- Department of Pathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
- Department of Radiodiagnosis, Post Graduate Institute of Medical Education and Research, Chandigarh, India
- Department of Neurosurgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Rakesh K. Vasishta
Department of Pathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
DOI:10.4103/2152-7806.125777Copyright: © 2014 Bhaker P. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Bhaker P, Tyagi R, Mahajan D, Mohindra S, Vasishta RK. Optic nerve glioma: A great mimicker. Surg Neurol Int 23-Jan-2014;5:9
How to cite this URL: Bhaker P, Tyagi R, Mahajan D, Mohindra S, Vasishta RK. Optic nerve glioma: A great mimicker. Surg Neurol Int 23-Jan-2014;5:9. Available from: http://sni.wpengine.com/surgicalint_articles/optic-nerve-glioma-a-great-mimicker/
Background:Arachnoid proliferation, although rare, is known to occur in association with optic gliomas. However, chondroid and chordoid metaplasia has not been reported previously.
Case Description:A 27-year-old male presented with progressive, painless loss of vision in right eye, associated with vomiting and headache for one and a half months. Computed tomography (CT) scan revealed a contrast enhancing mass arising from planum sphenoidale. Perioperative findings showed the tumor adherent to the right optic nerve and attached to basal dura and falx. A clinical impression of an intradural, optic nerve sheath meningioma was made. Histopathological examination revealed a glial tumor with adjacent areas displaying marked fibroblastic and arachnoid cell proliferation with chondroid as well as chordoid differentiation along with myxoid change and dense collagenisation. Reticulin stain, immunochemistry with glial fibrillary acid protein (GFAP), epithelial membrane antigen (EMA), and S-100 helped to arrive at the final diagnosis of optic glioma displaying exuberant arachnoid proliferation with cartilaginous metaplasia.
Conclusion:We report a case of optic nerve glioma displaying extensive arachnoid proliferation, chordoid, and cartilaginous metaplasia, which mimicked chondrosarcoma or chordoid meningioma, posing a diagnostic dilemma. A clinical feedback, simple reticulin stain, and GFAP staining is of immense value in such cases to arrive at the correct diagnosis.
Keywords: Arachnoid hyperplasia, chondroid, chordoid, glioma, optic nerve
Optic nerve gliomas are rare tumors, accounting for 1.5-3% of orbital tumors, 1% of intracranial tumors, 1.7-7% of gliomas, and 3-5% of gliomas in children.[
History and Examination
A 27-year-old male presented with progressive, painless loss of vision in right eye, associated with vomiting and headache for one and a half months, without accompanying loss of consciousness or fever. He had a history of seizures 4 years back, for which he was on antiepileptic drugs. On clinical examination, there was no perception of light in the right eye while left eye vision was normal. Computed tomography (CT) scan revealed a contrast enhancing mass arising from planum sphenoidale with mass effect [
MRI Brain shows a heterogeneously hyperintense mass extending from planum sphenoidale and compressing both frontal lobes on T2WI sagittal images (a) with surrounding edema. The lesion shows heterogeneous postcontrast enhancement and extends to the right frontal sinus and anterior cerebral artery on T1W1 image with contrast (b)
Perioperative findings showed a grayish pink, nonsuckable tumor firmly adherent to the right optic nerve and expanding it. The tumor was also attached to basal dura and falx. Debulking surgery was performed. A clinical impression of an intradural, optic nerve sheath meningioma was made.
Histopathological examination of the excised tumor revealed a moderately cellular glial tumor intermixed with extensive reactive proliferation of arachnoid cells. The tumor cells were present in a fibrillary background and exhibited minimal nuclear pleomorphism. No mitosis, necrosis or micro-vascular proliferation was noted. Adjacent to the tumor, there was marked fibroblastic and arachnoid cell proliferation with chondroid as well as chordoid differentiation along with myxoid change and dense collagenisation. The sharply outlined tumor was reticulin poor while the adjacent reactive areas were rich in reticulin fibers. On immunohistochemistry using Dako antibody kit, the tumor cells expressed glial fibrillary acid protein (GFAP). [
Photomicrograph showing (a) Islands of gliomatous component scattered among areas of marked fibroblastic proliferation (H and E, ×100); (b) Areas of cartilaginous and chordoid metaplasia (H and E, ×200). Inset showing positivity for S-100 in cartilaginous areas; (c) Sharp demarcation between reticulin poor gliomatous component and reticulin rich fibroblastic area (Reticulin stain, ×100); (d) Glioma showing strong positivity for GFAP (×40)
Treatment and Follow-up
Postoperatively, there was no improvement in the vision. Recurrence of tumor was noted after a period of four months and debulking surgery was repeated. Histopathological examination confirmed the presence of glioma (grade I) having similar morphology as the previous tumor displaying extensive fibro-mesenchymal proliferation of the meninges. The patient is on regular follow-up, however, there is still no improvement in the vision.
Optic nerve gliomas account for 1.7-7% of gliomas.[
Arachnoidal cell proliferation causing distension of the perineural space is a common observation in optic gliomas and there have been case reports where glioma has been mistaken for meningioma due to such marked proliferation of meningothelial cells.[
Optic nerve gliomas are usually low grade tumors with variable clinical course. Arachnoidal hyperplasia is a common phenomenon associated with optic gliomas. However, chordoid and chondroid metaplasia has not been reported previously. Clinicoradiological consultation, simple reticulin stain and immunohistochemistry can be of great help in such a situation to distinguish these tumors from a chondrosarcoma or chordoid meningioma.
1. Ahn Y, Cho BK, Kim SK, Chung YN, Lee CS, Kim IH. Optic pathway glioma: Outcome and prognostic factors in surgical series. Childs Nerv Syst. 2006. 22: 1136-42
2. Cooling RJ, Wright JE. Arachnoid hyperplasia in optic nerve glioma: Confusion with orbital meningioma. Br J Ophthal. 1979. 63: 596-9
3. Dutton JJ. Gliomas of the anterior visual pathway. Surv Ophthalmol. 1994. 38: 427-52
4. Karp LW, Zimmerman LE, Borit A, Spencer WH. Primary intraorbital meningiomas. Arch Ophthalmol. 1974. 91: 24-8
5. King A, Listernick R, Charrow J, Piersall L, Gutmann DH. Optic pathway gliomas in neurofibromatosis type 1: The effect of presenting symptoms on outcome. Am J Med Genet A. 2003. 122: 95-9
6. Miller NM. Primary tumours of the optic nerve and its sheath. Eye. 2004. 18: 1026-37
7. Thompson CR, Lessell S. Anterior visual pathway gliomas. Int Ophtalmol Clin. 1997. 37: 261-79
8. Wilhelm H. Primary optic nerve tumours. Curr Opin Neurol. 2009. 22: 11-8