Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature.

Unusual Cerebellopontine Angle Neoplasms
Glenn W. Knox, M.D.
November 4, 1993

Cerebellopontine angle lesions are the predominant skull base neoplasms that affect the posterior fossa. Although vestibular schwannomas account for the majority of primary neoplasms, a wide array of other lesions must also be considered in the differential diagnosis. In Brackmann and Bartels' series of CPA neoplasms, vestibular schwannoma accounted for over 90%. The remaining primary tumors were meningiomas (3.1%), primary cholesteatomas (2.4%), facial nerve schwannomas (1.2%), other schwannomas (0.2%), and other tumors (1.9%).

Meningiomas represent up to 18% of all intracranial tumors and approximately 3% of CPA tumors. The cells lining the arachnoid villae are the cells of origin. These cells are distributed throughout the intracranial space predominantly in relation to veins and dural sinuses. Meningiomas are benign but locally aggressive tumors which occur at different anatomic sites in the following order of frequency: parasaggital, falx, convexity, olfactory groove, tuberculum sellae, sphenoid ridge, CPA, tentorium, lateral ventricle, clivus. The gross appearance is typically a globular mass that is firmly adherent to the dura mater, with characteristic speckles scattered throughout the tumor that correspond to the microscopic psammoma bodies. The tumor displaces but does not invade adjacent neural tissue.

Case Presentation

A 51-year-old white man noted the onset of vertigo in 1980. This resolved, but returned in the late 1980s, reaching a peak in 1988. His vertigo symptoms had improved since then. He also complained of a multi-year history of mild right-sided tinnitus, which had been variable but more noticeable this year. He complained of a two-year history of mild right-sided hearing loss which had also been more noticeable this year. His major complaint was a sense of fullness in the right ear which he had noticed in January of 1993, which did not improve with medical treatment. Past medical history was noncontributory. Physical examination was unremarkable. A recent audiogram showed a right-sided 40 dB high frequency sensorineural hearing loss with a PB max of 96%. Tympanograms were type A. MRI revealed an intracanalicular mass.

The patient was admitted to TMH and underwent a right excision of cerebellopontine angle mass via the midfossa approach. During surgery, the tumor appeared to be associated with the superior vestibular nerve. The tumor was noted to be tightly adherent to the surrounding structures, was rubbery in consistency, and contained several apparent venous sinuses. The patient recovered uneventfully with useful hearing preserved. Pathology revealed benign fibrous tissue with abundant vessels, consistent with a benign vascular tumor.

Bibliography

Arriaga MA, Brackmann DE, Hitselberger WE. Extended middle fossa resection of petroclival and cavernous sinus neoplasms. Laryngoscope 1993;103:693-698.

Balkany T, Fradis M, Jafek BW, Rucker NC. Hemangioma of the facial nerve: role of the geniculate capillary plexus. Skull Base Surg 1991;1:59-63.

Batsakis JG. Tumors of the Head and Neck, 2nd ed. Baltimore: Williams & Wilkins, 1979.

Beatty CW, Ebersold MJ, Harner SG. Residual and recurrent acoustic neuromas. Laryngoscope 1987;97:1168-1171.

Bellet PS, Benton C Jr, Matt BH, Myer CM 3d. The evaluation of ear canal, middle ear, temporal bone, and cerebellopontine angle masses in infants, children, and adolescents. Adv Pediatr 1992;39:167-205.

Bourgouin PM, Tampiere D, Robitaille Y, Robert F, Bergeron D, del Carpio R, et al. Low-grade myxoid chondrosarcoma of the base of the skull: CT, MR, and histopathology. J Comput Assist Tomogr 1992;16:268-273.

Brackmann DE, Anderson RG. Cholesteatomas of the cerebellopontine angle. In: Silverstein H, Norrell H, editors. Neurological Surgery of the Ear. Birmingham: Aesculapius, 1979.

Brackmann DE, Bartels LJ. Rare tumors of the cerebellopontine angle. Otolaryngol Head Neck Surg 1980;88:555-559.

Brackman DE, Gherini SE. Differential diagnosis of skull base neoplasms involving the posterior fossa. In: Cummings CW, Harker MD, editors. Otolaryngology - Head and Neck Surgery IV. St. Louis: Mosby, 1986:3421-3436.

Brackmann DE, Kwartler JA. A review of acoustic tumors: 1983-1988. Am J Otol 1990;11:216-232.

Bushe KA, Naumann M, Warmuth-Metz M, Meizensberger J, Muller J. Maffucci's syndrome with bilateral cartilaginous tumors of the cerebellopontine angle. Neurosurgery 1990;27:625-628.

O'Keefe LJ, Ramsden RT, Birzgalis AR. Cerebellopontine angle lipoma. J Laryngol Otol 1993;107:553-555.

Cosnard G, Galidie G, Cordoliani YS, Le Gall R. Rare tumors of the cerebellopontine angle. Ann Radiol (Paris) 1992;35:36-43.

Dort JC, Fisch U. Facial nerve schwannomas. Skull Base Surg 1991;1:51-56.

Elster AD, Challa VR, Gilbert TH, Richardson DM. Meningiomas: MR and histopathologic features. Radiology 1989;179:857-862.

Gao PY, Osborn AG, Smirniotopoulos JG, Harris CP. Radiologic-pathologic correlation: epidermoid tumor of the cerebellopontine angle. AJNR Am J Neuroradiol 1992;13:863-872.

Garcia CA, McGarry PA, Rodriguez F. Primary intracranial squamous cell carcinoma of the right cerebellopontine angle. J Neurosurg 1981;54:824-828.

Giddings NA, Brackmann DE, Kwartler JA. Transcanal infracochlear approach to the petrous apex. Otolaryngol Head Neck Surg 1991;104:29-36.

Haberkamp TJ, Monsell EM, House WF, Levine SC, Piazza L. Diagnosis and treatment of arachnoid cysts of the posterior fossa. Otolaryngol Head Neck Surg 1990;103:610-614.

Hamann G, Bier B, Schimrigk K. Basilar aneurysm in the cerebello-pontine angle. Case report with review of the literature. Nervenarzt 1992;63:113-115.

Iplikcioglu AC, Ozer F, Benli K, Bertan V, Ruacan S. Malignant teratoma of the cerebellopontine angle: case report. Neurosurgery 1990;27:137-139.

Jackler RK, Shapiro MS, Dillon WP, Pitts L, Lanser MJ. Gadolinium-DTPA enhanced magnetic resonance imaging in acoustic neuroma. Otolaryngol Head Neck Surg 1990;102:670-677.

Jamjoom A, Jamjoom ZA, Griffiths D, Moss T, Coakham H. Cerebellopontine angle epithelial cyst. A case report. Neurosurg Rev 1993;16:67-70.

Janecka IP, Sekhar LN, Horton JA, Yonas H. General blood flow evaluation. In: Cummings CW, et al. Otolaryngology - Head and Neck Surgery Update II. St. Louis: Mosby, 1990:54-63.

Jenkins HA. Hearing preservation in acoustic neuroma surgery. Laryngoscope 1992;102:125-128.

Jerger J, Oliver TA, Jenkins H. Suprathreshold abnormalities of the stapedius reflex in acoustic tumors: a series of case reports. Ear Hear 1987;8:131-139.

Johnson EW. Results of auditory tests in acoustic tumor patients. In: House WF, Luetje CM, editors. Acoustic Tumors. Baltimore: University Park Press, 1979.

Kartush JM, Niparko JK, Graham MD, Kemink JL. Electroneurography: preoperative facial nerve assessment for tumors of the temporal bone. Otolaryngol Head Neck Surg 1987;97:257-261.

Kasantikul V, Netsky MG, Glasscock ME 3d. Acoustic neurilemmoma: clinicoanatomical study of 103 patients. J Neurosurg 1980;52:28-35.

Kitamura K, Futaki T, Miyoshi S. Fluctuating hearing loss in lipoma of the cerebellopontine angle. ORL J Otorhinolaryngol Relat Spec 1990;52:335-339.

Knorr JR, Ragland RL, Smith TW, Davidson RI, Keller JD. Squamous carcinoma arising in a cerebellopontine angle epidermoid: CT and MR findings. AJNR Am J Neuroradiol 1991;12:1182-1184.

Koci TM, Chiang F, Mehringer CM, Yuh WT, Mayr NA, Itabashi H, et al. Adult cerebellar medulloblastoma: imaging features with emphasis on MR findings. AJNR Am J Neuroradiol 1993;14:929-939.

Lalwani AK. Meningiomas, epidermoids, and other nonacoustic tumors of the cerebellopontine angle. Otolaryngol Clin North Am 1992;25:707-728.

Lalwani AK, Jackler RK. Preoperative differentiation between meningioma of the cerebellopontine angle and acoustic neuroma using MRI. Otolaryngol Head Neck Surg 1993;109:88-95.

Langman AW, Jackler RK, Althaus SR. Meningioma of the internal auditory canal. Am J Otol 1990;11:201-204.

Le-Marc Hadour F, Mouret P, Bost F, Pasquier D, Pasquier B, Charachon R. Lipoma of the internal auditory canal: an anatomo-clinical case study and review of the literature about cranial nerve lipomas. Arch Anat Cytol Pathol 1991;39:147-150.

Linthicum FH Jr , Waldorf R, Luxford WM, Caltogirone S. Infrared/video ENG recording of eye movements to evaluate the inferior vestibular nerve using the minimal caloric test. Otolaryngol Head Neck Surg 1988;98:207-210.

Lipper MH, Cail WS. Chordoma of the petrous bone. South Med J 1991;84:629-631.

Lo WW. Tumors of the temporal bone and cerebellopontine angle. In: Som PM, Bergeron RT, editors. Head and Neck Imaging, 2nd ed. St. Louis: Mosby, 1991:1046-1108.

Lunardi P, Missori P, Salvati M, Licastro G. Cholesterol granuloma of the cerebello-pontine angle. Clin Neurol Neurosurg 1992;94:265-268.

Martuza RL, Eldridge R. Neurofibromatosis 2 (bilateral acoustic neurofibromatosis). N Engl J Med 1988;318:684-688.

McCormick PC, Bello JA, Post KD. Trigeminal schwannoma: surgical series of 14 cases with review of the literature. J Neurosurg 1988;69:850-860.

Mohsenipour I, Deusch E, Ortler M, Pallua A. Meningiomas of the cerebellopontine angle. Neurochirugia 1993;36:90-92.

Pappas DG, Brackmann DE. Arachnoid cysts of the posterior fossa. Otolaryngol Head Neck Surg 1981;89:328-332.

Pappas DG, Schneiderman TS, Brackmann DE, Simpson LC, Chandra Sekar B, Sofferman RA. Cavernous hemangiomas of the internal auditory canal. Otolaryngol Head Neck Surg 1989;101:27-32.

Parnes LS, Lee DH, Peerless SJ. Magnetic resonance imaging of facial nerve neuromas. Laryngoscope 1991;101:31-35.

Press GA, Hesselink JR. MR imaging of cerebellopontine angle and internal auditory canal lesions at 1.5T. AJR Am J Roentgenol 1988;150:1371-1381.

Rubenstein LI. Tumors of the central nervous system. In: Firminger HI, editor. Atlas of Tumor Pathology, Second series, fascicle 6. Washington, D.C.: Armed Forces Institute of Pathology, 1972.

Russell DS, Rubenstein LJ. Pathology of Tumors of the Nervous System, 4th ed. Edinburgh: T & A Constable, 1977.

Sataloff RT, Davies B, Myers DL. Acoustic neuromas presenting as sudden deafness. Am J Otol 1985;6:349-352.

Saunders JE, Kwartler JA, Wolf HK, Brackmann DE, McElveen JT Jr. Lipomas of the internal auditory canal. Laryngoscope 1991;101:1031-1037.

Segall HD, Zee CS, Naidich TP, Ahmadi J, Becker TS. Computed tomography in neoplasms of the posterior fossa in children. Radiol Clin North Am 1982;20:237-253.

Sekhar LN, Jannetta PJ. Cerebellopontine angle meningiomas: microsurgical excision and follow-up results. J Neurosurg 1984;60:500-505.

Selters WA, Brackmann DE. Acoustic tumor detection with brainstem electric response audiometry. Arch Otolaryngol 1977;103:181-187.

Shinozaki N, Sekiya T, Suzuki S, Iwabuchi T, Suzuki M. Five cases of intracranial lipoma; CT and magnetic resonance images. No Shinkei Geka 1992;20:289-293.

Spector GJ, Wobol S, Thawley SE, Maisel RH, Ogura JH. Glomus jugulare tumors of the temporal bone: patterns of invasion of the temporal bone. Laryngoscope 1979;89:1628-1639.

Tekkok IH, Suzer T, Erbengi A. Non-acoustic tumors of the cerebellopontine angle. Neurosurg Rev 1992;117-123.

Valvanis A, et al. Clinical Imaging of the Cerebellopontine Angle. Berlin: Springer-Verlag, 1986.

Weber AL. Magnetic resonance imaging and computed tomography of the internal auditory canal and cerebellopontine angle. Isr J Med Sci 1992;28:173-182.

Westra I, Drummond GT. Occult pontine glioma in a patient with hemifacial spasm. Can J Ophthalmol 1991;26:148-151.

Wilms G, Plets C, Boossens L, Goffin J, Vanwambeke K. The radiological differentiation of acoustic neuronoma and meningioma occurring together in the cerebellopontine angle. Neurosurgery 1992;30:443-5, 445-6.

Yokota J, Imai H, Okuda O, Sato K. Inverted Bruns' nystagmus in arachnoid cysts of the cerebellopontine angle. Eur Neurol 1993;33:62-64.

Grand Rounds Archive | Department Home page


BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map |

©2001-2006 Baylor College of Medicine
Bobby R. Alford Department of Otolaryngology-Head and Neck Surgery
Mail: One Baylor Plaza, NA102, Houston, TX 77030
Phone: 713-798-5906
E-mail: oto@bcm.edu

Last modified: Feb. 15, 2006