Large rhabdoid meningioma presenting prominent hyperintensity in the optic nerve: An indicator of visual disturbance on constructive interference steady-state sequence?
- Department of Neurological Surgery, Juntendo University Urayasu Hospital, Urayasu, Japan.
- Department of Pathology, Juntendo University Urayasu Hospital, Urayasu, Japan.
Satoshi Tsutsumi, Department of Neurological Surgery, Juntendo University Urayasu Hospital, Urayasu, Japan.
DOI:10.25259/SNI_364_2023Copyright: © 2023 Surgical Neurology International This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.
How to cite this article: Kasumi Inami1, Satoshi Tsutsumi1, Akane Hashizume2, Kohei Yoshida1, Natsuki Sugiyama1, Hideaki Ueno1, Hisato Ishii1. Large rhabdoid meningioma presenting prominent hyperintensity in the optic nerve: An indicator of visual disturbance on constructive interference steady-state sequence?. 14-Jul-2023;14:248
How to cite this URL: Kasumi Inami1, Satoshi Tsutsumi1, Akane Hashizume2, Kohei Yoshida1, Natsuki Sugiyama1, Hideaki Ueno1, Hisato Ishii1. Large rhabdoid meningioma presenting prominent hyperintensity in the optic nerve: An indicator of visual disturbance on constructive interference steady-state sequence?. 14-Jul-2023;14:248. Available from: https://surgicalneurologyint.com/surgicalint-articles/12425/
Background: Rhabdoid meningiomas (RMs) are a rare type of malignant meningioma. Here, we report a case of intracranial RM presenting with visual disturbance and prominent hyperintensity in the optic nerve (ON).
Case Description: A 20-year-old female presented with a 1-year history of headache. At presentation, her visual acuity (VA) was 20/50 on the right side and 20/40 on the left, with an intraocular pressure of 17 mmHg on both sides. Cerebral magnetic resonance imaging revealed a broad-based tumor in the right frontal convexity. It measured 82 mm × 65 mm × 70 mm in diameter, accompanied by cystic components, and was inhomogeneously enhanced. The intraorbital ONs demonstrated prominent intramedullary hyperintensity on the constructive interference steady-state sequence. Gross total tumor resection was performed and the pathology was consistent with RM. Immediately after surgery, her VA and IOP were 20/17 and 10 mmHg, respectively, with a remarkable resolution of the intramedullary hyperintensity.
Conclusion: Prominent hyperintensity in the ON identified in patients with chronic intracranial hypertension may be an indicator of visual disturbance. It can rapidly resolve after resolution of intracranial hypertension with functional recovery.
Keywords: Chronic intracranial hypertension, Optic nerve, Intramedullary hyperintensity, Rhabdoid meningioma, Visual disturbance
Meningiomas are the most common primary brain tumors in adulthood. Most of them are benign tumors and classified as the World Health Organization (WHO) Grade I. The epidemiology and treatment outcomes are reported to differ among children/adolescents (0–21 years), young adults (22–45 years), and older adults (>45 years).[
Studies have suggested that hyperintense areas in the optic nerves (ONs) and tracts that can be detected using T2-weighted imaging, constructive interference steady-state (CISS) sequences, and contrast-enhanced fluid-attenuated inversion recovery imaging may be important for maintaining normal visual function.[
Here, we report a 20-year-old woman with intracranial RM who presented with visual disturbances and prominent CISS hyperintensity in the intraorbital ONs that rapidly resolved following tumor resection.
A 20-year-old woman without neurofibromatosis presented with a 1-year history of headaches. At presentation, the patient was well oriented and did not show any focal neurological deficits. Ophthalmological examination revealed papilledema in both the optic fundi [
Axial computed tomography, bone-target image (a) and T1- (b), T2- (c), and postcontrast axial (d) T1-weighted magnetic resonance images at the same level showing a large tumor (T) in the right frontal lobe. The tumor has a broad-based attachment to the convexity dura mater. It appears heterogeneous intensity both on T1- and T2-weighted sequences, accompanied by focal erosion in the adjacent skull (a, arrow) and cyst components (b and c, asterisk), and inhomogeneously enhanced (d). No apparent dural tail is identified.
Photomicrographs of the resected specimens showing tumor tissue comprised of rhabdoid-shaped, anaplastic cells with pleomorphism and intervening necrotic foci (a, asterisks). Immunohistochemical analysis show positive staining for vimentin (b), S100 protein (c), progesterone receptor (d), and glial fibrillary acidic protein (e). The MIB-1 index accounts for 50% (f). (a) Hematoxylin and eosin stain, ×200; (b-d, f) ×400; (e) ×40.
In this case, the prominent hyperintensity identified in the ONs presented simultaneously with long-term headache, bilateral papilledema, and visual disturbance, suggesting chronic intracranial hypertension. The offending tumor was large and occupied considerable space in the intracranial cavity. Furthermore, the intramedullary hyperintensity rapidly resolved after tumor resection, with remarkable improvement in visual function and decrease in IOP. Therefore, we assumed that the hyperintensity reflected the visual function both in before and after surgery. Recently, inactivation of the breast cancer-associated protein-1 tumor suppressor gene, BAP1, has been proposed to be the most common mutation associated with an aggressive nature of RMs.[
In our patient, the width of the right ON decreased postoperatively. This probably indicates long-term, indirect tumor compression of the intracranial right ON, and consequent optic atrophy. Despite this, the visual disturbance improved immediately after surgery. Such intramedullary hyperintensity may serve as a radiological biomarker for indicating a prompt surgical intervention. The diameter of the OS did not show immediate shrinkage, consistent with the results of previous report.[
Prominent peritumoral edema, cystic components, and bone involvement are documented as characteristic neuroimaging findings of RMs.[
Prominent hyperintensity in the ON identified in patients with chronic intracranial hypertension may be an indicator of visual disturbance. It can rapidly resolve after resolution of intracranial hypertension with functional recovery.
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There are no conflicts of interest.
The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.
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