Optic nerve glioma natural history, complications and prognosis
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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
If left untreated, less than 5% of patients with optic nerve gliomas may progress to develop blindness. The clinical course of optic nerve gliomas is unpredictable.[1] Common complications of optic nerve glioma include decreased vision, blindness, growth hormone deficiency, precocious puberty, and hydrocephalus. Prognosis is generally good in most patients with optic pathway gliomas. Most optic nerve gliomas are benign and produce slowly progressive visual loss associated with variable proptosis and anterior or posterior optic neuropathy.
Natural History
- Optic nerve gliomas have an unpredictable natural history.[2]
- The majority of the optic nerve gliomas grow slowly in a self limited manner and some of them spontaneously regress.[3]
- If left untreated, less than 5 percentage of patients with optic nerve gliomas may progress to develop blindness. Significant visual impairment is frequently present in neurofibromatosis type 1 patients with optic nerve gliomas.[4][5]
- These tumors demonstrate variable clinical and radiological progression. In patients with neurofibromatosis type 1, it is not unusual for these tumors to be quiescent, with little progression demonstrated over a number of years. In others, the tumors are more aggressive with extension along the optic pathways.[6]
- Normally, the growth of the tumor is very slow, and the condition remains stable for long periods of time. However, in adults and some children, when the optic chiasm is involved, the tumor is more aggressive.
- In adults, the clinical course of an optic nerve gliomas resembles the behavior of the tumors's histology, which is commonly a malignant glioma. Malignant optic nerve gliomas can result in rapidly progressive visual loss. Rapid onset blindness is seen in elderly and middle-aged individuals.[7][8][9]
- Death can occur with in few months of presentation if tumor invasion into adjacent brain occurs.[10]
Complications
Complications of optic nerve gliomas include:
- Decreased vision
- Blindness
- Hydrocephalus
- Growth hormone deficiency
- Precocious puberty
Prognosis
- There is nearly 90 percent survival rate for all optic pathway gliomas whether those with or without neurofibromatosis type 1.
- Children with neurofibromatosis and older children have a better prognosis.
- In patients with optic nerve gliomas associated with neurofibromatosis type 1, beter prognosis is seen.
- Spontaneous remission of the optic pathway gliomas is seen in two-thirds of children with neurofibromatosis type 1.
- Metastatic dissemination through ventriculoperitoneal shunts, malignant degeneration, and spontaneous regression have all been reported.
- Patients with chiasmal gliomas have usually less favorable prognosis.
- Patients with neurofibromatosis type 1 have appoximately twice the recurrence rate following complete excision of an intraorbital glioma as compared with patients without neurofibromatosis type 1.
- Approximately sixty percentage of children treated with chemotherapy for gliomas of the hypothalamus and optic pathways had a relapse.[11]
| Prognosis | Age at onset | Clinical features | Radiographic location |
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Early childhood to adolescence |
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Infancy to early childhood and adulthood |
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References
- ↑ Shuper A, Horev G, Kornreich L, Michowiz S, Weitz R, Zaizov R; et al. (1997). "Visual pathway glioma: an erratic tumour with therapeutic dilemmas". Arch Dis Child. 76 (3): 259–63. PMC 1717103. PMID 9135269.
- ↑ Schupper A, Kornreich L, Yaniv I, Cohen IJ, Shuper A (2009). "Optic-pathway glioma: natural history demonstrated by a new empirical score". Pediatr Neurol. 40 (6): 432–6. doi:10.1016/j.pediatrneurol.2008.12.015. PMID 19433276.
- ↑ Parsa CF, Hoyt CS, Lesser RL, Weinstein JM, Strother CM, Muci-Mendoza R; et al. (2001). "Spontaneous regression of optic gliomas: thirteen cases documented by serial neuroimaging". Arch Ophthalmol. 119 (4): 516–29. PMID 11296017.
- ↑ Dalla Via P, Opocher E, Pinello ML, Calderone M, Viscardi E, Clementi M; et al. (2007). "Visual outcome of a cohort of children with neurofibromatosis type 1 and optic pathway glioma followed by a pediatric neuro-oncology program". Neuro Oncol. 9 (4): 430–7. doi:10.1215/15228517-2007-031. PMC 1994100. PMID 17704361.
- ↑ Moreno L, Bautista F, Ashley S, Duncan C, Zacharoulis S (2010). "Does chemotherapy affect the visual outcome in children with optic pathway glioma? A systematic review of the evidence". Eur J Cancer. 46 (12): 2253–9. doi:10.1016/j.ejca.2010.03.028. PMID 20400294.
- ↑ Optic nerve glioma. Radiopedia(2015) http://radiopaedia.org/articles/optic-nerve-glioma Accessed on October 5 2015
- ↑ Spoor TC, Kennerdell JS, Zorub D, Martinez AJ (1981). "Progressive visual loss due to glioblastoma: normal neuroroentgenorgraphic studies". Arch Neurol. 38 (3): 196–7. PMID 7469853.
- ↑ Spoor TC, Kennerdell JS, Martinez AJ, Zorub D (1980). "Malignant gliomas of the optic nerve pathways". Am J Ophthalmol. 89 (2): 284–92. PMID 6243868.
- ↑ Dario A, Iadini A, Cerati M, Marra A (1999). "Malignant optic glioma of adulthood. Case report and review of the literature". Acta Neurol Scand. 100 (5): 350–3. PMID 10536925.
- ↑ Taphoorn MJ, de Vries-Knoppert WA, Ponssen H, Wolbers JG (1989). "Malignant optic glioma in adults. Case report". J Neurosurg. 70 (2): 277–9. doi:10.3171/jns.1989.70.2.0277. PMID 2643688.
- ↑ 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.