Cerebellopontine angle (CPA) tumors are masses located in the region between the cerebellum and pons. They constitute the most frequently diagnosed tumors of the posterior fossa and account for up to 10% of all intracranial neoplasms.
A majority of the CPA tumors are benign and can be divided into various types, depending on their histopathological features, such as lipomas, acoustic neuromas, meningiomas, hemangiomas and vascular malformations. Most CPA tumors belong to the category of vestibular neuromas (VS, acoustic neuromas) and their symptomatology is dependent upon their anatomical location and their size.
Vestibular neuromas typically cause no symptoms in the initial stages of the disease; occasionally, unilateral tinnitus, headaches, and sensorineural impairment are experienced . Sudden-onset hearing loss is attributed to vascular irregularities since the impairment caused by the acoustic neuroma develops gradually . Similarly, during the initial stages of the condition, vertigo and imbalance tend to be mild, since the unaffected side compensates for the abnormalities. Other symptoms include facial asymmetry, pain, and weakness; These symptoms also tend to be mild and are attributed to the expansion of the tumor with compression of the trigeminal nerve  . A severe degree of facial asymmetry and weakness experienced by the patient usually indicates an extensive tumor and constitutes a more emergent condition.
At the more progressed stages of the disease, ataxia, tremor and loss of coordination begin to manifest. If a VS is left untreated or undiagnosed, it is expected to ultimately lead to communicative hydrocephalus due to elevated intracranial pressure .
Meningiomas are a different subcategory of CPA tumors and are the most frequently diagnosed CPA tumors of non-acoustic nature. Tinnitus, deafness, and imbalance are amongst the primary symptoms induced by meningiomas. Should they expand excessively, symptoms associated with trigeminal involvement and hydrocephalus may be elicited.
Entire Body System
Brainstem candidiasis in immunocompetent host can masquerade the CP angle tumor. The MRI and MRS are not always diagnostic. [ncbi.nlm.nih.gov]
Mendelsohn D.B. 2009 19 A 47-year old male with a cerebellopontine angle tumor. ( 19744047 ) Bumm K....Steinhart H. 2009 20 Chemical meningitis following cerebellopontine angle tumor surgery. ( 18312887 ) Sanchez G.B....Haynes D.S. 2008 21 Brain stem candidiasis [malacards.org]
A 17-month-old boy presented with abnormal movements of his left arm and difficulty walking for 2 weeks. The MRI of the patient showed an enhancing partially cystic and partially solid mass in the right CP angle cistern, interpreting as CP tumor. [ncbi.nlm.nih.gov]
Unable to Stand
He had right cerebellar signs and truncal ataxia, and was unable to stand without support. Audiogram showed profound mixed type of hearing loss in the right ear, with normal hearing in the left ear. [neurologyindia.com]
Vestibular neuromas typically cause no symptoms in the initial stages of the disease; occasionally, unilateral tinnitus, headaches, and sensorineural impairment are experienced. [symptoma.com]
The most common symptoms from tumors in this location include: hearing loss, tinnitus, balance dysfunction. Larger tumors may also result in numbness of the face due to compression of the fifth cranial nerve. [sdearcenter.com]
In some patients the tinnitus is a pure tone, and in others the tinnitus is a noise. Many patients with acoustic neuroma have combined tinnitus and hearing loss. [hopkinsmedicine.org]
3a88T MRI study. ( 29343821 ) Yamamoto H....Kohmura E. 2018 4 Glioneuronal Heterotopia Presenting as Cerebellopontine Angle Tumor of Cranial NerveA VIII. ( 29625310 ) Peris-Celda M....Van Gompel J.J. 2018 5 The relation between flocculus volume and tinnitus [malacards.org]
Figure 10: Mastoid pneumocele on the left side is causing tinnitus. [mjdrdypu.org]
Pulsatile tinnitus: Imaging and differential diagnosis. Dtsch Arztebl Int 2013;110:451-8. 40. Wuertenberger CJ, Rosahl SK. [mjdrdypu.org]
CPA tumor should be a prime suspicion in patients with acute vertigo and ipsilesional HIN, especially when the vertigo accompanies hearing impairments. [ncbi.nlm.nih.gov]
Similarly, during the initial stages of the condition, vertigo and imbalance tend to be mild, since the unaffected side compensates for the abnormalities. [symptoma.com]
It can be asymptomatic in early stage or it can give vertigo, tinnitus, or hearing loss. Vascular compression of the vestibulochoclear nerve also causes vertigo and tinnitus. [mjdrdypu.org]
Cerebellopontine Angle Lesion (relate to invasion or compression of local structures of the brainstem and cerebellum; the more common presenting symptoms and signs are in red ) • Unilateral sensorineuronal hearing loss (CN VIII) • Tinnitus (CN VIII) • Vertigo [casemed.case.edu]
CONCLUSIONS: The contribution of hyperventilation on vestibular nystagmus differs depending on the disease phase or underlying pathologies. [ncbi.nlm.nih.gov]
Identifier Bruns_nystagmus Title Bruns nystagmus due to a cerebellopontine angle tumor Subject Jerk Nystagmus, Gaze Evoked Nystagmus, Vestibular Nystagmus Creator Daniel R. [collections.lib.utah.edu]
: Imbalance, nystagmus and diplopia in Wallemberg's Syndrome. Acta Otolaryng. 55, 269–281, 1962. [link.springer.com]
[…] study versus placebo. ( 25924803 ) Bozorg Grayeli A....Sterkers O. 2015 10 Clinical use of skull tap vestibular evoked myogenic potentials for the diagnoses of the cerebellopontine angle tumor patients. ( 24804198 ) Yavuz E....Delgado R.E. 2014 11 Bruns nystagmus [malacards.org]
A case of trigeminal neuralgia caused by a contralateral acoustic neurinoma is reported. The patient's tic pain was completely alleviated after removal of the tumor. [ncbi.nlm.nih.gov]
Other tumours may cause trigeminal neuralgia without other obvious angle symptoms or signs; this occurred with some epidermoids and with a few meningiomas. [link.springer.com]
neuralgia: a case report. ( 3497359 ) Snow R.B....Fraser R.A. 1987 47 Facial nerve function in cerebellopontine angle tumor surgery. ( 3878090 ) Gardner G....Robertson J.H. 1985 48 Chronic granulomatous abscess simulating cerebellopontine angle tumor [malacards.org]
neuralgia (fifth cranial nerve) and hemifacial spasm ("facial tic", 7th cranial nerve). [uniklinik-freiburg.de]
After a stellate ganglion block with transient nausea and dizziness, the pain was noticeably relieved. [ncbi.nlm.nih.gov]
But also dizziness, tinnitus, torticollis and high blood pressure may arise. [uniklinik-freiburg.de]
Other symptoms of the acoustic neuroma include asymmetric tinnitus (ringing in the ear), dizziness and disequilibrium (difficulty with balance). [hopkinsmedicine.org]
Hearing loss, dizziness, and tinnitus were the most common presenting symptoms. [neurocirugia.com]
About half the patients had signs of cerebellar ataxia at presentation (table), with only a third presenting with subjective hearing loss. [jnnp.bmj.com]
Vestibular schwannomas are optimally depicted with the aid of a gadolinium-enhanced magnetic resonance imaging scan (MRI), since it can illustrate even tumors that are smaller than 4 mm . In cases where gadolinium-enhanced MRI is not available, a computerized tomography scan can help to detect small acoustic neuromas; nevertheless, this modality is limited to detecting tumors larger than 1 cm .
Brain stem evoked response audiometry (BERA) is a method that monitors cerebral response to auditory stimuli (clicking sounds). The patterns are evaluated using the normal side as a comparative parameter; retrocochlear tumors are suspected when the pattern deviates considerably from the one displayed on the normal side or when there is an interaural delay. Tumors that are small in size cannot be detected via this modality.
Meningiomas cannot be distinguished from acoustic neuromas by means of examinations which evaluate auditory capacity. A gadolinium-enhanced MRI is the optimum choice in order to diagnose a suspected meningioma. Typical characteristics of a meningioma include a hemispherical tumor, with a broad attachment, forming an obtuse angle with the bone and with an eccentric location.
Eventhough surgical treatment remains the recommended attitude, these cases show that conservative treatment can be an option in some cases. [ncbi.nlm.nih.gov]
The Line of treatment adopted The treatment is in two types. One is Surgery and the other is radiation therapy. Surgery is removal of tumour or most of the lesions. Radiation therapy is targeted to arrest the growth of the tumour. [kimshospitals.com]
Mehta is certified in Gamma Knife radiosurgery and performs radiosurgery treatments at the San Diego Gamma Knife Center. Surgical removal of the tumor with microsurgical techniques. [sdearcenter.com]
On the surgical treatment of bilateral acoustic neurinomas. Acta neurochir., Wien, 1964 (in press). Google Scholar Olivecrona, H., On suprasellar cholesteatomas. Brain, London, 55 (1932), 122–134. Google Scholar Pool, J. L., and A. A. [link.springer.com]
This distinction is essential because of the difference in the overall treatment plan and prognosis. [surgicalneurologyint.com]
[…] related hypertrophic pachymeningitis  CPA Metastases in cancer patients with inner ear symptoms (rare) Diagnosis [ edit ] Radiography [ edit ] Subsequent to diagnosis of sensorineural hearing loss, and differential diagnosis of retrocochlear or neural etiologies [en.wikipedia.org]
Etiology A study by Berkowitz et al found a tendency for patients diagnosed with vestibular schwannoma to be white, aged 50-60 years, and in a professional occupation, but there seemed to be no specific sex preference in the disease. [emedicine.medscape.com]
The etiology and the radiological features of CPA lipomas are reviewed and discussed. CPA lipomas are maldevelopmental lesions that may cause slowly progressive symptoms. Neuroradiology enables a reliable preoperative diagnosis. [neurocirugia.com]
Objective To analyze the epidemiological, clinical and neuropathological data of cases of cerebellopontine angle (CPA) tumors. [revistaneurocirugia.com]
Epidemiology Rare, approximately 1/100,000 per year Anatomy The cerebellopontine angle is located in the posterior fossa. Key to Figure Above 1. Sensory root of trigeminal nerve 2. Pons 3. Vestibulocochlear nerve 4. Facial nerve 5. Abducent nerve 6. [casemed.case.edu]
Epidemiology Acoustic neuroma represents 8% of all intracranial tumours and 80% of those arising at the CPA. [ 1 ] Risk factors Confirmed risk factors include: Neurofibromatosis. [ 2 ] High-dose ionising radiation (children who received radiation for [patient.info]
Epidemiology Frequency See the list below: CPA tumors other than vestibular schwannoma (percentage of total CPA occurrence) Meningiomas (3-13%) Epidermoids (2-6%) Facial and lower cranial nerve schwannomas (1-2%) Arachnoid cysts (1%) Rare tumors Lipomas [emedicine.medscape.com]
Previously reported cases of trigeminal neuralgia caused by contralateral cerebellopontine angle tumors are reviewed, and the pathophysiology of this disorder is discussed. [ncbi.nlm.nih.gov]
Clinically relevant depression and/or anxiety was reported by approximately one third of individuals in the study, while 48% stated that they suffered from high fatigue levels.  Relevant Anatomy See the Pathophysiology section. [emedicine.medscape.com]
Pathophysiology [ edit ] Various kinds of tumors, usually primary and benign, are represented in the pathology. [en.wikipedia.org]
Thorough understanding of patient pathophysiology, meticulous planning, and better communication with the surgeons are necessary for the successful patient outcome. Financial support and sponsorship Nil. [saudija.org]
Reminding of one or oth of the above possibilities whenever we are performing surgery around the superior petrosal vein may help us to prevent the rare but fatal complication of this kind. [jkns.or.kr]
It is useful to wrap skin flap in moist raytech to prevent from dessication during case. [medicine.uiowa.edu]
He argued that benign tumors should be removed completely in order to prevent recurrence at a later stage, even at the expense of a somewhat higher perioperative mortality rate. [clinicalgate.com]
The importance of lung isolation to prevent spillage and avoidance of one lung ventilation to control the arterial CO 2 are highlighted in this case report. [saudija.org]
Conflicts between the cranial nerves and blood vessels (microvascular compression) can be eliminated by relocating the irritating vessel from the nerve and inserting Teflon -wool or a muscle patch as cushion between them to prevent new contact. [uniklinik-freiburg.de]
- Mathew GD, Facer GW, Suh KW, Houser OW, O'Brien PC. Symptoms, findings, and methods of diagnosis in patients with acoustic neurinoma. Laryngoscope. 1978;88:1893–1903
- Braunwald E, Fauci AS, Kasper DL, Hauser SL, Longo DL, Jameson JL. Tumores primários e metastáticos do sistema nervoso. 2002. Harrison, Medicina Interna, ed. Rio de Janeiro, McGraw Hill.
- Plum F, Posner JB. Andreoli TE, Benett JC, Carpenter CCJ, Plum F. Intracranial neoplasms, CNS complications of cancer, and states of altered intracranial pressure. 4th ed. Philadelphia: Saunders; 1997. Cecil essentials of medicine; pp. 888–897.
- Tierney LM., Júnior . Current medical diagnosis and treatment. 43th ed. United States: Mc Graw Hill; 2004.
- Zhang MS, Zhang HW, Gu CY, et al. Strategy of the diagnosis and treatment for hydrocephalus associated with acoustic neuroma. Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2016 Jun 7;51(6):419-22.
- Wayman JW, Dutcher PO, Manzione JV, Nelson CN, Kido DK. Gadolinium-DPTA-enhanced magnetic resonance imaging in cerebellopontine angle tumors. Laryngoscope. 1989;99:1167–1170.
- House CT, Baltimore WF. A history of acoustic tumor surgery. University Park Press. 1979; 9–32.