Case Report

Spinal Clear Cell Meningioma without Dural Attachment: Case Report and Review of Literature

Kais Maamri¹*, Mohamed Amine Hadj Taieb¹, Ghassen Elkahla¹, Maher Hadhri¹, Souheil Khalfaoui¹, Manel Njima², Beya Soula¹ and Mehdi Darmoul¹

¹Department Neurosurgery, Faculty of Medicine of Monastir, Fatouma Bourguiba Hospital, Tunisia

²Department of Pathology, Faculty of Medicine of Monastir, Fatouma Bourguiba Hospital, Tunisia

Submission: July 8, 2020; Published: November 05, 2020

*Corresponding author: Kais Maamri, Department Neurosurgery, Fatouma Bourguiba Hospital, Faculty of Medicine of Monastir, Monastir, Tunisia

How to cite this article: Kais M, Mohamed A H T, Ghassen E, Maher H, Souheil K, et al. Spinal Clear Cell Meningioma without Dural Attachment: Case Report and Review of Literature. Open Access J Neurol Neurosurg 2020; 14(3): 555889.DOI: 10.19080/OAJNN.2020.14.555889.

Abstract

Background: Clear Cell Meningioma (CCM) is a very rare histologic subtype of meningioma usually affecting younger patient. The reported data on spinal CCMs are extremely rare. Furthermore, only few cases without dural attachment have been reported in literature.

Methods: We report a case of a 58-year-old female patient who was presented to our department with pain in her lower back and bilateral sciatica for 6 months. Magnetic resonance imaging (MRI) showed an intra-dural well demarcated lesion at L3. The mass had an iso-intense signal on both T1 and T2 weighted images with intense and homogeneous gadolinium enhancement.

Result: Via a posterior approach, a total resection was possi¬ble due to lack of dural adhesion of the tumor. Histologic diagnosis was clear cell meningioma. Patient’s recovery course after the operation was uneventful.

Conclusion: Gross total resection is recommended in treating spinal CCM whenever possible. However, radiotherapy could be considered for patients who have undergone STR or younger patients, regardless of the extent of resection.

Keywords:Clear cell; Meningioma; Spine; Dural attachment; Lumbar

Introduction

Clear Cell Meningioma (CCM) is a rare histologic subtype of meningioma. It accounts for less than 1% of all meningiomas [1]. Compared to ordinary meningiomas, CCMs have higher recurrence rate (~50%) and higher tendency to metastasize (4.1%) [2,3]. For these reasons, the World Health Organization (WHO) classification system has classified them as grade II tumors. Among the few CCMs’ reported cases, the majority of them had an intracranial location. Intra-spinal CCMs were even rarer since only less than 100 cases have been reported until 2019 [4]. Most meningiomas are attached to the dura mater, therefore it is exceptional to witness a meningioma without any dural attachment. To the day we speak, only 19 cases of non-dura attached spinal CCM have been reported [4]. In this article, we present an unusual case of a spinal CCM characterized by the absence of dural attachment

Case Report

A 58-year-old female patient was admitted with complaints of low back pain and bilateral sciatica. Her symptoms started 6 months before her admission and have worsened over time. The patient took a symptomatic treatment without any amelioration. On examination, her general condition as well as her cardiorespiratory system was normal. The neurologic examination did not reveal any specific signs except a classical lumbar spinal syndrome. Nevertheless, it is important to mention that there was no neurologic deficit. A provisional clinical diagnosis of lumber disc herniation was made and a Magnetic Resonance Imaging (MRI) was suggested. The MRI showed an intra-dural lesion at L3 measuring 31 mm of height. The mass was well demarcated and had an isointense signal on both T1-and T2-weighted images with intense and homogeneous gadolinium enhancement (Figure1). There was no foraminal extension and no associated bone destruction.

Based on the clinical condition of the patient and the MRI findings, we decided to operate the patient. We started with a L2-L3 laminectomy. The intra-dural exposure revealed an elliptic, encapsulated yellow mass of firm consistency. The tumor was found to be draped by and adhered to the nerve roots without any dural attachment. After an easy dissection, we managed to perform a total resection. On microscopic examination (Figure 2), the tumor was composed of layers of polygonal cells with a clear glycogenrich cytoplasm and monomorphic non-mitotic nuclei. There were prominent perivascular and interstitial collagen. Whorl formation and psammoma bodies were absent. The pathological diagnosis was a CCM (WHO II). The patient’s postoperative course was uneventful. All of her symptoms did disappear and she was able to get up the first day after the operation. A postoperative MRI was scheduled but due to the exceptional situation of COVID19 pandemic, the patient was given an appointment in late 2020 as her case wasn’t judged as urgent.

Discussion

First reported by Manivel and Sung [5] in 1990, CCM is one of the rarest histologic subtype of meningiomas, representing 0.2-0.8% [6-8]. Zorludemir et al. (6) & Oviedo et al. [7] found that this subtype of meningiomas had a higher local recurrence rate and a more aggressive clinical course compared to ordinary meningiomas. These differences led to the classification of a grade II type tumor in 2016 [9]. Around 300 cases of CCMs are reported in the literature, most of them had an intracranial location. The reported data on intraspinal CCMs are extremely rare. To date, only 87 cases of spinal CCMs have been reported since 1996, and only 19 of them had no dural attachment. Detailed information is documented in (Table 1). However, it is noteworthy that the proportion of spinal to total meningiomas is higher for CCMs (45%) than for other meningiomas (the highest being 20% of the reported cases) [10]. In 2019, Zhang et al. (4) found that CCMs mostly attack young patient with a mean age at resection of 24 years old. Amazingly, 36 (42.9%) patients suffered from spinal CCM at an age of under 18 years. As it is the case of other meningiomas, CCMs have a slight female predominance with a female to male ratio at 1.7:1 (53 vs 31). In our review, 37 (42%) patients were under 18 years old and the mean age at resection was 24.6 years. 56 (63.6%) patients were female, 32 (36.4%) patients were male and the female-to-male ratio was 1.75:1. As for the most affected spinal region, the ordinary meningiomas were located in the thoracic region [11-13] whereas (66.7%) of CCMs were located in the lumbar region [4]. The imaging features of CCMs is very similar to those of ordinary meningiomas [15,16]. The MRI of CCMs often reveals an intradural-extramedullary, well-demarcated, homogeneously enhanced mass. It is isointense on both T1-and T2-weighted images, and demonstrates fairly homogeneous enhancement after the injection of gadolinium [8, 17]. Some cases showed foraminal extension and lacking of dural at-tachment, just like schwannomas [18] and other cases showed even an intramedullary CCM [10]. Because of radiologic and gross morphologic similarities between the spinal meningiomas and other intradural extramedullary spinal tumors such as schwannomas, neurofibromas, and ependymomas, an accurate histological diagnosis is mandatory. Just like any other intra-spinal lesion, the bone destruction by a CCM is rare, it was reported only two times until 2018. Both cases were reported by Jian Yang et al. [19]and they showed serious bone destruction at admission. The rarity of bone destruction may be explained with the fact that neurological symptoms usually appear before bone involvement due to the limited space of spine canal, especially in the lumbosacral region. Histologically, CCM contains layers of clear, glycogen-rich (Periodic acid-Schiff positive, diastase-labile), polygonal cells forming only a few vague whorls [6]. Its abundant glycogen is the reason why it is called a clear cell meningioma [14]. These cells are almost Immunoreactive (IR) to Epithelial Membrane Antigen (EMA) and vimentin, while not IR to GFAP, S-100, CK and SMA, with a ki67 index varying from 0 to 40%. The over expression of EGFR, PDGF-receptor and VEGF in CCMs promotes meningioma cell proliferation, a key process in meningioma angiogenesis, the formation of peritumoral edema, and the tumor aggressiveness [15,16]. The potential aggressiveness and recurrence are the main reason of the very challenging management of this tumor [20-45]. For now, for spinal CCMs, total resection should be considered as the optimal treatment. In our review of literature, 79 (89.77%) cases received Gross Total Resection (GTR) as the optimal treatment. However, 20 cases of spinal CCMs (22.72%) still experienced one or more recurrences until the last follow up. The use of postoperative radiotherapy has been increasingly affirmed and highlighted in the treatment of intracranial CCMs [3,21]. However, because of its rarity, the efficacy of postoperative radiotherapy for spinal CCM cannot be irrefutably concluded, therefore it requires further investigation. Tao et al. [21] reported that radiotherapy should not be performed immediately after the first operation of spinal CCMs, because the recurrence rate is lower than the one of intracranial CCM. This may be explained by the higher rate of GTR in spinal CCMs. According to the reported data, it is evident that spinal CCMs are more aggressive, with a much greater progression rate (38.0%) after initial treatment compared with ordinary spinal meningiomas (~3%) [13]. Jiu Hong Li et al. showed in their study that younger patients had better evolution after treatment (54.2%) compared with older ones (23.1%) [22, 46-53].

Conclusion

Spinal CCM, a rare subtype of meningioma, is an aggressive neoplasm with a high rate of local recurrence. It has a predilection for younger patients, and it is mostly located in the lumbar spine. Commonly, the spinal CCM is attached to the dura mater and it is exceptional to see a non-dura linked CCM. Gross total resection remains recommended in treating spinal CCM whenever it is possible. However, radiotherapy could be considered for patients who have undergone subtotal resection or for younger patients, regardless of the extent of resection [54-56].

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