"Never doubt that a small group of thoughtful, committed citizens can change the world. Indeed, it is the only thing that ever has."

Margaret Mead
Case report
peer-reviewed

Dural Arteriovenous Fistula Associated With a Vestibular Tumor: An Unusual Case and Review of the Literature



Abstract

Intracranial dural arteriovenous fistulae (DAVF) are rare vascular malformations. They are generally considered to be acquired lesions, often attributed to dural sinus thrombosis and intracranial venous hypertension. The authors encountered a case of DAVF associated with an octreotide-positive vestibular schwannoma. A 46-year-old female had symptoms of right ear congestion accompanied by pulsatile tinnitus and mild hearing loss. Magnetic resonance imaging (MRI) identified a lobulated mass centered at the cerebellopontine angle. Preoperatively, on cerebral angiography, there was an incidental discovery of a DAVF in the right posterior fossa. The decision was made to proceed with resection of the tumor in a staged fashion. Her latest follow-up MRI showed no evidence of recurrent tumor. This is the second reported case of DAVF associated with an intracranial schwannoma. Findings are discussed along with a thorough review of the literature. This case, combined with the data from the literature review, led us to believe that tumor-related angiogenesis might contribute to DAVF formation.

Introduction

Intracranial dural arteriovenous fistulae (DAVF) are abnormal direct shunts between dural arteries and dural venous sinuses, meningeal veins or cortical veins. They are located within or near the wall of the dural sinus and account for 10%-15% of all intracranial arteriovenous malformations [1]. DAVF primarily occur in adult patients and are most commonly located in the transverse-sigmoid sinus region followed by the cavernous sinus; however, any intracranial venous sinus may be involved [2]. They are widely considered to be acquired lesions. Their acquired nature is further supported by their association with dural sinus thrombosis, head trauma, intracranial or spinal tumors [3-4], localized infection, previous neurosurgical procedures [1-2], and hypercoagulation states [1].

We encountered a rare case of a transverse-sigmoid sinus DAVF associated with the presence of a vestibular schwannoma, which occluded the right transverse and sigmoid sinuses. The pre-operative diagnosis of this lesion was confounded by the fact that the tumor was positive on octreotide nuclear scintigraphy.

Case Presentation

A 46-year-old female had symptoms of right ear congestion accompanied by pulsatile tinnitus and mild hearing loss. Neurological examination revealed weakness of cranial nerves X and XII. Magnetic resonance imaging (MRI) identified a 4.2 x 4.7 x 4.1 cm lobulated mass centered at the cerebellopontine angle that was hypointense on T1-weighted, heterogeneous on T2-weighted, and avidly enhancing on post-contrast images (Figure 1A). No significant component was noted within the internal auditory canal. An Octreoscan was performed, which showed intense increased tracer uptake in the mass centered in the region of the right jugular foramen, thereby supporting the suspected diagnosis of paraganglioma (Figure 1B).

Digital subtraction angiography (DSA) demonstrated a surprising lack of vascularity associated with the tumor. However, there was an incidental discovery of a Cognard IIa+b dural arteriovenous fistula in the right posterior fossa associated with an occluded right sigmoid sinus (Figure 2A-2D).

Due to the extensive volume of tumor, the patient underwent a staged surgical resection. The first surgery included a modified radical right neck dissection, right superficial parotidectomy with facial nerve dissection, as well as the initial approach to the tumor and partial extradural resection. One week later, the patient underwent the second stage surgery where the tumor was radically resected using a right transcochlear approach. The right sigmoid sinus was ligated. The facial nerve was mobilized and transposed. Postoperative MRI confirmed complete resection of the tumor (Figure 1C). Histological analysis of samples from both surgeries confirmed the tumor not to be a paraganglioma, but instead a schwannoma (Figure 3).

She had a long recovery from the surgery due to the lower cranial nerve palsies but has shown gradual improvement. Her latest follow-up MRI (48 months from surgery) (Figure 1D) showed no evidence of recurrent tumor; however, there was some residual posterior fossa DAVF. At that time, she also reported bothersome pulsatile tinnitus that she perceives on the right despite a complete hearing loss on the right side. DSA revealed new large feeders from the right middle meningeal artery (MMA) and occipital arteries into the residual DAVF (figure 2E), which were not present on initial presentation (Figure 2D). The DAVF was embolized utilizing Onyx in the right MMA. Final right common carotid artery (CCA) angiogram demonstrated resolution in arteriovenous shunting (Figure 2F). Vertebral artery injection after embolization demonstrated minimal residual shunting via the right anterior inferior cerebellar artery (AICA) (Figure 2G). A further intervention was declined by the patient and angiographic follow-up in one year has been scheduled.

Discussion

In our patient, we encountered a transverse-sigmoid sinus DAVF associated with a schwannoma of the right skull base. Although angiography was not available prior to diagnosis of the large skull base mass, one could hypothesize that the schwannoma led to the venous sinus obstruction, and subsequently the formation of the DAVF. DAVF formation attributed to a tumor has been described, but it is rare. A detailed PubMed query was performed, which identified 26 previously reported cases (Table 1[3-17]. Analysis of the previously reported cases exhibited a slight female predominance (61.5%) and a mean age of 60.26 years (range 25-78). This is only the second case associated with an intracranial schwannoma. In the vast majority of cases (78%), the tumor was a meningioma. Although there are exceptions, most cases of DAVF associated with tumor are also associated with sinus obstruction, underlining the importance of venous outflow obstruction in DAVF pathophysiology. Specifically, the most common location of occlusion was the sigmoid sinus, which was occluded in 38.5% of cases. Interestingly, 23% of cases were not related to sinus occlusion. The most common location for a DAVF associated with a tumor was the transverse-sigmoid sinus (48% of cases). Some investigators hypothesize that meningiomas are the most likely tumor type to be associated with DAVF due to their highly vascular nature, which in turn accentuates venous hypertension, especially when the sinus is occluded [14].

Author (year) Age/Sex Tumor/Location DAVF Sinus Occlusion Treatment of DAVF Follow-up
Sawamura et al. (1991) [7] 68/M Meningioma/R occipital (parafalcine) Tentorium Unclear Unclear (tumor surgical resection) None reported
Yokota et al. (1993) [8] 65/F Meningioma/R sigmoid sinus groove R transverse sinus R sigmoid sinus Pre-op TAE and surgical packing 36 mo (disappeared)
Yamakami et al. (1998) [9] 69/M Schwannoma/R jugular foramen R sigmoid sinus R sigmoid sinus Pre-op TAE and surgical resection None (disappeared post-op)
    Arnautovic et al. (1998) [3] 63/F Meningioma/L tentorium L transverse and sigmoid sinuses L transverse sinus Pre-op TAE and surgical resection 40 mo (disappeared)
57/F Paraganglioma/R jugular foramen R transverse and sigmoid sinuses R sigmoid sinus Pre-op TAE and surgical resection 28 mo (disappeared)
Chung et al. (1999) [10] 62/F Meningioma/R tentorium R transverse sinus R transverse and sigmoid sinuses None (tumor subtotal surgical resection) None (disappeared post-op)
        Vilela et al. (2001) [6] 51/M Meningioma/ Parasagittal 2 DAVFs: R superior petrosal sinus AND R parietal convexity Superior sagittal sinus Surgical disconnection of DAVFs w/o tumor resection 60 mo (no DAVF recurrence)
56/ Unspecified Meningioma/R skull base R transverse sinus R sigmoid sinus None (Pre-op tumor TAE and tumor surgical resection) 24 mo (clinically stable)
Horinaka et al. (2003) [11] 69/F Meningioma/R transverse sinus R transverse sinus R transverse sinus and part of sigmoid sinus Transverse sinus resection (part of tumor resection surgery) 9 mo (disappeared)
Ahn et al. (2003) [5] 45/F Meningioma/L parietal convexity L transverse and sigmoid sinuses L sigmoid sinus (partial, w/o venous congestion) None (Pre-op tumor TAE and tumor surgical resection) 12 mo (disappeared)
Inoue et al. (2007) [12] 76/M Meningioma/L sphenoidal ridge R cavernous sinus None TVE (followed by tumor surgical resection) 12 mo (disappeared)
Zhou et al. (2007) [13] 42/M Meningioma/L anterior clinoid (tumor unnoticed pre-op) L spheno-basilar sinus None Pre-op TAE and surgical disconnection 24 mo (disappeared)
Toledo et al. (2010) [14] 60/M Meningioma/L parietal lobe (parafalcine) Superior sagittal sinus Superior sagittal sinus Pre-op TAE and surgical occlusion None (disappeared post-op)
Kalani et al. (2011) [15] 25/F Hemangiopericytoma/L parieto-occipital (parafalcine) Multiple DAVFs: Unclear locations (one torcular DAVF) Superior sagittal sinus Pre-op TAE and surgical disconnection (during tumor surgical resection) 7 mo (patient asymptomatic but has residual DAVF)
Enatsu et al. (2012) [16] 76/F Meningioma/R tentorium R transverse sinus R transverse and sigmoid sinuses Pre-op TAE and intra-op feeder coagulation and occluded sinus resection (during tumor surgical resection) 1 mo (disappeared)
Vellimana et al. (2014) [17] 55/M Meningioma/L sphenoid wing/orbit 2 DAVFs: both L convexity None None (tumor subtotal surgical resection) None reported
73/F Meningioma (radiologic diagnosis) /L occipital/tentorium L cavernous sinus L cavernous sinus and L transverse sinus (partial) TAE and TVE followed by GK (no tumor resection) None reported
69/M Meningioma/L convexity (fronto-temporal) R transverse sinus Partial of multiple unspecified sinuses None (TAE of tumor followed by tumor surgical resection) None reported
41/F Meningioma/R lateral ventricle-trigone L convexity None TAE (followed by tumor surgical resection) None reported
54/F Meningioma (radiologic diagnosis)/R petrous apex L transverse and sigmoid sinuses R cavernous sinus TAE (followed by GK for tumor) None reported
57/M Meningioma (radiologic diagnosis)/L parasellar L convexity L cavernous sinus None (fractionated radiotherapy for tumor) None reported
78/F Meningioma (radiologic diagnosis)/L convexity (frontal) L cavernous sinus L cavernous sinus TAE (no tumor resection) None reported
67/F Phosphaturic mesenchymal tumor/L anterior cranial fossa Lateral tentorial None Surgical resection (following failed TAE) None reported
72/M Meningioma/ Planum sphenoidale Ethmoidal sinus None Surgery (during tumor resection) None reported
61/F Meningioma/ Olfactory groove R transverse and sigmoid sinuses Superior sagittal sinus GK and TAE None reported
Hatanaka et al. (2015) [4] 70/F Schwannoma/ Spinal (C2 level) R transverse and sigmoid sinuses R sigmoid sinus TAE and TVE (followed by tumor surgical resection). GK after DAVF recurrence 6 mo post-intervention 33 mo (disappeared)
DAVF: dural arteriovenous fistula; TAE: transarterial embolization; TVE: transvenous embolization; GK: gamma knife radiosurgery

Another unique characteristic in our case was the positive 111In-octreotide scintigraphy (Octreoscan), which is used to evaluate the presence of somatostatin receptors in intracranial tumors in vivo. Expression of somatostatin receptors has been linked with increased angiogenic activity in meningiomas and other neoplasias [18]. This is only the second reported case of an intracranial schwannoma with positive octreotide scintigraphy [19]. Past studies have shown that somatostatin receptors are expressed in most meningiomas, paragangliomas and hemangiopericytomas, but not in schwannomas, with some investigators using octreotide scintigraphy for the differential diagnosis of meningioma versus schwannoma [19-20]. Our case further supports the conclusion by Dupuch et al. that no investigation is able to establish a definitive preoperative differential diagnosis between schwannoma and meningioma [19]. Taking into consideration the case we encountered, we hypothesize that aberrant angiogenesis through somatostatin receptor pathways may play a role in the pathogenesis of DAVF associated with intracranial tumors. Even though there is currently no evidence showing a direct connection between somatostatin pathways and DAVF formation, studies have shown that the presence of somatostatin receptors correlates with aberrant angiogenesis in various tumor settings [18]. Our hypothesis aims at opening new avenues of investigation that will help in better understanding DAVF pathophysiology.

Conclusions

In summary, we present an unusual case of DAVF associated with an octreotide positive vestibular schwannoma. This case demonstrates that lesions in the cerebellopontine angle could affect local vasculature, possibly by both causing local venous hypertension and somatostatin receptor-related angiogenesis, to the extent it may lead to a DAVF formation.


References

  1. Gandhi D, Chen J, Pearl M, Huang J, Gemmete JJ, Kathuria S: Intracranial dural arteriovenous fistulas: classification, imaging findings, and treatment. AJNR Am J Neuroradiol. 2012, 33:1007-1013. 10.3174/ajnr.A2798
  2. Cognard C, Gobin YP, Pierot L, et al.: Cerebral dural arteriovenous fistulas: clinical and angiographic correlation with a revised classification of venous drainage. Radiology. 1995, 194:671-680. 10.1148/radiology.194.3.7862961
  3. Arnautovic KI, Al-Mefty O, Angtuaco E, Phares LJ: Dural arteriovenous malformations of the transverse/sigmoid sinus acquired from dominant sinus occlusion by a tumor: report of two cases. Neurosurgery. 1998, 42:383-388. 10.1097/00006123-199802000-00112
  4. Hatanaka R, Takai K, Iijima A, Taniguchi M: Intracranial dural arteriovenous fistula associated with a spinal tumor: a case report. Acta Neurochir (Wien). 2015, 157:1825-1827. 10.1007/s00701-015-2533-2
  5. Ahn JY, Lee BH, Cho YJ, Joo JY, Lee KS: Dural arteriovenous fistula associated with meningioma: spontaneous disappearance after tumor removal. Neurol Med Chir (Tokyo). 2003, 43:308-311. 10.2176/nmc.43.308
  6. Vilela P, Willinsky R, terBrugge K: Dural arteriovenous fistula associated with neoplastic dural sinus thrombosis: two cases. Neuroradiology. 2001, 43:816-820. 10.1007/s002340100570
  7. Sawamura Y, Janzer RC, Fankhauser H, de Tribolet N: Arteriovenous malformation in meningothelial meningioma: case report. Neurosurgery. 1991, 29:109-112. 10.1227/00006123-199107000-00019
  8. Yokota M, Tani E, Maeda Y, Yamaura I: Meningioma in sigmoid sinus groove associated with dural arteriovenous malformation: case report. Neurosurgery. 1993, 33:316-319. 10.1227/00006123-199308000-00022
  9. Yamakami I, Ono J, Yamaura A: Sigmoid sinus dural arteriovenous malformation resulting from jugular foramen schwannoma--case report. Neurol Med Chir (Tokyo). 1998, 38:43-46. 10.2176/nmc.38.43
  10. Chung YG, Lee KC, Lee HK, Lee NJ: Tentorial meningioma encroaching the transverse sinuses and sigmoid sinus junction area associated with dural arteriovenous fistulous malformation: a case report. J Korean Med Sci. 1999, 14:465-468. 10.3346/jkms.1999.14.4.465
  11. Horinaka N, Nonaka Y, Nakayama T, Mori K, Wada R, Maeda M: Dural arteriovenous fistula of the transverse sinus with concomitant ipsilateral meningioma. Acta Neurochir (Wien). 2003, 145:501-504. 10.1007/s00701-003-0030-5
  12. Inoue T, Nishimura S, Hayashi N, Numagami Y, Tomita T, Nishijima M: Cavernous sinus dural arteriovenous fistula associated with the development of sphenoidal ridge meningioma--case report. Neurol Med Chir (Tokyo). 2007, 47:317-321. 10.2176/nmc.47.317
  13. Zhou LF, Chen L, Song DL, Gu YX, Leng B: Dural arteriovenous fistula of the sphenobasilar sinus with concomitant meningioma: case report and review of the literature. Neurosurg Rev. 2007, 30:269-274. 10.1007/s10143-007-0071-y
  14. Toledo MM, Wilson TJ, Dashti S, McDougall CG, Spetzler RF: Dural arteriovenous fistula associated with superior sagittal sinus occlusion secondary to invasion by a parafalcine meningioma: case report. Neurosurgery. 2010, 67:205-207. 10.1227/01.NEU.0000370089.94032.4F
  15. Kalani MY, Martirosyan NL, Eschbacher JM, Nakaji P, Albuquerque FC, Spetzler RF: Large hemangiopericytoma associated with arteriovenous malformations and dural arteriovenous fistulae. World Neurosurg. 2011, 76:597-510. 10.1016/j.wneu.2011.05.023
  16. Enatsu R, Asahi M, Matsumoto M, Hirai O: Meningioma-related dural arteriovenous fistula fed via a vascular tumor bed: a case report and literature review. Clin Neurol Neurosurg. 2012, 114:1010-1013. 10.1016/j.clineuro.2012.01.017
  17. Vellimana AK, Daniels DJ, Shah MN, Zipfel GJ, Lanzino G: Dural arteriovenous fistulas associated with benign meningeal tumors. Acta Neurochir (Wien). 2014, 156:535-544. 10.1007/s00701-013-1946-z
  18. Barresi V, Alafaci C, Salpietro F, Tuccari G: Sstr2A immunohistochemical expression in human meningiomas: is there a correlation with the histological grade, proliferation or microvessel density?. Oncol Rep. 2008, 20:485-492. 10.3892/or_00000032
  19. Dupuch V, Pastourel R, Gilain L, Mom T: A case of facial nerve schwannoma with positive octreotide scintigraphy [Article in English, French]. Eur Ann Otorhinolaryngol Head Neck Dis. 2015, 132:113-114. 10.1016/j.anorl.2014.06.004
  20. Schmidt M, Scheidhauer K, Luyken C, et al.: Somatostatin receptor imaging in intracranial tumours. Eur J Nucl Med. 1998, 25:675-686. 10.1007/s002590050269
Case report
peer-reviewed

Dural Arteriovenous Fistula Associated With a Vestibular Tumor: An Unusual Case and Review of the Literature


Author Information

Michael E. Kritikos Corresponding Author

Neurosurgery, Hospital of the University of Pennsylvania, Philadelphia, USA

Martin Oselkin

Neuroradiology, Hospital of the University of Pennsylvania, Philadelphia, USA

Radiology, Hospital of the University of Pennsylvania, Philadelphia, USA

Nikhil Sharma

Neurosurgery, Hospital of the University of Pennsylvania, Philadelphia, USA

Pallavi P. Gopal

Pathology, Hospital of the University of Pennsylvania, Philadelphia, USA

Douglas C. Bigelow

Otorhinolaryngology, Hospital of the University of Pennsylvania, Philadelphia, USA

Sean Grady

Neurosurgery, Hospital of the University of Pennsylvania, Philadelphia, USA

Robert W. Hurst

Radiology, Hospital of the University of Pennsylvania, Philadelphia, USA

Bryan A. Pukenas

Radiology, Hospital of the University of Pennsylvania, Philadelphia, USA

Omar Choudhri

Neurosurgery, Hospital of University of the Pennsylvania, Philadelphia, USA

David Kung

Neurosurgery, Hospital of the University of Pennsylvania, Philadelphia, USA


Ethics Statement and Conflict of Interest Disclosures

Human subjects: Consent was obtained by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.


Case report
peer-reviewed

Dural Arteriovenous Fistula Associated With a Vestibular Tumor: An Unusual Case and Review of the Literature


Figures etc.

SIQ
-
RATED BY 1 READER
CONTRIBUTE RATING

Scholary Impact Quotient™ (SIQ™) is our unique post-publication peer review rating process. Learn more here.