High-grade spheno-orbital meningioma in patients with systemic lupus erythematosus: Two case reports and literature review
- Department of Neurosurgery, National Neuroscience Institute, King Fahad Medical City, Riyadh, Saudi Arabia,
- College of Medicine, King Saud University, Riyadh, Saudi Arabia,
- Department of Pathology and Clinical Laboratory Medicine, King Fahad Medical City, Riyadh, Saudi Arabia.
Department of Neurosurgery, National Neuroscience Institute, King Fahad Medical City, Riyadh, Saudi Arabia,
DOI:10.25259/SNI_583_2020Copyright: © 2020 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, tweak, 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: Sarah Bin Abdulqader1, Nasser Almujaiwel2, Wafa Alshakweer3, Gmaan Alzhrani1. High-grade spheno-orbital meningioma in patients with systemic lupus erythematosus: Two case reports and literature review. 29-Oct-2020;11:367
How to cite this URL: Sarah Bin Abdulqader1, Nasser Almujaiwel2, Wafa Alshakweer3, Gmaan Alzhrani1. High-grade spheno-orbital meningioma in patients with systemic lupus erythematosus: Two case reports and literature review. 29-Oct-2020;11:367. Available from: https://surgicalneurologyint.com/surgicalint-articles/10355/
Background: Spheno-orbital meningiomas (SOMs) are often benign. The association of meningioma and systemic lupus erythematosus (SLE) is rarely discussed in the literature. Here, we report two patients with high-grade, SOMs with a prolonged history of SLE and review the literature.
Case Description: The first case is a 52-year-old female patient with a 15-year history of SLE diagnosis who was referred to our center with a 1-year history of proptosis and excessive tearing of the left eye. This patient was operated for the left SOM with histopathological diagnosis of the World Health Organization (WHO) Grade III rhabdoid meningioma. The second case is a 36-year-old female patient with a 12-year history of SLE diagnosis who presented to our clinic with a 5-year-history of progressive right eye proptosis and occasional headaches. She was operated for the right SOM with histopathological diagnosis of the WHO Grade II chordoid meningioma.
Conclusion: Rhabdoid and chordoid SOMs are uncommon and no previous report discussed their occurrence in patients with SLE. The association of high-grade meningiomas and SLE deserves further exploration.
Keywords: Chordoid meningioma, Rhabdoid meningioma, Spheno-orbital meningioma, Systemic lupus erythematosus
Spheno-orbital meningioma (SOM) is a complex and unique pathological condition that accounts for 9% of all intracranial meningiomas.[
Systemic lupus erythematosus (SLE) is an autoimmune, multiorgan, connective tissue disease with diverse pathogenesis and unexplored etiology that frequently affects women.[
In this paper, we describe two patients who were diagnosed with SLE and referred to us for the surgical treatment of SOM. These patients exhibited uncommon histopathological variants of SOM.
A 52-year-old female patient with a 15-year history of SLE diagnosis was referred to our center with a 1-year history of proptosis and excessive tearing of the left eye. On examination, she was found to have a visual acuity of 20/20 in the right eye and 20/25 in the left eye, with intact extraocular movement and facial sensation. She had been on a regimen of azathioprine therapy to manage her SLE.
Radiological imaging showed left SOM with extension into the superior orbital fissure, left cavernous sinus, and left petrous apex. In addition, there was a small extension toward the left cerebellopontine angle (CPA) with significant hyperostosis of the sphenoid bone and lateral orbital wall [
Case 1. (a and b) Preoperative T1-weighted postgadolinium administration MR images demonstrating left SOM with extension into the superior orbital fissure, left cavernous sinus, left petrous apex, and left cerebellopontine angle (CPA). (c) Preoperative CT head bone window demonstrating hyperostosis. (d) Postoperative T1-weighted postgadolinium administration MR images demonstrating postoperative changes as well as residual lesion at the left cavernous sinus and CPA.
The patient was subsequently operated through the left pterional approach. The histopathological examination established a diagnosis of the World Health Organization (WHO) Grade III rhabdoid meningioma (RM) [
A 36-year-old female patient with a 12-year history of SLE diagnosis presented to our clinic with a 5-year history of progressive right eye proptosis and occasional headaches. On examination, she was found to have right eye exophthalmos with normal visual acuity, as well as intact extraocular motility and normal facial sensation bilaterally. She was on a regimen of hydroxychloroquine to manage her lupus. In addition, she used steroids, which was discontinued a year ago because of remission.
Radiological imaging findings showed right SOM with extension into the orbit and cavernous sinus [
Case 2. (a) Preoperative T1-weighted postgadolinium administration MR images demonstrating right SOM extending into the right orbit and cavernous sinus. (b) Preoperative CT head demonstrating hyperostosis. (c) Postoperative T1-weighted postgadolinium administration MR images demonstrating intraorbital residual lesion approaching the superior orbital fissure.
Right frontotemporal craniotomy was performed for tumor resection. The histopathological examination established a diagnosis of the WHO Grade II chordoid meningioma (CM) [
Literature review methods
To investigate for previous reports of cases of meningioma occurring in patients with SLE, we searched the PubMed database using the following terms; meningioma AND “systemic lupus erythematosus,” meningioma AND “autoimmune disease,” and meningioma AND “connective tissue disease.” Data on age, sex, time of meningioma diagnosis, location of meningioma, histopathological grade of meningioma, and medications used for the treatment of SLE were collected for each case.
Literature review results
Seven cases were identified and were available for review in addition to one case that was retrieved through cited references. Ten cases were analyzed [including our two cases;
This report describes two rare histopathological variants of SOM that occurred in patients with a prolonged history of SLE. The association of meningioma and SLE has been discussed earlier;[
Meningioma is the most common primary intracranial tumor in adults, accounting for one-third of all primary intracranial tumors, with a female-to-male ratio of 2:1; and most patients have low-grade meningioma.[
RM is an uncommon variant of meningioma, which represents 0.28% of all meningioma;[
CM was additionally introduced by Kepes et al.[
Meningiomas of the cranial base show an indolent growth pattern and are often classified as low-grade meningiomas as compared with its noncranial base counterparts. The low-grade nature of these meningiomas has been theoretically attributed to the fact that most cranial base meningiomas present earlier; thus, cases of aggressive or malignant transformation are less likely to occur due to early intervention.[
High-grade SOMs, specifically RMs and CMs, are rare. Various case series have discussed the natural history and outcomes of SOMs and demonstrated that Grade I meningiomas occur in 78%,[
An association between SOM and other medical conditions, specifically, hypothyroidism has been identified;[
Prevailing theories have proposed an association between malignancies and SLE, including relatively high expression of interleukin 6 (IL-6) and IL-10 in patients with non-Hodgkin lymphoma and in patients with SLE.[
The role of estrogen and progesterone receptors in the development of meningioma is well established. This was evident by accelerated growth of meningiomas during pregnancy and in postmenopausal women receiving exogenous hormone therapy.[
From the current literature, we identified eight cases of meningiomas that were reported in patients with SLE. [
This report provides an insight into the possible attribution of SLE to the development of high-grade meningiomas. We report an unusual association between rare histopathological entities of SOM and SLE. The use of specific medications to manage SLE as well as the overexpression of IL-6 observed in both CM and SLE might play a role in the pathogenesis of high-grade (rhabdoid and chordoid) SOM. However, further epidemiological and genetic studies are needed to validate this association. Moreover, a coincidental association cannot be ruled out given that both meningioma and SLE are common disease conditions.
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