The Architecture of Optic Nerve Gliomas With and Without Neurofibromatosis

The ecology of brain tumors: lessons learned from neurofibromatosis-1

“…Second, gliomas predominate in the optic pathway as opposed to the cerebellum, where they more commonly arise in the general population (Listernick et al, 1995). Third, NF1-associated optic gliomas arise at a younger age (mean ¼ 4.5-5.8 years) than in the general population (mean ¼ 5.1-12 years) (Stern et al, 1980;Listernick et al, 1989Listernick et al, , 1994Singhal et al, 2002;Thiagalingam et al, 2004). Fourth, unlike their sporadic counterparts, NF1-associated optic gliomas are less likely to progress and require treatment (Listernick et al, 1997).…”

Primary tumours of the optic nerve and its sheath

“…In patients with NF1, the tumour not only expands the optic nerve parenchyma but often breaks through the pia-arachnoid to fill both the subarachnoid and subdural spaces (Figure 4b). 10 However, the tumour almost always remains within the confines of the dural sheath of the optic nerve as long as it stays within the confines of the orbit or optic canal. Once it extends intracranially, however, it may remain primarily intraneural or develop a sizable exophytic component that in rare cases compresses the opposite optic nerve, optic chiasm, or both.…”

Teaching Neuro Images : Optic nerve glioma with perineural arachnoid gliomatosis in a patient with neurofibromatosis-1

“…The growth patterns of optic nerve gliomas are classified into intraneural and perineural forms. 1 The intraneural form is characterized by fusiform enlargement of the nerve, whereas PAG shows astrocytic proliferation in the subarachnoid space surrounding the relatively preserved optic nerve. 2 Rarely, both forms can coexist, as in this case.…”