Primary and secondary tumours of the optic nerve, with emphasis on invasion by uveal malignant melanoma

Abstract

Tumours of the optic nerve may originate in the optic nerve itself (i.e. primary tumours) or in structures adjacent to the optic nerve (i.e. secondary tumours). Furthermore, leukaemic infiltration and tumour metastases from distant solid tumours are also found in the optic nerve (i.e. secondary tumours). Large surveys have described primary optic nerve tumours and their treatment modalities, whereas secondary tumours have received little attention. Most reported work-ups have been based on material from referral centres and specialized departments.The correlation between invasion of the optic nerve in uveal melanoma and the risk of death from the disease has not been fully elucidated. The impact on prognosis may be correlated with tumour cells left in the proximal nerve after enucleation. In addition, melanoma with secondary invasion of the optic nerve may represent a highly malignant invasive subcategory of uveal melanoma. Clinical factors that make the ophthalmologist aware of optic nerve invasion when investigating a uveal melanoma are most important.Melanomas that invade the optic nerve may histopathologically display different invasive patterns and represent different types of uveal melanoma. A subtype of uveal melanoma with a preponderance for neural invasion may exist. Such a subtype has been identified for cutaneous melanoma (desmoplastic ⁄ neurotropic).In order to investigate tumours of the optic nerve in a known population, we studied all optic nerve tumours that had been surgically removed in Denmark over a period of 25 years. Furthermore, in order to resolve questions about secondary invasion of the optic nerve in uveal melanoma, we investigated all eyes with uveal melanoma and optic nerve invasion enucleated in Denmark between 1942 and 2001 (n ¼ 157).Tumours of the optic nerve in children were dominated by optic glioma and invasion from retinoblastoma, whereas tumours in adults mainly comprised optic nerve sheath meningioma and invasion from uveal melanoma.Optic nerve invasion of uveal melanoma was found in one in 20 patients. Increased IOP and juxtapapillary location were associated with both pre-laminar ⁄ laminar and post-laminar invasion of the optic nerve. Furthermore, ageReuse of this article is permitted in accordance with the Creative Commons Deed, Attribution 2.5, which does not permit commercial exploitation. Acta Ophthalmologica Scandinavica 20071 over 70 years, vision reduced to light perception, non-visible fundus and large tumour size were associated with post-laminar invasion. Only patients with optic nerve invasion had metastases to the CNS, kidney and heart. Histopathological features associated with optic nerve invasion were focal retinal invasion, neovascularization of the chamber angle and scleral invasion. Furthermore, non-spindle cell type and rupture of the ILM were all associated with post-laminar invasion.The optic nerve was invaded in four different ways:(1) by tumour extension from the neuroretina through the lamina cribrosa; (2) by direct extension into the optic ...

Primary and secondary tumours of the optic nerve, with emphasis on invasion of uveal malignant melanoma

“… Note from the Editor: This PhD thesis is published as an electronic supplement, see: Lindegaard J (2007): Primary and secondary tumors of the optic nerve, with emphasis on invasion of uveal malignant melanoma. Acta Ophthalmol Scand 85 (Suppl): 1‐22. …”

“…Lindegaard J (2007): Primary and secondary tumors of the optic nerve, with emphasis on invasion of uveal malignant melanoma. Acta Ophthalmol Scand 85 (Suppl): 1‐22.…”