Unilateral adult malignant optic nerve glioma

Abstract

Unilateral adult malignant glioma of the optic nerve is exceptional. The final diagnosis was only confirmed by optic nerve biopsy. In the literature, only one patient has been reported with a unilateral tumour manifestation; he was lost to follow-up 3 months later. All other cases were bilateral. To date, 44 case reports of adult malignant optic nerve glioma have been published, either malignant astrocytoma or glioblastoma. These tumours can mimic optic neuritis in their initial presentation. The diagnosis is seldom made before craniotomy. On MRI images, malignant glioma cannot be distinguished from optic nerve enlargement due to other causes. Although radiotherapy appears to prolong life expectancy, all presently available treatment options (radiation, surgery, radio-chemotherapy) are of limited value. Most patients go blind and die within 1 or 2 years.

Malignant optic glioma – the spectrum of disease in a case series

“…Most authors did not comment on neuroradiologic findings of T2-weighted images in malignant optic glioma. In two more recent cases [5,15] T2 hyperintensity was described, whereas Wabbels et al [4] explicitly did not find T2 hyperintensity of the optic nerve and chiasm. Our case series supports T2 or FLAIR hyperintensity as a characteristic, albeit unspecific finding of malignant optic glioma, since it was evident in all five patients.…”

“…Neuroradiologic findings are unspecific, usually described as contrast enhancement and eventual thickening of the optic nerve, chiasm or tract in T1-weighted images [4,26], with iso-to hypointensity on native T1 images [27,28]. T2 hyperintensity of the affected anterior visual pathway is a matter of debate [27].…”

“…Recurrence or progression may be treated with re-resection, a second course of radiotherapy, or most commonly, using systemic alkylating agent chemotherapy or the VEGF antibody, bevacizumab [33]. The course of disease has not considerably improved over the last century [1], and malignant optic glioma remains lethal within one to two years [4]. The latter also holds true for our patients.…”

“…RESPONSE: Information about the referral centers has been added in this paragraph. (p. 4) COMMENT Also , the ages and sex of the patients should be mentioned here . RESPONSE:…”

“…)such as fluorescein angiography for example ? RESPONSE: This information has been added to the methods paragraph (p. [4][5] COMMENT Moreover always in this paragraph, if all patients have had radiotherapy and chemotherapy , the field of irradiation for radiotherapy and the doses for both technics should be precised .It should be interesting to know if there were differences in these technics according to the 3 centers . RESPONSE: The authors felt that discussion of radiotherapy and chemotherapy belongs to the result section and discussion sections rather than to the methods section.…”