Abstract
Peripheral nerve sheath tumors of the uveal tract arise mainly in the sheath of peripheral nerves that are found in the uvea. The ciliary nerve is most often affected and hence these tumors tend to be located anteriorly, in the ciliary body or peripheral choroid. There are several systemic predisposing risk factors, which include schwannomatosis, neurofibromatosis 1 (NF1) and 2, and other rare genetic syndromes. The benign tumors can be subclassified as neurofibroma and schwannoma. The malignant nerve sheath tumors are more difficult to subclassify if they lose the histological characteristics of their benign counterparts. In this chapter we discuss uveal neurofibroma and schwannoma.
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References
Shields JA, Sanborn GE, Kurz GH, Augsburger JJ. Benign peripheral nerve tumor of the choroid: a clinicopathologic correlation and review of the literature. Ophthalmology. 1981;88(12):1322–9.
Burke JP, Leitch RJ, Talbot JF, Parsons MA. Choroidal neurofibromatosis with congenital iris ectropion and buphthalmos: relationship and significance. J Pediatr Ophthalmol Strabismus. 1991;28(5):265–7.
Woog JJ, Albert DM, Craft J, et al. Choroidal ganglioneuroma in neurofibromatosis. Graefes Arch Clin Exp Ophthalmol. 1983;220(1):25–31.
Brownstein S, Little JM. Ocular neurofibromatosis. Ophthalmology. 1983;90(12):1595–9.
Yasunari T, Shiraki K, Hattori H, Miki T. Frequency of choroidal abnormalities in neurofibromatosis type 1. Lancet. 2000;356(9234):988–92.
Lewis RA, Riccardi VM. Von Recklinghausen neurofibromatosis. Incidence of iris hamartomata. Ophthalmology. 1981;88(4):348–54.
Fan JT, Campbell RJ, Robertson DM. A survey of intraocular schwannoma with a case report. Can J Ophthalmol. 1995;30(1):37–41.
Wei WB, Jie Y, Mo J, Li B. Clinical characteristics and treatment of neurofibroma of the choroid. Chin Med J (Engl). 2012;125(10):1832–5.
Smith PA, Damato BE, Ko MK, Lyness RW. Anterior uveal neurilemmoma–a rare neoplasm simulating malignant melanoma. Br J Ophthalmol. 1987;71(1):34–40.
Kurtkaya-Yapicier O, Scheithauer B, Woodruff JM. The pathobiologic spectrum of Schwannomas. Histol Histopathol. 2003;18(3):925–34.
You JY, Finger PT, Iacob C, et al. Intraocular schwannoma. Surv Ophthalmol. 2013;58(1):77–85.
Reith JD, Goldblum JR. Multiple cutaneous plexiform schwannomas. Report of a case and review of the literature with particular reference to the association with types 1 and 2 neurofibromatosis and schwannomatosis. Arch Pathol Lab Med. 1996;120(4):399–401.
Freedman SF, Elner VM, Donev I, et al. Intraocular neurilemmoma arising from the posterior ciliary nerve in neurofibromatosis. Pathologic findings. Ophthalmology. 1988;95(11):1559–64.
MacCollin M, Willett C, Heinrich B, et al. Familial schwannomatosis: exclusion of the NF2 locus as the germline event. Neurology. 2003;60(12):1968–74.
Antinheimo J, Sankila R, Carpen O, et al. Population-based analysis of sporadic and type 2 neurofibromatosis-associated meningiomas and schwannomas. Neurology. 2000;54(1):71–6.
Carney JA. The Carney complex (myxomas, spotty pigmentation, endocrine overactivity, and schwannomas). Dermatol Clin. 1995;13(1):19–26.
Venturini G, Moulin AP, Deprez M, et al. Clinicopathologic and molecular analysis of a choroidal pigmented schwannoma in the context of a PTEN hamartoma tumor syndrome. Ophthalmology. 2012;119(4):857–64.
Matsuo T, Notohara K. Choroidal schwannoma: immunohistochemical and electron-microscopic study. Ophthalmologica. 2000;214(2):156–60.
Saavedra E, Singh AD, Sears JE, Ratliff NB. Plexiform pigmented schwannoma of the uvea. Surv Ophthalmol. 2006;51(2):162–8.
Lee SH, Hong JS, Choi JH, Chung WS. Choroidal schwannoma. Acta Ophthalmol Scand. 2005;83(6):754–6.
Shields JA, Font RL, Eagle Jr RC, et al. Melanotic schwannoma of the choroid. Immunohistochemistry and electron microscopic observations. Ophthalmology. 1994;101(5):843–9.
Xian J, Xu X, Wang Z, et al. MR imaging findings of the uveal schwannoma. AJNR Am J Neuroradiol. 2009;30(4):769–73.
Kuchle M, Holbach L, Schlotzer-Schrehardt U, Naumann GO. Schwannoma of the ciliary body treated by block excision. Br J Ophthalmol. 1994;78(5):397–400.
Goto H, Mori H, Shirato S, Usui M. Ciliary body schwannoma successfully treated by local resection. Jpn J Ophthalmol. 2006;50(6):543–6.
Cho YJ, Won JB, Byeon SH, et al. A choroidal schwannoma confirmed by surgical excision. Korean J Ophthalmol. 2009;23(1):49–52.
Turell ME, Hayden BC, McMahon JT, et al. Uveal schwannoma surgery. Ophthalmology. 2009;116(1):163–e6.
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Cohen, V., Damato, B., Singh, A.D. (2014). Uveal Neural Tumors. In: Damato, B., Singh, A. (eds) Clinical Ophthalmic Oncology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-54255-8_24
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DOI: https://doi.org/10.1007/978-3-642-54255-8_24
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