Chordoid Glioma Infiltrating Optic Structures

Chordoid glioma of the third ventricle (CGTV) is a rare, slow-growing, World Health Organization Grade II glial tumor, with stereotyped localization in the anterior third ventricle. Despite being considered a noninvasive tumor, CGTV is usually associated with a poor clinical outcome due to its close proximity to important cerebral structures, such as the hypothalamus and visual pathways. Our patient with CGTV experienced visual involvement, but after subtotal surgical resection showed no evidence of progression at 5-year follow-up.

Photo Essay Section Editors: Melissa W. Ko, MD Dean M. Cestari, MD Chordoid Glioma Infiltrating Optic Structures Nicolae Sanda, MD, PhD, Claudiu-Nicolae Mircea, MD, Michèle Bernier, MD, PhD, Avinoam B. Safran, MD, Sorin Aldea, MD FIG. 1. Initial brain MRI. Axial T2 scan shows hyperintense mass in the suprasellar space (A), which demonstrates contrast enhancement on axial (B) and coronal (C) T1 images. The coronal view also reveals downward displacement of the chiasm (arrow). D. Postcontrast sagittal scan reveals the mass to be separate from the pituitary gland. Abstract: Chordoid glioma of the third ventricle (CGTV) is a rare, slow-growing, World Health Organization Grade II glial tumor, with stereotyped localization in the anterior third ventricle. Despite being considered a noninvasive tumor, CGTV is usually associated with a poor clinical outcome due to its close proximity to important cerebral structures, such as the hypothalamus and visual pathways. Our patient with CGTV experi- enced visual involvement, but after subtotal surgical resection showed no evidence of progression at 5-year follow-up. Journal of Neuro-Ophthalmology 2019;39:408-410 doi: 10.1097/WNO.0000000000000757 © 2019 by North American Neuro-Ophthalmology Society Sorbonne Universités (NS, ABS), UPMC Université Paris 06, UMR S968, Paris, France; Institut de la Vision (NS, ABS), Paris, France; Department of Clinical Neurosciences (NS, ABS), Geneva University School of Medicine, Geneva, Switzerland; Radiology Department (CNM), Hôpital Foch, Suresnes, France; Cytology and Pathology Department (MB), Hôpital Foch, Suresnes, France; and Neurosurgery Department (SA), Hôpital Foch, Suresnes, France. The authors report no conflicts of interest. Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Web site (www.jneuro-ophthalmology.com). The pathological data of this case were previously included in Bielle et al's (1) cohort. Address correspondence to Nicolae Sanda, MD, PhD, Department of Clinical Neurosciences, Geneva University Hospital, Gabrielle-PerretGentil 4, 1205 Geneva, Switzerland; E-mail: [e-mail] 408 Sanda et al: J Neuro-Ophthalmol 2019; 39: 408-410 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay FIG. 2. Pathology of chordoid glioma. A. There are clusters and cords of epithelial cells within a mucinous stain (hematoxylin & eosin, ·40). The tumor specimen shows positive staining for TTF-1 (B), CD34 (C), and GFAP (D) (B-D, ·20). A 39-year-old man with no significant medical history complained of headaches and sleep difficulty and was discovered to have bitemporal visual field defects. On examination, visual acuity was 20/20 in the right eye and 20/25 in the left eye, with a right relative afferent pupillary defect and bilateral optic disc pallor. Automated perimetry revealed a temporal defect in the right eye and a more diffuse inferotemporal defect in the left eye. Brain MRI revealed a relatively homogenous suprasellar mass in the anterior part of the third ventricle, which was T1 isointense and T2 hyperintense (Fig. 1). Endocrine evaluation demonstrated slightly diminished prolactin level and thyrotropin deficiency. The patient underwent subtotal tumor resection through right frontopterional craniotomy and trans-lamina terminalis approach. The tumor, a gray mass with a jelly-like consistency, was located in the anterior part of the third ventricle floor between infundibulum and optic chiasm. On the right side, the tumor displaced the anterior visual pathway laterally. On the left side, the tumor seemed to be more invasive. The excision was subtotal, leaving a residual fibrous tumor fragment adherent to the upper left side of the chiasm (See Supplemental Digital Content 1, Video, http://links.lww.com/WNO/A362). Pathological examination demonstrated typical features of chordoid glioma of the third ventricle (CGTV) with clusters and cords of epithelioid tumor cells within a mucinous stroma, containing a moderate plasma cell infiltrate (Fig. 2). Tumor cells expressed TTF1 (nuclear pattern), GFAP and CD34 antibodies (diffuse and strong cytoplasmic reactivity), negative immunostaining for NF, IDH1 R132, IDH2 R172, BRAFV600E, and a Ki-67 proliferative index of 2%-3%. The patient received no other treatment. After surgery, he developed cortisol deficiency, requiring substitution. Visual fields improved, showing less dense right temporal and left inferotemporal defects. Residual tumor remained stable on follow-up MRI imaging over a 5-year period (Fig. 3). CGTV is typically considered a limited, noninvasive tumor (2,3), although one report suggests that it may invade the optic chiasm (4). It is classified as World Health Organization Grade II glial tumor and presumed Sanda et al: J Neuro-Ophthalmol 2019; 39: 408-410 to originate in the organum vasculosum of the lamina terminalis (1). In our patient, the tumor seemed to involve right anterior visual pathway structures as a well delimited proliferation, and not diffuse tumor infiltration. The residual tumor, attached to the left side of the optic chiasm, may have represented direct tumor invasion or tumor-induced arachnoid proliferation, mimicking invasion, as described in optic nerve gliomas (5). The long-term stability of the residual tumor for 5 years after surgery in our patient and for 18 months after surgery in a report by Petrecca and Al Hinai (4) suggests that invasion of optic structures might not necessarily indicate a poor outcome. Due to its localization in the third ventricle, CGTV's clinical presentation commonly includes headaches, FIG. 3. Follow-up brain MRI. Postcontrast coronal and sagittal T1 scans at 18 months (A, B) and 5 years (C, D) demonstrate no change in the appearance of residual tumor (arrows). 409 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. Photo Essay ophthalmic findings (visual field defects, visual acuity loss, and papilledema), memory loss, and endocrine symptoms (2,6,7). MRI features (T1 isointensity, T2 hyperintensity, and contrast enhancing) are nonspecific and cannot differentiate CGTV from other suprachiasmatic tumors such as craniopharyngioma (7). CGTV does not have standardized treatment guidelines, but surgery often is the preferred approach (2,3). However, surgery has a particularly high mortality in the first year (up to 28%), especially after gross tumor resection. Mortality is mainly related to pulmonary embolism, infection, and cardiovascular causes (2,3). The reason for the high mortality rate is unknown but may be related to hypothalamic dysfunction (3,8,9). If there is tumor recurrence, it usually occurs soon after surgery (2,3). Radiotherapy after biopsy or partial resection has mixed results: from the 15 published cases with reported follow-up after biopsy or partial resection-6 relapsed. By contrast, of 12 published cases with partial resection and no radiotherapy, only 2 relapsed (2-4,7,9). Radiosurgery also has shown promising results (3). To the best of our knowledge, chemotherapy has not been administered in any of the reported cases. STATEMENT OF AUTHORSHIP Category 1: a. conception and design: N. Sanda, A. B. Safran, and S. Aldea; b. acquisition of data: N. Sanda, C. -N. Mircea, M. Bernier, A. B. Safran, and S. Aldea; c. analysis and interpretation of data: N. Sanda, C. -N. Mircea, M. Bernier, A. B. Safran, and S. Aldea. Category 2: a. drafting the manuscript: N. Sanda, C. -N. Mircea, and M. Bernier; b. revising it for intellectual content: A. B. Safran and S. Aldea. 410 Category 3: a. final approval of the completed manuscript: N. Sanda, C. -N. Mircea, M. Bernier, A. B. Safran, and S. Aldea. REFERENCES 1. Bielle F, Villa C, Giry M, Bergemer-Fouquet A-M, Polivka M, Vasiljevic A, Aubriot-Lorton M-H, Bernier M, Lechapt-Zalcman E, Viennet G, Sazdovitch V, Duyckaerts C, Sanson M, FigarellaBranger D, Mokhtari K. Chordoid gliomas of the third ventricle share TTF-1 expression with organum vasculosum of the lamina terminalis. Am J Surg Pathol. 2015;39:948-956. 2. DeSouza RM, Bodi I, Thomas N, Marsh H, Crocker M. Chordoid glioma: ten years of a low-grade tumor with high morbidity. Skull Base. 2010;20:125-138. 3. Kobayashi T, Tsugawa T, Hashizume C, Arita N, Hatano H, Iwami K, Nakazato Y, Mori Y. Therapeutic approach to chordoid glioma of the third ventricle. Neurol Med Chir. 2013;53:249-255. 4. Petrecca K, Al Hinai Q. Rarest of the rare: chordoid glioma infiltrating the optic chiasm. Surg Neurol Int. 2011;2:53. 5. Sanders GS, Allen RA, Straatsma BR. Arachnoidal proliferation of optic nerve simulating extension of intracranial glioma. A Case Rep Arch Ophthalmol. 1965;74:349-352. 6. Al-Zubidi N, McGlynn MM, Chévez-Barrios P, Yalamanchili S, Lee AG. Neuro-ophthalmologic features of chordoid glioma. J Neuroophthalmol. 2014;34:47-49. 7. Yang D, Xu Z, Qian Z, Wang L, Nie Q, Ge J, Qiu Y, Wang G. Chordoid glioma: a neoplasm found in the anterior part of the third ventricle. J Craniofac Surg. [published ahead of print July 13, 2017] doi: 10.1097/SCS.0000000000002514. 8. Nakajima N, Nakasu S, Hatsuda N, Takeichi Y, Watanabe K, Matsuda M. Third ventricular chordoid glioma: case report and review of the literature. Surg Neurol. 2003;59:424-428. 9. Erwood AA, Velazquez-Vega JE, Neill S, Solomon DA, Butowski N, Nowlan A, Dunbar E, Brat DJ. Chordoid glioma of the third ventricle: report of a rapidly progressive case. J Neurooncol. 2017;132:487-495. Sanda et al: J Neuro-Ophthalmol 2019; 39: 408-410 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.