The architecture of optic nerve gliomas with and without neurofibromatosis
J. Stern, F. A. Jakobiec and E. M. Housepian
A retrospective clinicopathologic study of 34 patients with optic nerve
glioma disclosed two important architectural tumor forms:
circumferential-perineural pattern featuring tumor eruption and
proliferation in the subarachnoid space correlating with the presence of
neurofibromatosis, and an expansile-intraneural pattern correlating with
the absence of neurofibromatosis. The 18 patients with neurofibromatosis
had a mean age of 4.9 years at presentation and tended to be younger than
the 16 patients without neurfibromatosis (mean age, 12 years). Instead of
"arachnoidal hyperplasia," electron microscopic studies in the electron
microscopic studies in the circumferential-perineural pattern showed tumor
astrocytes admixed with reactive meningothelial cells, fibroblasts, and
collagen in distended subarachnoid space. Florid invasion of the
leptomeninges (arachnoidal gliomatosis) exhibited by many optic nerve
gliomas in neurofibromatosis suggests that they are true neoplasms rather
than hamartomas, but their frequent location in the distal anterior visual
pathway often confers a good clinical prognosis.
