J Neurosurg 79: 223–227. [DOI] [PubMed] [Google Scholar]
  • 25. Okuno T., Preosky A., Gado M. (1985) The moyamoya syndrome associated with iradiation of an optic glioma in children: report of two cases and review of the literature. Pediatr Neurol 1: 311–6. [DOI] [PubMed] [Google Scholar]
  • 26. Oxenhandler DC, Sayers MP (1978) The dilemma of childhood optic gliomas. J Neurosurg 48: 34–41. [DOI] [PubMed] [Google Scholar]
  • 27. Packer RJ, Savino PJ, Bilaniuk LT, Zimmerman RA, Schatz NJ, Rosenstock JG, Nelson DS, Jarrett PD, Bruce DA, Schut L. (1983) Chiasmatic gliomas of childhood. A reappraisal of natural history and effectiveness of cranial irradiation. Childs Brain 10: 393–403. [PubMed] [Google Scholar]
  • 28. Packer RJ, Sutton LN, Bilaniuk LT, Radcliffe J., Rosenstock JG, Siegel KR, Bunin GR, Savino PJ, Bruce DA, Schut L. (1988) Treatment of chiasmatic hypothalamic gliomas of childhood with chemotherapy: an update. Ann Neurol 23: 79–85. [DOI] [PubMed] [Google Scholar]
  • 29. Petronio J., Edward MSB, Prados M., Freyberger S., Rabbitt J., Silver P., Levin VA (1991) Management of chiasmal and hypothalamic gliomas of infants and childhood with chemotherapy. J Neurosurg 74: 701–8. [DOI] [PubMed] [Google Scholar]
  • 30. Pierce SM, Barnes PD, Loeffier JS, McGinn C., Tarbell NJ (1990) Definitive radiation therapy in the management of symptomatic patients with optic glioma. Survival and long‐term effects. Cancer 65: 45–52. [DOI] [PubMed] [Google Scholar]
  • 31. Rappaport R., Brauner R. (1989) Growth and endocrine disorders secondary to cranial irradiation. Pediatr Res 25: 561–567. [DOI] [PubMed] [Google Scholar]
  • 32. Redfern RM, Scholtz CL (1980) Long‐term Survival with optic nerve glioma. Surg Neurol 14: 371–5. [PubMed] [Google Scholar]
  • 33. Rhoton AL (1985) Microsurgical anatomy of the sellar region. In: Neurosurgery, Wilkins RH, Rengachary SS (eds.), p. 811, McGraw‐Hill: Toronto , ON Canada . [Google Scholar]
  • 34. Richards GE (1980) Effects of irradiation on the hypothalamic and pituitary regions. In: Radiation Damage To The Nervous System, Gilbert HA, Kogar AR (eds.), pp. 175–80, Raven: New York , NY USA . [Google Scholar]
  • 35. Rosenstock JG, Packer RJ, Bilaniuk L., Bruce DA, Radcliffe JL, Savino P. (1985) Chiasmatic optic glioma treated with chemotherapy. J Neurol Sci 63: 862–866. [DOI] [PubMed] [Google Scholar]
  • 36. Rush JA, Younge BR, Campbell RJ, McCarty CS (1982) Optic glioma. Long‐term follow‐up of 85 histopathologically verified cases. Ophthalmology 89: 1213–9. [PubMed] [Google Scholar]
  • 37. Rutka JT, Hoffman HJ, Drake JM, Humphreys RP (1992) Suprasellar and sellar tumors in chiidhood and adolescence. Neurosurgery Clinics of North America 3: 803–20. [PubMed] [Google Scholar]
  • 38. Sayers MP (1982) Optic nerve gliomas. In: Pediatric Neurosurgery, MeLaurin RL, (ed.), pp. 513–522, Grune & Stratton: New York , NY USA . [Google Scholar]
  • 39. Schuster G., Westberg G. (1967) Glomas of the optic nerve and chiasm. Acta Radiol 6: 221–32. [DOI] [PubMed] [Google Scholar]
  • 40. Servo A., Puranen M. (1978) Moyamoya syndrome as a complication of radiation therapy. Case report. J Neurosurg 48: 1026–9. [DOI] [PubMed] [Google Scholar]
  • 41. Stem J., DiGiacinto GV, Housepian EM (1979) Neurofibromatosis and optic glioma: clinicai and morphological correlations. Neurosurgery 4: 524–84. [DOI] [PubMed] [Google Scholar]
  • 42. Sutton LN, Molloy PT, Semyak H., Goldwein J., Philips PL, Rorke LB, Moshang T. Jr., Lange B., Packer RJ (1995) Long‐term outcome of hypothalamic astrocytomas in children treated with conservative surgery. J Neurosura 83: 583–589. [DOI] [PubMed] [Google Scholar]
  • 43. Taveras JM, Mount LA, Wood EH (1956) The value of radiation therapy in the management of glioma of the optic nerves and chiasm. Radiology 66: 513–28. [DOI] [PubMed] [Google Scholar]
  • 44. Tenny RT, Laws ER Jr., Younge BR. Rush JA (1982) The neurosurgical management of optic glioma. Results in 104 patients. J Neurosurg 57: 452–8. [DOI] [PubMed] [Google Scholar]
  • 45. Thapar K., Fukuyama K., Rutka JT (1995) Neurogenetics ana the molecular biology of human bran. In: Brain Tumors, Kaye AH, Laws JR, ER , (eds), pp. 69–95, Churchill Livingstone: Edinburgh , Hong Kong , Madrid , Melbourne , New York and Tokyo . [Google Scholar]
  • 46. Venes JL, Latack J., Kandt RS (1984) Postoperative regression of opticochiasmatic astrocytoma. A case for expectant therapy. Neurosurgery 15: 421–423. [DOI] [PubMed] [Google Scholar]
  • 47. Weiss L., Sagerman RH, King GA, Chung CT, Dubowy RL (1987) Controversy in the management cf optic nerve glioma. Cancer 59: 1000–4. [DOI] [PubMed] [Google Scholar]
  • 48. Wisoff JH, Abbott R., Epstein F. (1990) Surgical management of exophytic chiasmatic‐hypothalamic tumers of childhood. J Neurosurg 73: 661–7. [DOI] [PubMed] [Google Scholar]
  • 49. Wong JYC, Uhl V., Wara WM, Shelne GE (1987) Optic glioma. A reanalysis of the University of California. San Francisco experience. Cancer 60: 1847–55. [DOI] [PubMed] [Google Scholar]

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