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.
Presentation
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 [1]. Sudden-onset hearing loss is attributed to vascular irregularities since the impairment caused by the acoustic neuroma develops gradually [2]. 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 [3] [4]. 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 [5].
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.
Respiratoric
- Aspiration
Figure 3: Removal of a tumor (vestibular schwannoma) in the cerebellopontine angle using an ultrasound aspirator. [uniklinik-freiburg.de]
Clinical features Chochlear CN-VIII : asymmetric sensorineural hearing loss & tinnitus Vestibular CN-VIII : dysequilibrium & vertigo CN-VII : facial weakness CN-VI : lateral rectus palsy CN-IX : dysphagia CN-X : hoarsness & aspiration CN-XI [slideshare.net]
On aspiration, a small quantity of xanthochromic fluid and altered blood was seen. Arterial bleeding occurred from 2 needle hole sites, which was controlled with difficulty. There was good tumor brain interface. [neurologyindia.com]
Paragangliomas : A diagnostic dilemma on fine needle aspirates. Indian J Cancer 1993;30:152-7. [ PUBMED ] 10. Scheithauer BW, Brandner S, Soffer D. Spinal paraganglioma. In: Louis DN, Ohgaki H, Weistler OD, Cavenee WK, editors. [indianjotol.org]
The first patient, requiring the emergency evacuation of postoperative haematoma, died of aspiration pneumonia partially attributable to poor gag and swallow reflexes secondary to ninth/10th nerve palsies after surgery. [jnnp.bmj.com]
- Hoarseness
As a result, facial pain, paralysis of facial muscles, diplopia (double vision), dysphagia (swallowing disorder) or hoarseness may occur. [uniklinik-freiburg.de]
Clinical features Chochlear CN-VIII : asymmetric sensorineural hearing loss & tinnitus Vestibular CN-VIII : dysequilibrium & vertigo CN-VII : facial weakness CN-VI : lateral rectus palsy CN-IX : dysphagia CN-X : hoarsness & aspiration CN-XI [slideshare.net]
We report a case of a 55-year-old female presented with otalgia and decreased hearing in the left ear, hoarseness of voice, difficulty in swallowing, nasal regurgitation, and tinnitus. [indianjotol.org]
Eyes
- Diplopia
[…] location of the lesion and may include any of the following: Hearing loss (most commonly unilateral) Tinnitus (ringing sounds in ear) Vertigo/unsteadiness/disequilibrium Headache Facial hypesthesia Decreased facial sensation Facial weakness and spasm Diplopia [thebrainandspine.com]
CASE HISTORY A 21-year-old gentleman presented with numbness of right side of the face for 1 month, and diplopia for 1 week. Neurological examination showed numbness in right V1, V2 distribution, and paresis of right VI nerve. [surgicalneurologyint.com]
• Facial numbness (CN V sensory) • Absent corneal reflex (CN V sensory) • Difficulty chewing (CN V motor) • Facial weakness (CN VII) • Dysphagia (CN IX or X) • Babinski responses/hyperreflexia (2nd to compression of pontine corticospinal tracts) • Diplopia [casemed.case.edu]
: Imbalance, nystagmus and diplopia in Wallemberg's Syndrome. Acta Otolaryng. 55, 269–281, 1962. [link.springer.com]
As a result, facial pain, paralysis of facial muscles, diplopia (double vision), dysphagia (swallowing disorder) or hoarseness may occur. [uniklinik-freiburg.de]
Ears
- Tinnitus
Vestibular neuromas typically cause no symptoms in the initial stages of the disease; occasionally, unilateral tinnitus, headaches, and sensorineural impairment are experienced. [symptoma.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]
Figure 10: Mastoid pneumocele on the left side is causing tinnitus. [mjdrdypu.org]
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]
Classic presentation of acoustic neuroma confined to the internal auditory canal, involves unilateral progressive hearing loss, vestibular dysfunction and tinnitus. [patient.info]
- Pulsatile Tinnitus
Pulsatile tinnitus: Imaging and differential diagnosis. Dtsch Arztebl Int 2013;110:451-8. 40. Wuertenberger CJ, Rosahl SK. [mjdrdypu.org]
Psychiatrical
- Aggressive Behavior
behavior.[ 10 ] One of the best characterized of these tumors is the medulloblastoma, which occurs in the posterior fossa while those occurring in the cerebrum are morphologically identical to the aforementioned, they are referred to as supratentorial [surgicalneurologyint.com]
Although the angioblastic variant has been associated with more aggressive behavior, no significant correlation has been found between the other histologic subtypes and clinical behavior. [emedicine.medscape.com]
Neurologic
- Vertigo
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]
Symptoms of Cerebellopontine angle (CPA) tumors The symptoms depend upon the size and location of the lesion and may include any of the following: Hearing loss (most commonly unilateral) Tinnitus (ringing sounds in ear) Vertigo/unsteadiness/disequilibrium [thebrainandspine.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]
- Nystagmus
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]
On the affected side, there is jerk nystagmus with the fast phase to the affected side (if the nystagmus is cerebellar; but in the early stage the initial nystagmus may be of vestibular origin with the fast phase to the unaffected side). [mrcophth.com]
Bruns nystagmus is an unusual type of bilateral nystagmus most commonly occurring in patients with cerebellopontine angle tumours. [diki.pl]
- Trigeminal Neuralgia
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]
- Dizziness
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]
- Cranial Neuropathy
With brainstem extension, midfacial and corneal hypesthesia, hydrocephalus, and other cranial neuropathies become more prevalent. [en.wikipedia.org]
Partial surgical resection is indicated only to alleviate intractable cranial neuropathies or relieve brainstem compression 6). 2002 Tankéré et al., report four new cases of CPA lipomas diagnosed in the Department of Otorhinolaryngology-Head and Neck [neurocirugia.com]
Workup
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 [6]. 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 [7].
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.
Colonoscopy
- Polyps
NIH: National Cancer Institute Biopsy - polyps (Medical Encyclopedia) Cherry angioma (Medical Encyclopedia) [ Read More ] Brain Diseases The brain is the control center of the body. It controls thoughts, memory, speech, and movement. [icdlist.com]
Treatment
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]
Embolization (interventional vascular treatment method using catheters) is suitable for the treatment of vascular processes. In some tumors radiation therapy and radiosurgery are employed very successfully. [uniklinik-freiburg.de]
Prognosis
This distinction is essential because of the difference in the overall treatment plan and prognosis. [surgicalneurologyint.com]
The prognosis of CPA medulloblastomas remains poor despite of all available modalities of treatment and a combination treatment with surgical decompression followed by radiotherapy and/or chemotherapy is the best modality as their vermian counterparts [pediatricneurosciences.com]
Etiology
[…] related hypertrophic pachymeningitis [3] 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]
Epidemiology
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, pathogenesis, and genetics of acoustic tumors. Otolaryngol Clin North Am 1992;25:499-520. House WF. A history of acoustic tumor surgery: 1917-1961, the Dandy era. In: House WF, Luetje CM, editors. Acoustic tumors, vol. 1. [medigraphic.com]
Pathophysiology
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. [2] 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]
Pathophysiology of acoustic tumors. In: House WF, Luetje CM, editors. Acoustic tumors, vol. 1. Baltimore: University Park Press, 1979; pp:45-83. Cushing H. [medigraphic.com]
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]
Prevention
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]
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]
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]
*while the posterior meatal lip is removed, mastoid air cells that extend into the lip may be opened, and must then be sealed carefully to prevent CSF leak or meningitis. [medschool.lsuhsc.edu]
References
- 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.