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• W2097408033 abstract "In this review of cerebellopontine angle tumors, we focus on the most common neoplasm in this location—vestibular schwannoma. We examine both the usual and unusual imaging manifestations of this tumor, which often presents with sensorineural hearing loss, tinnitus, dizziness, and rarely vertigo. We also review key imaging findings that can have significant implications in presurgical planning. Thereafter, we undertake a brief review of the next most common tumors that occur in this location, including meningioma, facial nerve schwannoma, metastasis, and congenital epidermoid cyst. We emphasize distinctive imaging features of these entities, which can help increase our diagnostic accuracy. In this review of cerebellopontine angle tumors, we focus on the most common neoplasm in this location—vestibular schwannoma. We examine both the usual and unusual imaging manifestations of this tumor, which often presents with sensorineural hearing loss, tinnitus, dizziness, and rarely vertigo. We also review key imaging findings that can have significant implications in presurgical planning. Thereafter, we undertake a brief review of the next most common tumors that occur in this location, including meningioma, facial nerve schwannoma, metastasis, and congenital epidermoid cyst. We emphasize distinctive imaging features of these entities, which can help increase our diagnostic accuracy. The cerebellopontine angle (CPA) cistern is a subarachnoid space that contains cranial nerves and vessels surrounded by cerebrospinal fluid (CSF) and lined by meninges. The CPA is bordered by the pons medially, the anterior aspect of the cerebellum posteriorly, and the petrous temporal bone anteriorly. Within the center of the CPA is the internal auditory canal (IAC), which serves as the passageway for cranial nerves VII and VIII from the pons to the facial nerve canal and the membranous labyrinth, respectively. CPA masses represent 6%-10% of all intracranial tumors.1Moffat D.A. Ballagh R.H. Rare tumours of the cerebellopontine angle.Clin Oncol. 1995; 7: 28-41Abstract Full Text PDF PubMed Scopus (76) Google Scholar As they are extra-axial in location, they widen the ipsilateral subarachnoid cisterns. They may either encase or displace neurovascular structures. As CPA masses are clinically nonspecific and presenting symptoms are not related to the nature of the lesion itself but rather to the nerves or cerebral structures involved, imaging is key in the preoperative diagnosis of a CPA tumor. Although computed tomography (CT) provides important information regarding the temporal bone and the osseous labyrinth, magnetic resonance imaging (MRI) is the imaging modality of choice for evaluating CPA pathology owing to its excellent soft tissue contrast.2Curtin H.D. Hirsch Jr, W.L. Imaging of acoustic neuromas.Otolaryngol Clin North Am. 1992; 25: 553-607PubMed Google Scholar Specifically, thin-section T1 contrast-enhanced images and 3-dimensional (3D) heavily T2-weighted images are crucial sequences when evaluating the CPA and IAC. Vestibular schwannomas (VS) are by far the most common CPA tumor and account for approximately 85% of all CPA masses.3St Martin M.B. Hirsch B.E. Imaging of hearing loss.Otolaryngol Clin North Am. 2008; 41: 157-178Abstract Full Text Full Text PDF PubMed Scopus (25) Google Scholar Most VS develop from the Schwann sheath of the inferior vestibular nerve (IVN) in the IAC near the Obersteiner-Redlich zone, which marks the transition from glial cells (central nervous system) to Schwann cells (peripheral nervous system).3St Martin M.B. Hirsch B.E. Imaging of hearing loss.Otolaryngol Clin North Am. 2008; 41: 157-178Abstract Full Text Full Text PDF PubMed Scopus (25) Google Scholar Although there is some variation in the literature as to which percentage of VS arrive from the IVN vs the superior vestibular nerve, 1 large series found that 85% of VS in their study arose from the IVN, whereas only 9% arose from the superior vestibular nerve.4Komatsuzaki A. Tsunoda A. Nerve origin of the acoustic neuroma.J Laryngol Otol. 2001; 115: 376-379Crossref PubMed Scopus (59) Google Scholar Growing slowly within the IAC with a mean growth rate of 1-2 mm/y,5Nikolopoulos T.P. Fortnum H. O'Donoghue G. et al.Acoustic neuroma growth: A systematic review of the evidence.Otol Neurotol. 2010; 31: 478-485Crossref PubMed Scopus (99) Google Scholar these tumors may either remain purely intracanalicular (Figure 1) or may widen the porus acusticus giving rise to a round or oval component in the CPA which produces the familiar “ice-cream-on-cone” appearance (Figure 2). In addition to the classic appearance of an intracanalicular or IAC-CPA VS, there is also the less common intralabyrinthine schwannoma6Mafee M.F. MR imaging of intralabyrinthine schwannoma, labyrinthitis, and other labyrinthine pathology.Otolaryngol Clin North Am. 1995; 28: 407-430PubMed Google Scholar (Figure 3), as well as the dumbbell-shaped schwannoma, which may extend from the IAC into the cochlea (transmodiolar), into the vestibule (transmacular), or into both the cochlea and vestibule (combined transmodiolar or transmacular).7Salzman K.L. Davidson H.C. Harnsberger H.R. et al.Dumbbell schwannomas of the internal auditory canal.Am J Neuroradiol. 2001; 22: 1368-1376PubMed Google ScholarFigure 2Classic IAC-CPA vestibular schwannoma. (A) Axial T2 and axial and (B and C) coronal T1 postcontrast images of an IAC-CPA VS, demonstrating the classic “ice-cream-on-cone” appearance with significant widening of the porus acusticus. Note the mass effect on the brainstem and partial effacement of the fourth ventricle.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Figure 3Intralabyrinthine schwannoma. (A) Axial 3D heavily T2-weighted image demonstrates a small filling defect within the T2 hyperintense perilymph of the left cochlea. (B and C) Axial and coronal T1 postcontrast images demonstrate an intensely enhancing schwannoma within the apical and middle turns of the left cochlea.View Large Image Figure ViewerDownload Hi-res image Download (PPT) On MRI, VS are typically T1 isointense and T2 hyperintense, but appear as hypointense-filling defects within the hyperintense CSF and perilymph on 3D heavily T2-weighted images (Figure 1, Figure 3). On postcontrast images, VS typically demonstrate intense enhancement. However, this enhancement may be either homogeneous or heterogeneous. Furthermore, 5%-15% of VS may have a cystic appearance8Charabi S. Tos M. Thomsen J. et al.Cystic vestibular schwannoma—clinical and experimental studies.Acta Otolaryngol Suppl. 2000; 543: 11-13PubMed Google Scholar (Figure 4). Small VS are usually homogeneously enhancing and composed of highly cellular and organized Antoni type A regions histologically, whereas larger VS tend to be heterogeneously enhancing or cystic and composed of hypocellular, loosely organized Antoni B regions or may demonstrate a mixed type A or type B pattern.9Gomez-Brouchet A. Delisle M.B. Cognard C. et al.Vestibular schwannomas: Correlation between magnetic resonance imaging and histopathologic appearance.Otol Neurotol. 2001; 22: 79-86Crossref PubMed Scopus (58) Google Scholar In the discussion of VS, we must also briefly discuss neurofibromatosis type 2 (NF 2). NF 2 is an autosomal dominant heritable neoplasia syndrome, which results from a germline mutation of the NF2 tumor suppressor gene located on the long arm of chromosome 22.10Trofatter J.A. MacCollin M.M. Rutter J.L. et al.A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor.Cell. 1993; 72: 791-800Abstract Full Text PDF PubMed Scopus (1076) Google Scholar NF 2 has an incidence of 1 in 25,000 persons and a penetrance of almost 100% by 60 years of age.11Evans D.G. Huson S.M. Donnai D. et al.A genetic study of type 2 neurofibromatosis in the United Kingdom. I. Prevalence, mutation rate, fitness, and confirmation of maternal transmission effect on severity.J Med Genet. 1992; 29: 841-846Crossref PubMed Scopus (395) Google Scholar Bilateral VS are the hallmark of NF 2 (Figure 5A). When bilateral VS are encountered, a thorough search should be undertaken for other cranial nerve schwannomas (Figure 5B), and the entire neuraxis must be imaged to search for schwannomas, meningiomas, and ependymomas in the spine as well (Figure 5C). It is noteworthy that several studies have shown that there is no direct association between the size or growth rate of VS in patients with NF 2 and hearing loss.12Fisher L.M. Doherty J.K. Lev M.H. et al.Concordance of bilateral vestibular schwannoma growth and hearing changes in neurofibromatosis 2: Neurofibromatosis 2 natural history consortium.Otol Neurotol. 2009; 30: 835-841Crossref PubMed Scopus (52) Google Scholar More recent studies have shown that hearing loss in NF 2 is related to cochlear aperture obstruction and accumulation of intralabyrinthine protein, and furthermore that this elevated intralabyrinthine protein may be identified on MRI using high-resolution fluid-attenuated inversion recovery (FLAIR) sequence13Asthagiri A.R. Vasquez R.A. Butman J.A. et al.Mechanisms of hearing loss in neurofibromatosis type 2.PLoS One. 2012; 7: e46132Crossref PubMed Scopus (44) Google Scholar (Figure 5D). Once a VS has been diagnosed on imaging, there are several key imaging features that must be relayed to the surgeon. In addition to the size and location of the tumor that are of obvious importance, the distance between the lateral extent of the intracanalicular portion of the tumor and the fundus of the IAC should be measured as this affects hearing prognosis and may modify the surgical approach.14Kocaoglu M. Bulakbasi N. Ucoz T. et al.Comparison of contrast-enhanced T1-weighted and 3D constructive interference in steady state images for predicting outcome after hearing-preservation surgery for vestibular schwannoma.Neuroradiology. 2003; 45: 476-481Crossref PubMed Scopus (33) Google Scholar Furthermore, the intralabyrinthine signal intensity should be examined as this may also predict hearing prognosis as described earlier in the setting of NF 2. This may be examined on high-resolution fast spin-echo T2-weighted images and on high-resolution FLAIR images, with a poor hearing prognosis predicted by hypointensity of the labyrinth on T2 and mild hyperintensity of the labyrinth on FLAIR. Identifying the facial nerve and describing its position relative to the VS, best seen on 3D heavily T2-weighted images,15Sartoretti-Schefer S. Kollias S. Valavanis A. Spatial relationship between vestibular schwannoma and facial nerve on three-dimensional T2-weighted fast spin-echo MR images.Am J Neuroradiol. 2000; 21: 810-816PubMed Google Scholar are critical because of its implications for surgery. However, it is important to note that identification of the facial nerve is not always possible, especially when evaluating larger VS. Finally, anatomical variants, such as a high-riding jugular bulb and pneumatization of the temporal bone adjacent to the IAC, should also be mentioned as knowledge of these variants before surgery may help limit intraoperative and postoperative complications.16Silk P.S. Lane J.I. Driscoll C.L. Surgical approaches to vestibular schwannomas: What the radiologist needs to know.Radiographics. 2009; 29: 1955-1970Crossref PubMed Scopus (25) Google Scholar Once a VS is diagnosed, there are 3 main management options: observation, radiosurgery, and resection.17Kondziolka D. Mousavi S.H. Kano H. et al.The newly diagnosed vestibular schwannoma: Radiosurgery, resection, or observation?.Neurosurg Focus. 2012; 33: E8Crossref PubMed Scopus (93) Google ScholarAlthough the observation strategy is typically employed for elderly patients, individuals with significant medical comorbidities, or asymptomatic patients with small incidentally discovered VS,18Hoistad D.L. Melnik G. Mamikoglu B. et al.Update on conservative management of acoustic neuroma.Otol Neurotol. 2001; 22: 682-685Crossref PubMed Scopus (77) Google Scholar the choice between radiosurgery and microsurgery is much more controversial. However, a recent meta-analysis found that for small vestibular schwannomas (<2 cm), patients who underwent stereotactic radiation had significantly better long-term hearing preservation outcome rates than patients who underwent microsurgery and that long-term tumor outcome was not significantly different between the 2 groups.19Maniakas A. Saliba I. Microsurgery versus stereotactic radiation for small vestibular schwannomas: A meta-analysis of patients with more than 5 years' follow-up.Otol Neurotol. 2012; 33: 1611-1620Crossref PubMed Scopus (36) Google Scholar If surgical resection is chosen, there are 3 basic surgical approaches: the middle cranial fossa (MCF) approach, retrosigmoid approach (Figure 6), and translabyrinthine approach (Figure 7).16Silk P.S. Lane J.I. Driscoll C.L. Surgical approaches to vestibular schwannomas: What the radiologist needs to know.Radiographics. 2009; 29: 1955-1970Crossref PubMed Scopus (25) Google Scholar Each of these approaches offers certain advantages but also imposes certain limitations. The MCF approach is typically used for small tumors that are entirely or predominantly intracanalicular with less than 1 cm of CPA extension and for patients with preserved hearing, as this is the only technique that allows complete access to the IAC without violating inner ear structures.20Jackler R.K. Pitts L.H. Selection of surgical approach to acoustic neuroma: 1992.Neurosurg Clin N Am. 2008; 19: 217-238Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar However, this technique provides limited exposure of the CPA cistern, and therefore tumors with a significant CPA component cannot be treated with this method. The retrosigmoid approach allows greater access to the CPA while maintaining the option of hearing preservation.21Ojemann R.G. Retrosigmoid approach to acoustic neuroma (vestibular schwannoma).Neurosurgery. 2001; 48: 553-558Crossref PubMed Scopus (59) Google Scholar However, this technique provides limited access to the lateral IAC, which increases the risk of residual tumor when the VS occupies more than two-thirds of the IAC. The translabyrinthine approach, which entails a complete mastoidectomy and labyrinthectomy, allows for a wide CPA exposure as well as access to the cochlea and vestibule and results in the lowest tumor recurrence rate.22Schmerber S. Palombi O. Boubagra K. et al.Long-term control of vestibular schwannoma after a translabyrinthine complete removal.Neurosurgery. 2005; 57: 693-698Crossref PubMed Google Scholar However, if the patient has any preservation of hearing before surgery, this technique will eliminate that hearing.Figure 7Translabyrinthine approach. Axial CT image demonstrating postsurgical changes after translabyrinthine approach for resection of a VS, with a triangular translabyrinthine fat graft in place and a fluid collection overlying the mastoidectomy.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Review of these surgical approaches underscores the importance of imaging in delineating the full extent of the tumor preoperatively so that the optimal approach may be chosen. Meningiomas are the second most common lesion in the CPA representing approximately 10% of all tumors in this location.3St Martin M.B. Hirsch B.E. Imaging of hearing loss.Otolaryngol Clin North Am. 2008; 41: 157-178Abstract Full Text Full Text PDF PubMed Scopus (25) Google Scholar They arise from the arachnoid meningothelial cells and grow slowly in the CPA, from where they can easily extend into the IAC. However, unlike VS, they typically do not widen the porus acusticus.23Helie O. Soulie D. Sarrazin J.L. et al.Magnetic resonance imaging and meningiomas of the posterior cerebral fossa: 31 cases.J Neuroradiol. 1995; 22: 252-270PubMed Google Scholar CT may be particularly valuable in the diagnosis of CPA meningioma as it can demonstrate calcification, which is seen in about 25% of these tumors (Figure 8) and may also demonstrate hyperostosis of the adjacent bone, which is a common finding. On MRI, CPA meningiomas are usually broad-based dural lesions attached to the petrous dura mater or to the inferior aspect of the tentorium. Meningiomas are usually isointense to cortex on T1- and T2-weighted images, and they enhance intensely and homogeneously on postcontrast images with thickening and enhancement of the adjacent dura—the so-called dural tail sign, which, however, is not specific to meningiomas.24Guermazi A. Lafitte F. Miaux Y. et al.The dural tail sign—beyond meningioma.Clin Radiol. 2005; 60: 171-188Abstract Full Text Full Text PDF PubMed Scopus (88) Google Scholar Less commonly, meningiomas may also present as smaller intracanalicular tumors, with little or no component in the CPA (Figure 9).Figure 9Meningioma. (A) Axial T2 image demonstrates a small T2 isointense mass in the left IAC with mild hyperostosis of the adjacent petrous bone. (B) Axial T1 postcontrast image demonstrates intense enhancement of this intracanalicular meningioma and a small dural tail.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Facial nerve schwannomas (FNS) involving the CPA-IAC may be difficult to distinguish from VS owing to similar anatomical location and clinical presentation. Classically, these tumors have been described as tubular-enhancing masses on MRI with smooth enlargement of the facial nerve canal on CT. However, the imaging appearance of FNS is actually more varied and depends on the specific segments of the facial nerve that are involved.25Wiggins 3rd, R.H. Harnsberger H.R. Salzman K.L. et al.The many faces of facial nerve schwannoma.Am J Neuroradiol. 2006; 27: 694-699PubMed Google Scholar For example, FNS that extend from the CPA-IAC to the geniculate fossa typically have a dumbbell shape (Figure 10), whereas FNS centered in the geniculate ganglion and greater superficial petrosal nerve may present as a round, MCF mass (see article by Chung et al in this issue). Meningeal metastases must also be considered in the differential diagnosis of an enhancing lesion in the CPA-IAC. Most commonly, these metastases arise from primary malignancies of the breast or lung or from melanoma. Although in some instances, it may be difficult to distinguish these lesions from the more common benign lesions within this location, particularly when they are the first or only site of intracranial metastatic disease (Figure 11), a few imaging features are more characteristic of metastatic lesions. For example, metastatic lesions tend to be eccentric to the IAC, they may elicit vasogenic edema in the adjacent cerebellum or brainstem or both, and they are often associated with multiple other central nervous system lesions26Warren F.M. Shelton C. Wiggins 3rd, R.H. et al.Imaging characteristics of metastatic lesions to the cerebellopontine angle.Otol Neurotol. 2008; 29: 835-838Crossref PubMed Scopus (11) Google Scholar (Figure 12).Figure 12Metastatic disease in 40-year-old woman with breast cancer. Axial T1 postcontrast image demonstrates a round, enhancing metastatic lesion within the right CPA that is located eccentric to the IAC and is associated with multiple other metastatic lesions within the cerebellum.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Congenital epidermoid cysts (CEC), also known as congenital cholesteatoma, are the third most common mass in the CPA after VS and meningiomas.27Bonneville F. Sarrazin J.L. Marsot-Dupuch K. et al.Unusual lesions of the cerebellopontine angle: A segmental approach.Radiographics. 2001; 21: 419-438Crossref PubMed Scopus (131) Google Scholar, 28Mafee M.F. Acoustic neuroma and other acoustic nerve disorders: Role of MRI and CT: Analysis of 238 cases.Semin Ultrasound CT MR. 1987; 8: 266-283Google Scholar They are congenital lesions that arise from the accidental inclusion of ectodermal epithelial tissue during neural tube closure. Their growth is due to accumulation of keratin produced by desquamation of the squamous epithelium lining the mass. These slow-growing masses encase nerves and vessels in the cisterns rather than displacing them.29Gao P.Y. Osborn A. Smirniotopoulos J. et al.Epidermoid tumor of the cerebellopontine angle.Am J Neuroradiol. 1992; 13: 863-872PubMed Google Scholar, 30Mafee M.F. MRI and CT in the evaluation of acquired and congenital cholesteatomas of the temporal bone.J Otolaryngol. 1993; 22: 239-248PubMed Google Scholar, 31Mafee M.F. Kumar A. Heffner D.K. Epidermoid cyst (cholesteatoma) and cholesterol granuloma of the temporal bone and epidermoid cysts affecting the brain.Neuroimaging Clin N Am. 1994; 4: 561-578PubMed Google Scholar, 32Valvassori G.E. Imaging of the temporal bone.in: Mafee M.F. Valvassori G.E. Becker M. Imaging of the Head and Neck. ed 2. Georg Thieme Verlag, Stuttgart2005: 3-133Google ScholarOn imaging, CEC have characteristic irregular and lobulated margins. On CT, CEC are hypoattenuating lesions. On MRI, CEC are T1 hypointense and T2 hyperintense, mimicking a fluid-filled cyst. However, on FLAIR, CEC are mildly hyperintense and on diffusion-weighted imaging (DWI), they are strikingly hyperintense (Figure 13). It is noteworthy that CEC are often indistinguishable from arachnoid cysts on T1- and T2-weighted sequences. However, FLAIR and DWI allow reliable differentiation of these 2 lesions as, like CSF, arachnoid cysts will be suppressed on FLAIR and will have very low signal intensity on DWI (Figure 14) (see article by Mafee et al in this issue).Figure 14Arachnoid cyst. (A) Axial T1 and (B) T2 images demonstrate a lesion with fluid signal intensity filling the right cerebellomedullary angle. (C) Axial DWI image demonstrates that the lesion is hypointense (ie, facilitates diffusion), which is consistent with an arachnoid cyst.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Although this review is by no means an exhaustive review of tumors found in the CPA, the aim was to focus on the most common tumors in this location with special emphasis on VS, which account for the vast majority of tumors in the CPA-IAC." @default.
• W2097408033 created "2016-06-24" @default.
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• W2097408033 date "2014-03-01" @default.
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• W2097408033 title "Imaging of vestibular schwannoma and other cerebellopontine angle tumors" @default.
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