Malignant optic glioma in adults

Abstract

Malignant optic glioma in adulthood is a rare tumor that causes early loss of vision and nearly always leads to death within a year. A case history is presented illustrating the clinical and neuroradiological characteristics of the malignant optic glioma in adults. A review of the literature is given.

Unilateral adult malignant optic nerve glioma

“…Until now, 44 cases have been described, not including the present case [1,3,4,5,6,7,9,10,11,12,13,14,15,16,17,18,20,22,23,25,26,27,28,29]. They are summarised in Table 1.…”

Primary tumours of the optic nerve and its sheath

“…In some cases, the optic nerve appears diffusely thickened, and MR imaging after intravenous injection of a paramagnetic contrast agent may show marked enhancement of the nerve with inhomogeneity and cystic-appearing areas (Figure 5b). 15,16 The pathologic features of a malignant optic nerve glioma are characteristic. [14][15][16] The vascular and partially necrotic tumour occupies most of the nerve.…”

“…15,16 The pathologic features of a malignant optic nerve glioma are characteristic. [14][15][16] The vascular and partially necrotic tumour occupies most of the nerve. In the orbit, it usually infiltrates the meninges of the nerve and the surrounding soft tissue, whereas intracranially, it eventually infiltrates the optic chiasm, hypothalamus, and adjacent parts of the brain.…”

Imaging of the normal and pathological orbit

“…The tumor shows early growth, followed by stability in many patients; if the lesion is initially confined to the optic nerve alone, overall mortality is low (5 %), whereas it rises to about 50 % in cases of involvement of the hypothalamus [21]. Optic nerve gliomas are more frequent than meningiomas (ratio 4 : 1) [22,23]. There is no significant sex prevalence.…”

“…Malignant optic glioma is a distinct disease, which occurs in middleaged adults [21,22]. The clinical presentation depends on the location of the tumor; in cases of intraorbital optic nerve glioma, there is usually painless proptosis, and optic atrophy is also frequent [12].…”