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Cerebellopontine Angle Tumor

Neoplasm of Cerebellopontine Angle

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.

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]
  • 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]
  • Classic presentation of acoustic neuroma confined to the internal auditory canal, involves unilateral progressive hearing loss, vestibular dysfunction and tinnitus.[patient.info]
  • But also dizziness, tinnitus, torticollis and high blood pressure may arise.[uniklinik-freiburg.de]
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]
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]
  • Abstract We describe for the first time localized necrotizing meningoencephalitis as the cause of functional hearing loss, facial nerve palsy, and vertigo after cochlear implant (CI) surgery.[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]
  • 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]
  • […] studies report a female predominance. [1], [2] Average duration of symptoms varies from 9.3 to 10.6 years. [4], [13] Unilateral hearing loss is the most common symptom, and can often be of sudden onset. [13] The other common symptoms are tinnitus and vertigo[neurologyindia.com]
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]
  • Stellate ganglion block may provide pain relief to some patients who are suspected to have symptomatic trigeminal neuralgia.[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 (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]
Cerebellar Ataxia
  • About half the patients had signs of cerebellar ataxia at presentation (table), with only a third presenting with subjective hearing loss.[jnnp.bmj.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.

Candida
  • On the specimen, there were numerous fungal organisms consistent with Candida species. Our patient recovered completely after and 377-day voriconazole treatment. He was still quite well without neurologic sequelae at follow-up for 2 years.[ncbi.nlm.nih.gov]

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]
  • Followed by voriconazole treatment, the patient could be treated successfully.[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]

Prognosis

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]

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]
Sex distribution
Age distribution

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]
  • 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]

Prevention

  • 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]
  • 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]
  • *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

Article

  1. 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
  2. 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.
  3. 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.
  4. Tierney LM., Júnior . Current medical diagnosis and treatment. 43th ed. United States: Mc Graw Hill; 2004.
  5. 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.
  6. 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.
  7. House CT, Baltimore WF. A history of acoustic tumor surgery. University Park Press. 1979; 9–32.

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Last updated: 2019-07-11 20:20