Optic nerve glioma medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]
Overview
The mainstay of therapy for optic nerve glioma is chemotherapy and radiation therapy. Chemotherapy is used in children to delay radiation therapy, and to reduce the severity of radiation therapy induced side-effects. Radiation therapy is not generally used in children less than five years of age and usually avoided in children between five and ten years of age. Radiation therapy is delayed in young children to reduce the risk of significant side- effects such as developmental and neurocognitive delay. Chemotherapy using vincristine, actinomycin D, bevacizumab, etoposide, and other agents has also been reported to be effective in patients with progressive hypothalamic/chiasmal gliomas.
Medical Therapy
As most of optic nerve gliomas will not cause vision problems or will not continue to grow, knowing that a child has an optic glioma is not an indication to begin treatment.[1] Treatment of optic nerve glioma depend on clinical context, as well as on location and size of the tumor at presentation.
- If it is isolated to one optic nerve, and does not extend to the chiasm, then resection is curative (albeit with loss of vision in that eye).
- If the tumor extends to the chiasm or more posteriorly then curative resection is not possible, with resection reserved for treatment of mass effects (proptosis, intracranial mass effect).
The most common treatments for optic nerve glioma are:
Observation
Patients with good vision on the involved side require no therapy. Observation through followup examinations and appropriate radiographic studies, preferably MRI, must be performed. Many patients if regularly followed up maintain good vision and never require a surgery.
Chemotherapy
- Chemotherapy is used in children to delay radiation therapy, and to reduce the severity of radiation therapy induced side-effects.
- Chemotherapy has the ability to cause tumor regression and stabilize the vision.
- Chemotherapy may be useful when the tumor extends into the hypothalamus, as it has an ability to shrink hypothalamic tumors.
- It is useful in diencephalic syndrome as it allows for weight gain.
- About 40%-60% patients progress after chemotherapy and require further treatment.[2][3]
- Chemotherapy may carry long-term risks of blood-borne cancers.
Combination chemotherapy using vincristine, actinomycin D, bevacizumab, etoposide, and other agents has also been reported to be effective in patients with progressive hypothalamic/chiasmal gliomas. Chemotherapy alone very rarely cures low-grade gliomas. Mostly, chemotherapy can stop the progression of optic nerve gliomas. Chemotherapy is often used prior to initiation of radiation therapy in attempt to delay the exposure of radiation as much as possible.
Radiation
- Radiation therapy is used if the tumor is resistant to chemotherapy
- Radiation therapy is not generally used in children less than five years of age and usually avoided in children between five and ten years of age. Radiation therapy is delayed in young children to reduce the risk of significant side- effects such as developmental and neurocognitive delay.
- Radiation therapy is considered if the tumor is unresectable (optic tract or chiasmal lesions), and if neurological symptoms progress.
- Radiation therapy can be done before surgery to shrink the tumor before removing it or after surgery to kill the remaining cancer cells. If chiasmal and optic tract involvement is extensive and if there is growth of the tumor within the chiasm, postoperative radiation of the chiasm and optic tract may be considered.
- Where the tumor is large and surgery is not possible, radiation therapy may be recommended. In slow growing tumor, radiation therapy may be delayed.
- Corticosteroids may be prescribed if symptoms return or to reduce swelling and inflammation caused during radiation therapy.
- Similar prognosis following radiation of a optic nerve gliomas has been seen in patients with neurofibromatosis type 1, as compared to patients without neurofibromatosis type 1.[4][5][6]
Complications of radiation therapy
- Endocrinopathy
- Secondary malignancies[7][8]
- Dementia
- Radionecrosis of the medial temporal lobe
- Leukoencephalopathy
- Developmental and neurocognitive problems
- Growth retardation
- Precocious puberty
- Hypothyroidism[9]
- Vasculopathy[10]
- NF1 patients with optic pathway glioma are less likely to resond to radiotherapy and they have a higher risk of developing late side-effects of radiation therapy.
Hormone replacement therapy
Lifelong hormone replacement therapy is used if the endocrine systems is affected.
References
- ↑ Simmons I, Gogi D (2010). "Screening children with NF1 for optic pathway glioma--Yes". Eye (Lond). 24 (9): 1429–31. doi:10.1038/eye.2010.93. PMID 20577275.
- ↑ Janss AJ, Grundy R, Cnaan A, Savino PJ, Packer RJ, Zackai EH; et al. (1995). "Optic pathway and hypothalamic/chiasmatic gliomas in children younger than age 5 years with a 6-year follow-up". Cancer. 75 (4): 1051–9. PMID 7842408.
- ↑ Laithier V, Grill J, Le Deley MC, Ruchoux MM, Couanet D, Doz F; et al. (2003). "Progression-free survival in children with optic pathway tumors: dependence on age and the quality of the response to chemotherapy--results of the first French prospective study for the French Society of Pediatric Oncology". J Clin Oncol. 21 (24): 4572–8. doi:10.1200/JCO.2003.03.043. PMID 14673044.
- ↑ Listernick R, Louis DN, Packer RJ, Gutmann DH (1997). "Optic pathway gliomas in children with neurofibromatosis 1: consensus statement from the neurofibromatosis type 1 Optic Pathway Glioma Task Force". Ann Neurol. 41 (2): 143–9. doi:10.1002/ana.410410204. PMID 9029062.
- ↑ Listernick R, Charrow J, Greenwald MJ, Esterly NB (1989). "Optic gliomas in children with neurofibromatosis type 1". J Pediatr. 114 (5): 788–92. PMID 2497236.
- ↑ Deliganis AV, Geyer JR, Berger MS (1996). "Prognostic significance of type 1 neurofibromatosis (von Recklinghausen Disease) in childhood optic glioma". Neurosurgery. 38 (6): 1114–8, discussion 1118-9. PMID 8727140.
- ↑ Kovalic JJ, Grigsby PW, Shepard MJ, Fineberg BB, Thomas PR (1990). "Radiation therapy for gliomas of the optic nerve and chiasm". Int J Radiat Oncol Biol Phys. 18 (4): 927–32. PMID 2323979.
- ↑ Kingsley DP, Kendall BE (1981). "CT of the adverse effects of therapeutic radiation of the central nervous system". AJNR Am J Neuroradiol. 2 (5): 453–60. PMID 6792884.
- ↑ 9.0 9.1 Bataini JP, Delanian S, Ponvert D (1991). "Chiasmal gliomas: results of irradiation management in 57 patients and review of literature". Int J Radiat Oncol Biol Phys. 21 (3): 615–23. PMID 1907959.
- ↑ Epstein MA, Packer RJ, Rorke LB, Zimmerman RA, Goldwein JW, Sutton LN; et al. (1992). "Vascular malformation with radiation vasculopathy after treatment of chiasmatic/hypothalamic glioma". Cancer. 70 (4): 887–93. PMID 1643622.
- ↑ Kestle JR, Hoffman HJ, Mock AR (1993). "Moyamoya phenomenon after radiation for optic glioma". J Neurosurg. 79 (1): 32–5. doi:10.3171/jns.1993.79.1.0032. PMID 8315466.