Malignant tumors of the cpa includ ing primary malignancies of the cerebral paren. Computer tomography in the diagnosis of cerebellopontine. Introduction a schwannoma is a tumor that arises from schwann cells, named after their discoverer, theodor schwann a 19th century german histologist and. Paragangliomas are rarer still and, in a minority of cases 20%, are known to be caused by errors in the sdhd gene. Clinical features and outcomes in patients with non. Cerebellopontine angle tumor in a patient with a maternally. Hemangiopericytoma, cerebellopontine angle tumor, pericyte introduction hemangiopericytoma hpc is an aggressive and rare mesenchymal tumor which originates from the zimmermann pericyte1. The cerebellopontine angle is the site of the cerebellopontine angle cistern one of the subarachnoid cisterns that contains cerebrospinal fluid, arachnoid tissue, cranial nerves, and associated vessels. International journal of contemporary medicine surgery and radiology. Tumors managed by endoscopic third ventriculostomy. Objectivesnonacoustic tumours of the cerebellopontine angle differ from vestibular schwannomas in their prevalence, clinical features, operative management, and surgical outcome. Atypical teratoidrhabdoid tumor is a rare, aggressive intraaxial tumor of early childhood, most often presenting as heterogeneous intracranial mass in an infant. Metzemaekers a, peter jan van laar b, pim van dijk c, d, a department of neurosurgery, university of groningen, university medical center groningen, groningen, the netherlands.
These three tumors represent more than 90% of the tumors of the cerebellopontine angle. The characteristic morphological features of these depend on the tissue from which the tumors arise. Cerebellopontine angle cpa mbs are extremely rare tumors, with few cases. The majority of the cerebellopontine angle tumors are benign representing approximately 9698% of the cases valvassori, 1988. Conclusionwe suggest that malignant primary tumors of the central nervous system, especially those located at the cerebellopontine angle, in pediatric patients should be diagnosed with noninvasive methods such as cytogenetic analysis by stereotaxic sampling of tumoral tissue, or via very limited surgery due to the deleterious effects of extensive surgery on the morbidity of patients with an ini 1 negative medulloblastoma or an att rht. Cerebellopontine angle meningiomas account for 615% of the tumors in the cerebellopontine angle region. Surgical management of patients with cerebellopontine. Medulloblastoma in the cerebellopontine angle mimicking a. Of these benign tumors, 7590% are vestibular schwannomas these tumors. Surgical management of patients with cerebellopontine angle.
Cerebellopontine angle masses can be divided into four groups, based on imaging characteristics. Feb 08, 2021 this chapter describes the clinical presentation and imaging of the three most common tumors occurring in the cerebellopontine angle. Cranial nerve root entry zone primary cerebellopontine. Endoscopic techniques for cerebellopontine angle cpa surgery have been well studied and applied to tumor resection, vascular. For about 85% of tumors in the region of the cerebellopontine angle is acoustic neuroma, 10% are meningiomas and 5% are epidermoid tumor. There were 57 patients with cerebellopontine angle meningiomas, 37 women and 20 men, ranging in age from 38 to 89 years, with 10 over 70 years of age table 17. Present study aimed to evaluate the incidence of extra axial cerebellopontine angle tumours and also to characterize extra axial cerebellopontine angle tumours. Cerebellopontine angle myelography demonstrated nonfilling of the right internal auditory canal. Conventional approach to the cpa is through the retrosigmoid approach with optical aid by the microscope ojemann, 2000. The preoperative diagnosis is still troublesome due to the insufficiency of typical clinical and radiological features that discriminate cpa medulloblastoma from other cpa lesions such as cholesteatoma, epidermoid, meningioma, and schwannoma. As in this example, approximately 50% occur off of midline in infratentorial compartment. A highresolution 3 t mri study calcification of vestibular schwannoma. Usually, cerebellopontine angle cpa tumors are benign, classified into intra axial, extra axial, extradural and petrous axis tumors. Van hemert, 3 and john dornhoffer 1 1 department of otolaryngologyhead and neck surgery, 2 division of genetics, department of family medicine, winthrop p.
In this study, the presence of microhemorrhage mh and other imaging. Cerebellopontine angle cpa mbs are extremely rare tumors, with few cases previously described. Tumors of the cerebellopontine angle request pdf researchgate. Dif fusion imaging introduction the cerebellopontine angle cp a cistern is a subarachnoid space containing cranial nerves and vessels bathed in cerebrospinal fluid csf. Focal hyperintensity in the dorsal brain stem of patients with cerebellopontine angle tumor. The absence of a history of melanoma and nonspecific neuroradiological features presented a diagnostic dilemma. Acoustic neuromas are the most common tumor in the cerebellopontine angle cpa but are rare in the general population. Methodsa retrospective analysis of clinical notes identified 42 patients with nonacoustic tumours of the. Cerebellopontine angle tumors in infants and children pdf. Metastatic lesions involving the cerebellopontine angle. Indications include unilateral hearing loss, speech impediments, disequilibrium, tremors or other loss of motor control. The cp a is bounded by the pons, the anterior aspect of the cerebellum and the petrous. Cerebellopontine angle medulloblastomas are an outstandingly infrequent entity in children. Cerebellopontine angle cpa meningiomas comprise 10% of all intracranial meningiomas and due to their location, are producing different surgical challenges.
An acoustic neuroma is a benign tumour that develops in the acoustic or. Common pathologic entities in the cpa include vestibular schwannomas, which account for 10% of all primary brain neoplasms, meningiomas, and arachnoid cysts. Tumors of the cerebellopontine angle jama otolaryngology. Intracranial epidermoid tumors are commonly found in the cerebellopontine angle where they usually present with either trigeminal neuralgia or hemifacial spasm. Tumours of the cerebellopontine angle account for 8%10% of all intracranial tumours. Regarding tumors of the posterior fossa that may occur in the cpa. Of these benign tumors, 7590% are vestibular schwannomas these tumors are also called acoustic neurinomas. Utility of microhemorrhage as a diagnostic tool in. The purpose of this study is to assess the safety and clinical outcome of the treatment of cerebellopontine epidermoid tumors with gamma knife radiosurgery. The pathology of the cpa can arise from the different structures in its anatomy and vecinity, like the cisterns, arteries, meninges, nerves, skull base, and last but not least, the. Case report chondroma of the cerebellopontine angle. Epidermoid cysts are rare congenital tumors of the central nervous system cns, histologically benign and slow growing lesions.
The cerebellopontine angle syndrome is a distinct neurological syndrome of deficits that can arise due to the closeness of the cerebellopontine angle to specific cranial nerves. Management of tumors of the cerebellopontine angle in. Evolution of the management of hydrocephalus associated. They are characterized by the deep tumor location, narrow surgical field, and proximity to the brainstem, multiple pairs of vxi cranial nerves.
Request pdf on dec 31, 2012, madjid samii and others published tumors of the cerebellopontine angle find, read and cite all the research you need on. Tumor cerebellopontine angle wahyuliati mutiara medika. Jun 17, 2020 medulloblastoma mb is the most common malignant pediatric brain tumor arising in the cerebellum or the 4th ventricle. Evolution of the management of hydrocephalus associated with. Studies investigating the natural history and treatment of vestibular schwannomas have dominated the literature in this regard. Diagnostic efficacy of mri in the evaluation of cerebellopontine angle tumors. Cerebellopontine angle tumors are frequent and represent approximately. Cerebrospinal fluid cytology to aid the diagnosis of cerebellopontine angle tumors. The cerebellopontine angle cpa is the most common site for posterior fossa neoplasms. The combined microscopicendoscopic technique for radical. Cerebellopontine angle an overview sciencedirect topics. Mri evaluation of extra axial cerebello pontine angle tumours. The clinical treatment and outcome of cerebellopontine angle. Cerebrospinal fluid cytology to aid the diagnosis of.
Cerebellopontine angle cpa masses are relatively common. In preparation for a discussion of surgical options, the relevant surgical anatomy is presented. Describe the spectrum of unusual tumors of the cpa. Clinical features and outcomes in patients with nonacoustic. Distinguishing meningiomas from schwannomas in this location carries particular importance as each. Cerebellopontine angle cpa and cerebellomedullary fissure cmf tumors are rare in children and information is scarce in the literature. This retrospective study reports their histological distribution and tumor origin, and describes surgical resections and postoperative outcome based upon the authors consecutive personal series. Their frequency among primitive intracranial tumors is about 1% and they account for 40% of all intracranial epidermoid of the cerebellopontine angle cpa.
Synchronous tumors of the cerebellopontine angle mayo clinic. Parthasarathy3, ramya kalaiarasan4, rajkumar g5, farooque m. We present a case of cerebellopontine cp angle ganglioglioma in a young child with developmental delay and no trigeminal nerve symptoms. Pdf on may 31, 2020, sunil rauniyar and others published cerebellopontine angle tumors. These features were studied in patients presenting to the regional neurootological unit. Cerebellopontine angle mass radiology reference article. Any information contained in this pdf file is automatically generated from digital. Cerebellopontine angle cpa tumours can be divided into extraaxial. A general management scheme is proposed for all three that includes surveillance imaging and clinical followup. We report a rare case of a primary malignant melanoma presenting as a cpa tumor. C6 1final year postgraduate, department of radiology, chettinad hospital and research institute, 2professor, department of.
The lipoma constitutes an important differential diagnosis because the clinical management differs significantly from other cpa lesions. Objectivesnonacoustic tumours of the cerebellopontine angle diver from ves tibular schwannomas in their prevalence, clinical features, operative. Pdf cerebellopontine angle cpa is a triangular space in the posterior cranial fossa that is bounded by the tentorium superiorly, brainstem. Cerebellopontine angle cpa tumors are the most common neoplasms in the posterior fossa, accounting for 1015% of intracranial tumors. The case of an extensive lipoma of the cerebellopontine angle cpa represents 0. Cerebellopontine angle cpa tumors are the most common neoplasms in the posterior. The authors present their experience of 14 patients with hydrocephalus found in a recent series of 104 consecutive cases of acoustic neuroma. Tumors can also invade the cpa by extension from the petrous bone or skull base cholesterol granuloma, paraganglioma, chondromatous tumors, chordoma, en. Surgical and nonsurgical outcomes in a cohort 321 cases find, read and cite all the research you need. C63 ieraia ura ceprar meiie surger a raigy volume 4 issue 3 julyseptember 2019 issn o ic diagnostic efficacy of mri in the evaluation of cerebellopontine angle tumors amritha a1, abubacker sulaiman f2, e. Tumors of the cerebellopontine angle cpa have always proven difficult for neurosurgeons to optimally manage. Vertebral angiography revealed alarge anterior inferior. Tumors occupying this region account for approximately 10% of all intracranial neoplasms, with vestibular schwannoma accounting for 80% of these cpa tumors. The relation between flocculus volume and tinnitus after.
Tumors of the cerebellopontine angle are the other relatively frequent cause of brainstem auditory dysfunction. Cerebellopontine angle tumors posterior fossa meningioma epidermoid. Most cpa tumors are benign, with 8094% being vestibular schwannomas acoustic neuromas, lipomas, vascular malformations, and. After the treatment of cerebellopontine angle cpa tumors, there is a risk of worsening an existing hydrocephalus hcp or developing a new hydrocephalus.
Cerebellopontine angle tumor neoplasm of cerebellopontine angle. At present, surgical treatment is the first choice for large cerebellopontine angle meningiomas, while small. Both types of hcp have been usually managed by an external ventricular drain or shunt. Malignant tumors of the cerebellopontine angle cpa are also extremely unusual, accounting for fewer than 1% of tumors at this location. The term pericyte is also known as vascular smooth muscle cells or mural cells. They constitute the most frequently diagnosed tumors of the posterior fossa and account for up to 10% of all intracranial neoplasms. The most common histological types are vestibular schwannoma, meningioma and squamous cyst. Gamma knife radiosurgery for cerebellopontine angle.
Cerebellopontine angle is a common site for multiple pathologies, and cpa tumors are among the most common intracranial tumor accounting for 5 to 10% vilela, 2005. Umors of the cpa make up 5 to 10% of all intracranial tumors. Although a majority of tumors in the cerebellopontine angle cpa are vestibular schwannomas vs, other masses can also be seen in the region and differentiation of various cpa tumors, particularly meningiomas can be difficult on imaging alone. Cerebellopontine angle tumor neoplasm of cerebellopontine. Cerebellopontine angle tumors in infants and children. Thus there may occur cysts, gliomas, ependymomas, aneurysms, osteomas, meningiomas, and cholesteatomas. The cerebellopontine angle is also the site of a set of neurological disorders. Most cpa tumors are benign, with 8094% being vestibular schwannomas acoustic neuromas, lipomas, vascular malformations, and hemangiomas. Conclusionwe suggest that malignant primary tumors of the central nervous system, especially those located at the cerebellopontine angle, in pediatric patients should be diagnosed with noninvasive methods such as cytogenetic analysis by stereotaxic sampling of tumoral tissue, or via very limited surgery due to the deleterious effects of extensive surgery on the morbidity of patients with an ini 1. Cerebellopontine angle tumor in a patient with a maternally inherited sdhd gene mutation larry d. Cerebellopontine angle and retrosigmoid approach cerebellopontine angle. The results obtained by using the mf approach were superior for intracanalicular tumors p0. Indications include unilateral hearing loss 85%, speech impediments, disequilibrium, tremors or other loss of motor control.
Propose a diagnosis based on suggestive features for almost every tumor. Synchronous tumors of the cerebellopontine angle cpa are very rare and inconsistently described. The cerebellopontine angle is located between the superior and inferior limbs of the cerebellopontine fissure, an angular cleft formed by the petrosal cerebellar surface folding around the pons and middle cerebellar peduncle. Most cases of tumor related hfs are treated satisfactorily by open resection of the tumor alone or combined with vascular decompression. Recognize the ct and mr imaging features of these tumors.
Tumors of the cerebellopontine angle cpa represent about 10% of intracranial tumors in adults and about 1% in the pediatric series. Gamma knife surgery for hemifacial spasm related to. Treatment options may vary based on specific pathology of the cpa tumor. Cerebellopontine angle cpa tumours, are diverse pathologically with regard to the site of tumour origin and displacement of the.
Many different types of tumor may arise in the cerebellopontine angle. The cerebellopontine angle cistern is a subarachnoid cistern formed by the cerebellopontine angle that. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Cerebellopontine angle cpa tumors are masses located in the region between the cerebellum and pons.
The cerebellopontine angle cpa is the most common location of posterior fossa tumors. The clinical treatment and outcome of cerebellopontine. This study is evaluating surgical management and clinical outcome of cpa meningiomas. Although a diverse range of pathologies may be seen in this region, the most common by far is a vestibular schwannoma. Radiosurgery for these tumors has rarely been reported. There is growing evidence that complete removal of cerebellopontine angle tumors will result in resolution of hydrocephalus without requiring other methods of csf decompression. Acoustic neuromas vestibular schwannomas, arising from the neurilemmal junction of the vestibular nerve, account for between 80%90% of these tumours. Historadiological correlation of cerebellopontine angle tumors.
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