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|LETTER TO EDITOR
|Year : 2016 | Volume
| Issue : 7 | Page : 121-126
Glioblastoma multiforme in association with a dural arteriovenous fistula
Xunhui Yuan1, Mingtong Gao2, Hongyan Zhao3, Jianyi Niu4, Yun'an Bai1, Liemei Guo5
1 Department of Neurosurgery, Yidu Central Hospital of Weifang, Qingzhou, China
2 Department of Emergency, Affiliated Hospital of Weifang Medical University, Weifang, Shandong Province, China
3 Department of Pharmacy, Yidu Central Hospital of Weifang, Qingzhou, China
4 Department of Neurology, Yidu Central Hospital of Weifang, Qingzhou, China
5 Department of Neurosurgery, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
|Date of Web Publication||3-Mar-2016|
Department of Neurosurgery, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Yuan X, Gao M, Zhao H, Niu J, Bai Y, Guo L. Glioblastoma multiforme in association with a dural arteriovenous fistula. Neurol India 2016;64, Suppl S1:121-6
The coexistence of a cerebral glioma and a vascular malformation is rarely identified. ,,,,, Most frequently, lesions consisting of mixed elements of glial and vascular origin are identified as angiogliomas, which are predominantly highly vascular, low-grade gliomas.  However, the coexistence of a glioblastoma multiforme (GBM) and an arteriovenous malformation (AVM) is much less common, and only five cases have been reported to date. ,,, Here, we report a rare case of a GBM with a tentorial dural arteriovenous fistula (DAVF), which, to the best of our knowledge, is the first reported case of its kind.
A 23-year-old woman was admitted to a local hospital with severe headache for 5 days. Physical examination on admission did not reveal any neurological deficit. Emergent cranial computed tomography (CT) scans showed a right temporal mass typical of a hematoma [Figure 1]a, and cerebral angiography revealed a tentorial DAVF, which was fed by one branch of the meningohypophyseal trunk, and drained into the vein of Galen [Figure 1]b-e. The surgeon regarded the mass to be a hematoma originating from the rupture of the DAVF. Thus, microsurgical disconnection of the shunt and removal of the hematoma were considered to be the treatment of choice. The DAVF was then electrocoagulated and disconnected, and the mass was removed subtotally [Figure 1]f without it being subjected to further histopathological examination. The postoperative course was uneventful and the patient recovered well without any neurological deficit. However, 8 months later, the patient was admitted to our department complaining of severe headache and dizziness for 2 months. Cranial magnetic resonance imaging (MRI) showed a giant cystic as well as solid tumor infiltrating the temporal lobe and the basal ganglion, with a part of the tumor being located below the tentorium [Figure 2]a and b. Brain MR spectroscopy indicated the presence of a high-grade glioma. Angiography revealed the recurrence of the tentorial DAVF, which was now being fed by branches of the meningohypophyseal trunk and the middle meningeal artery, and was draining into the vein of Galen [Figure 2]c-e. In comparison to the size observed in the initial angiography, the DAVF had enlarged dramatically. In addition, intratumoral arteriovenous shunting supplied by the superior cerebellar and anterior inferior cerebellar arteries was observed [Figure 2]f. After careful consultation, endovascular embolization of the DAVF and intratumoral arteriovenous shunting, followed by tumor removal, were considered to be the optimal treatment options available. The tentorial DAVF was then embolized completely with onyx, although the occlusion of the intratumoral arteriovenous shunt was not successful because the introduction a microcatheter was hindered by the small diameter of the fine arteries [Figure 2]g and h. Two days after the embolization, a craniotomy with tumor removal was performed [Figure 2]i. Histopathological examination revealed that the tumor was composed of neoplastic astrocytic cells with a high mitotic activity, significant necrosis, and obvious microvascular proliferation, which indicated the diagnosis of a GBM [Figure 3]a-c. On immunohistochemical staining, the tumor cells were positive for glial fibrillary acidic protein (GFAP) and S-100, and diffusely positive for vascular endothelial growth factor (VEGF) [Figure 3]d-f. The patient then received external radiotherapy (up to 60 Gy) with concurrent daily temozolomide treatment (75 mg/kg), followed by standard temozolomide chemotherapy (150 mg/kg) for the subsequent 6 months. The patient tolerated the treatment well; however, she died 14 months later.
|Figure 1: Pre-operative and post-operative images associated with the first operation. Emergent cranial computed tomography (CT) scans at admission showed a right temporal mass with some calcification around the margin, typical of a hematoma (a). Cerebral angiography revealed a tentorial dural arteriovenous fistula (DAVF), which was fed by one of the branches of the meningohypophyseal trunk (b) and drained into the vein of Galen (c) The DAVF was not supplied by the external carotid or vertebral arteries (d and e). Post-operative CT scans showed sub-total removal of the mass (f)|
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|Figure 2: Pre - operative and post - operative images associated with the second operation. Cranial magnetic resonance imaging (MRI) showed a giant cystic as well as solid mass with contrast enhancement infiltrating the temporal lobe and the basal ganglion; part of the tumor was located below the tentorium (a and b). Angiography revealed recurrence of the tentorial DAVF, which was fed by branches of the meningohypophyseal trunk (c) and middle meningeal artery (d) and drained to the vein of Galen (e). An intratumoral arteriovenous shunt supplied by the the superior and anterior inferior cerebellar arteries was identified (f). The tentorial DAVF was embolized completely with onyx (g and h). Post - operative cranial MRI showed subtotal removal of the tumor (i)|
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|Figure 3: Histopathological and immunohistochemical evaluation of the tumor. Histopathological examination showed that the tumor was composed of neoplastic astrocytic cells with significant necrosis (a). Obvious microvascular proliferation (b) and a high mitotic activity (c) indicated the diagnosis of a GBM. Immunohistochemical staining of the tumor cells showed a highly positive and diffuse expression of GFAP (d) S-100 (e) and VEGF (f)|
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The coexistence of a glioma and a vascular malformation remains a rare event documented in only a few case reports. ,,,,, Generally, a vascular malformation can be associated with different glial components that include a benign or an anaplastic astrocytoma, an oligodendroglioma, a mixed oligo-astrocytoma or a GBM. The majority of vascular malformations in coexistence with a glioma consist of an AVM and the simultaneous presence of a cavernous angioma is less common.  The coexistence of a GBM and an AVM is extremely rare. ,,, A summary of the clinical characteristics of the six reported cases (including the present case) is presented in [Table 1]. To the best of our knowledge, this is the first reported case of a GBM in association with a DAVF.
|Table 1: A summary of the clinical characteristics in patients with the coexistence of GBM and AVM |
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Based on the available literature, multiple possible etiologies regarding the association of a glioma and an AVM have been proposed; their simultaneous presence could be a fortuitous separate association, a combined distinct entity, or a sequential development. ,
First, some case reports have documented the coexistence of separately located, but not combined or intermixed lesions that might represent a coincidental association. , Furthermore, an AVM and a glioma can also occur in a combined manner and present as one lesion. ,, The pre-operative images in some cases revealed only one lesion with the another lesion being detected either during surgery or on the histopathological examination. Cemil et al., reported a patient with an AVM who developed an intracranial hemorrhage pre-operatively; however, the post-operative histopathological analysis revealed a GBM.  Gmeiner et al., diagnosed a patient as an GBM from the initial MR imaging, but the AVM was detected intraoperatively and was confirmed by the initial histopathological analysis.  Surprisingly, in a reinvestigation of the histopathological sections 2 months later, the lesion was identified as a GBM.
The association of an AVM and GBM has also been regarded as a sequential development, and it has been suggested that glioma might be a secondary or reactive neoplastic glial change to a previously existing AVM. Others have also suggested that an AVM could be induced by the internal environment of the glioma. , Harri et al., reported one case that showed the development of an AVM in the setting of a high grade glioma.  Similarly, McKinney et al., provided evidence of the de novo formation of an AVM within an anaplastic oligodendroglioma in a patient who had an initially negative brain MR angiography.  These reports indicate that an AVM may be induced by a glioma. In contrast, the subsequent occurrence of a glioma after the diagnosis of an AVM has also been documented.  Goodkin et al., diagnosed a patient having an AVM that was detected utilizing the cerebral angiography initially. Although the AVM regressed and finally disappeared, a hypovascular mass simultaneously developed at the same site. This was subsequently diagnosed on histopathological examination as being an anaplastic astrocytoma.  Thus, it was concluded that the AVM might have stimulated the development of the glioma.
Considering the postulation of sequential development of the two entitites, investigations for the identification of the interacting mechanisms between the AVM and glioma are warranted. An AVM possesses inherent tumorigenic potential. First, AVM and other vascular malformations are known to induce a gliotic reaction in the surrounding neural parenchyma. In response to an insult, such as hemorrhage, glial cells can de-differentiate and then proliferate. After exposure to chronic injury in the form of intermittent hemorrhage over several decades, the proliferating glial cells may sustain a mutation into glioma cells. , Second, some growth factors and cytokines induced by an AVM can promote neoplasia. Vascular endothelial growth factor (VEGF), which is highly expressed in AVMs, has been shown to promote vascularity within a glioma and has been shown to correlate with tumor progression.  A glioma, especially a GBM, can also initiate and promote the development of an AVM, although most AVMs are traditionally considered to be congenital lesions. In several recent reports, AVMs have been demonstrated in GBMs by angiographic visualization. , It can be speculated that proangiogenic growth factors (e.g. VEGF), which are highly expressed in GBMs, play an important role in the pathogenesis and development of an AVM within the tumor. In the present case, as the tentorial DAVF enlarged and the intratumoral arteriovenous shunting developed 8 months later, it is possible that the presence of the GBM might have promoted the development of these conditions.
Importantly, both AVMs and GBMs express a distinct pattern of proangiogenic growth factors, such as VEGF.  In the present case, VEGF was highly and diffusely expressed in the GBM, which might have created the hyperangiogenic environment that fostered growth of the tentorial DAVF and an intratumoral arteriovenous shunting. The growth and enlargement of the DAVF and the intratumoral arteriovenous shunting require a significantly increased blood supply, which would clearly promote the development of a GBM. Thus, VEGF may promote the development of both a GBM and an AVM, and represent the link between these two pathologies.
The treatment of a GBM in association with an AVM remains complicated, and sometimes challenging. If the concurrent lesions are separate, both should be managed individually. The treatment of GBM should be the priority because of its malignant potential.  When the lesions occur in a combined manner, we suggest an initial endovascular embolization of the AVM followed by cytoreductive surgery, chemotherapy and radiotherapy. In addition, bevacizumab has been reported to be effective in reducing the tumor-related enhancement and edema as well as AVM-associated edema.  In addition, as shown in [Table 1], the prognosis of a GBM in association with an AVM is comparable with that of a primary GBM (with a median survival of approximately 14 months).
The limitations of this study should also be mentioned. One of the major limitations involves the diagnosis and treatment during the first admission in a local hospital. First, a tumor rather than a hematoma should have been suspected in the first CT scan because of the presence of calcification at the margin of the mass with only a mild midline shift. Second, the lesion was subtotally removed without conducting further histopathological examination, which delayed the diagnosis of a glioma. The incomplete removal of the tumor demonstrates that the treatment in our department was suboptimal. Despite the problems associated with the initial diagnosis and treatment, this rare and complex case indicates the association of a GBM and an AVM, with VEGF as a possible link between these two distinct pathologies.
In conclusion, the coexistence of a GBM and AVM is extremely rare. Here, we present the first reported case of a GBM with a tentorial DAVF, and found that VEGF was highly and diffusely expressed in the GBM tissue in this case. The exact mechanism of the interaction between the GBM and the vascular malformation remains to be fully elucidated, and the role of VEGF as the potential link between these two pathologies requires further investigation.
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Conflicts of interest
There are no conflicts of interest.
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