Left Cerebellopontine Angle Research Articles

Isolated Hypoglossal Nerve Schwannoma: An Uncommon Presentation of Schwannoma.

Isolated Hypoglossal Nerve Schwannoma: An Uncommon Presentation of Schwannoma.

Metastatic extrapulmonary small cell carcinoma to the cerebellopontine angle: a case report and review of the literature.

Extrapulmonary small cell carcinomas (EPSCC) are rare malignancies with poor patient prognoses. We present the case of a 63-year-old male who underwent surgical resection of a poorly differentiated small cell carcinoma, likely from a small intestinal primary tumor that metastasized to the cerebellopontine angle (CPA). A 63-year-old male presented with mild left facial paralysis, hearing loss, and balance instability. MRI revealed a 15 mm mass in the left CPA involving the internal auditory canal consistent with a vestibular schwannoma. Preoperative MRI eight weeks later demonstrated marked enlargement to 35 mm. The patient underwent a suboccipital craniectomy and the mass was grossly different visually and in consistency from a standard vestibular schwannoma. The final pathology revealed a poorly differentiated small cell carcinoma. Postoperative PET scan identified avid uptake in the small intestine suggestive of either a small intestinal primary tumor or additional metastatic disease. The patient underwent whole brain radiation therapy and chemotherapy and at last follow-up demonstrated improvement in his symptoms. Surgical resection and radiotherapy are potential treatment options to improve survival in patients diagnosed with NET brain metastases. We present the first documented case of skull base metastasis of a poorly differentiated small cell carcinoma involving the CPA.

ED-01 * MEDULLOBLASTOMA PRESENTING AS AN INTRACRANIAL HEMORRHAGE

CASE REPORT: Children with medulloblastoma usually present with ataxia, nystagmus, dysmetria, intention tremor, cranial nerve deficits or signs of elevated intra-cranial pressure. On MRI a midline non-homogenously enhancing lesion is seen often with associated brainstem compression, ventricular dilatation and transependymal CSF migration. We describe a 12 year old child who presented with dizziness, emesis, neck pain and occipital headache resulting from minor head trauma on the preceding day. Past history included recent onset intermittent diplopia corrected with lenses. Neurological examination was normal except for mild left hearing loss and decreased visual acuity. CT scan at admission revealed an acute subarachnoid hemorrhage in the left cerebellopontine angle (CPA) extending through the left foramina of Luschka and Magendie and compressing the adjacent cerebellum and brainstem. MRI /MRA/Cerebral angiogram did not uncover any underlying pathology. After observation for 3 days he was discharged with residual mild left hearing loss and left horizontal nystagmus. On follow up four months later he was found to have lost all hearing (left) and had difficulty with balance. MRI showed a 2.8 x 2.6 x 2.4 cm left CPA mass hypo-intense on T1 with hyperintense foci on T2. The mass extended into the internal auditory canal and compressed the brainstem without causing hydrocephalus. A peri-lesional hemorrhagic stain was observed. On repeat review of an MRI performed 2 months earlier the mass was noted to be present measuring 1.5 x 1.1 x 1.2 cm. Spinal cord MRI was negative. Gross total resection of the mass was complicated by left facial nerve palsy and severe obstructive hydrocephalus requiring temporary EVD placement. The patient is on treatment for medulloblastoma (classic histology/WNT subgroup).Hearing loss persists but his 7th CN palsy is improving. We seek to highlight that on MRI an acute intracranial hemorrhage can camouflage a neoplastic process.

Bilateral Optic Nerve Head Angiomas and Retrobulbar Haemangioblastomas in von Hippel-Lindau Disease

A 39-year-old man presented with progressive visual loss in both eyes. His past medical history was remarkable for biopsy-proven bilateral adrenal phaeochromocytomas. Ophthalmoscopy revealed an optic nerve head angioma and macular lipid exudates in the right eye and a regressed optic nerve head angioma in the left eye. A fluorescein angiogram revealed a lobulated pattern of leakage and venous staining from the optic nerve head angioma. Brain and orbital magnetic resonance imaging showed haemangioblastomas of the right intraorbital and left paraclinoid optic nerves and in the left cerebellopontine angle area. These findings were consistent with a clinical diagnosis of von Hippel-Lindau disease.

Primary cerebello-pontine angle melanoma: a case report

Intra-cranial melanomas are commonly metastatic from primary melanoma elsewhere in the body. The primary occurrence of a melanoma in the brain parenchyma is rare. We report a case of 38 year aged woman who presented with deviation of left eye and headache. On imaging, a space occupying lesion was found in the left Cerebello-pontine angle and a preoperative diagnosis of meningioma was made. She underwent left retro-mastoid sub-occipital craniectomy and excision of a black vascular tumor. Histopathological examination revealed a melanoma which was confirmed with Immunohistochemical assay. Search for dermal, mucous and ocular lesions were negative. She received adjuvant radiation to the post- operative tumor bed to 5400cGy in 30 fractions using Intensity Modulated Radiation Therapy technique along with concurrent Temozolomide. She tolerated the treatment well and is symptom free 12 months after treatment. Primary Melanomas are rare lesions of the Cerebello-pontine angle and its radiological features, evaluation and management have been discussed. ------------------- Cite this article as : Ponni A, Jagannatha A, Gururajachar J, Harjani R, Koushik K, Subramanian N, Sowmya R, Varma R. Primary cerebello-pontine angle melanoma: a case report. Int J Cancer Ther Onco l 2014; 2 (3):020315. DOI : 10.14319/ijcto.0203.15

Neuronavigation assisted decompression of trigeminal neuralgia caused by cerebellopontine angle osteoma

Abstract Trigeminal neuralgia (TN) is usually caused by vascular compression of the trigeminal nerve root entry zone, but can be caused by other factors such as tumors, vascular disorders, and demyelination in multiple sclerosis. We present a rare case with a huge osteoma located on the cerebellopontine angle (CPA) and causing TN. A 48-year-old woman presented with TN caused by a huge left CPA bone tumor. Surgery was performed by the lateral suboccipital approach under neuronavigation system guidance and regional decompression of the trigeminal nerve root entry zone was achieved. Intraoperative, neuro-navigation system was very useful for identification of the trigeminal nerve entry zone because normal anatomy had been obscured by the huge osteoma. Her pain disappeared completely immediately after surgery. The histological diagnosis was osteoma. TN is an expectative symptom of CPA tumors. Osteomas should be considered in patients with CPA tumors.

Toothache Caused by Trigeminal Neuralgia Secondary to Vestibular Schwannoma: A Case Report

IntroductionVestibular schwannoma, also called acoustic neuroma, is a tumor composed of Schwann cells that most commonly involves the vestibular division of the 8th cranial nerve. A thorough medical and dental history and properly conducted diagnostic testing and radiographic evaluation are essential in differentiating odontogenic tooth pain from tooth pain of a nonodontogenic origin. MethodsThis report discusses a case of acoustic neuroma mimicking odontogenic pain. A 61-year-old man presented with pain in the lower left quadrant. Medical history revealed peripheral neuropathy and prior exposure to Agent Orange. Diagnostic testing on teeth #19, #20, and #21 ruled out an odontogenic cause for the pain. Brushing of a specific region of the face elicited pain that resembled the patient's chief concern. The patient was referred to a craniofacial pain center with the tentative diagnosis of trigeminal neuralgia. Treatment for trigeminal neuralgia was not successful, prompting referral to the neurosurgery department where magnetic resonance imaging was performed. ResultsMagnetic resonance imaging revealed a moderate-size, lobular, left cerebellopontine angle mass lesion consistent with acoustic neuroma with compression of the left trigeminal nerve secondary to the lesion. ConclusionsAcoustic neuroma can cause tooth pain secondary to compression of the trigeminal nerve.

Angiosarcoma arising in schwannoma of cerebellopontine angle and later associating with meningioma in a patient with neurofibromatosis type 2

Here, we describe an extremely rare case of angiosarcoma arising in schwannoma of the cerebellopontine angle and later associating with meningioma in a patient with neurofibromatosis type 2. A 33-year-old disabled Japanese man with right drop foot after surgery for an unspecified tumor demonstrated multiple tumors, suspected to be schwannoma, in the bilateral cerebellopontine angles, the cervical and lumbar spinal cord, and on the right nuchal skin. Also present were several tumors in the medulla and thoracic spinal cord suspected to be ependymoma or astrocytoma. The patient was diagnosed with neurofibromatosis type 2 according to the diagnostic criteria by the U.S. National Institutes of Health. The bilateral tumors in the cerebellopontine angle were resected to reduce symptoms and brain stem compression. Histopathological analysis revealed angiosarcoma arising in schwannoma of the bilateral tumors, and angiosarcoma was proportionally larger in the right tumor than in the left. At age 36, the patient underwent a second resection of the regrown tumor in the left cerebellopontine angle, and histopathology demonstrated mixed angiosarcoma and meningioma. That angiosarcoma arises in schwannoma is a pathogenesis within the realm of conjecture, especially that the phenomenon of mixed meningioma and angiosarcoma has not been reported to date.

Large cerebello-pontine epidermoid tumor in a child

Abstract Cerebellopontine angle lesions are more commonly found in adults; however, they are uncommon in children. 13 year female child presented with progressive cerebellar signs and decreased hearing in left ear. Magnetic resonance imaging was suggestive of left cerebello-pontine angle epidermoid tumor that was treated surgically. In this rare case we discuss the imaging findings and review the relevant literature.

Solitary Metastasis to the Facial/Vestibulocochlear Nerve Complex Case Report and Review of the Literature

Background and Importance: Distant metastasis of mucinous adenocarcinoma from the gastrointestinal tract, ovaries, pancreas, lungs, breast or urogenital system is a well described entity. Mucinous adenocarcinomas from different primary sites are histologically identical with gland cells producing a copious amount of mucin. This report describes a very rare solitary metastasis of a mucinous adenocarcinoma of unknown origin to the facial/ vestibulocochlear nerve complex in the cerebellopontine angle. Clinical Presentation: A 71-year-old woman presented with several month history of progressive neurologic decline and a negative extensive workup performed elsewhere. She presented to our institution with complete left facial weakness, left-sided deafness, gait unsteadiness, headache and anorexia. A repeat MRI scan of the head revealed a cystic, enhancing abnormality involving the left cerebellopontine angle and internal auditory canal. A left retrosigmoid craniotomy was performed and the lesion was completely resected. The final pathology was a mucinous adenocarcinoma of indeterminate origin. Postoperatively, the patient continued with her preoperative deficits and subsequently died of her systemic disease six weeks after discharge. Conclusion: The facial/vestibulocochlear nerve complex is an unusual location for metastatic disease in the central nervous system. Clinicians should consider metastatic tumor as the possible etiology of an unusual appearing mass in this location causing profound neurologic deficits. The prognosis following metastatic mucinous adenocarcinoma to the cranial nerves in the cerebellopontine angle may be poo

Primary intracranial ectopic craniopharyngioma in a patient with probable Gardner's syndrome

The authors describe a patient with an adamantinomatous craniopharyngioma (CPG) arising in the cerebellopontine angle (CPA), who also had probable Gardner's syndrome. This 31-year-old man presented with headache and dizziness. Brain CT and MRI showed a 5 × 4-cm lesion with multiple small calcifications in the left CPA. The patient underwent suboccipital craniotomy with tumor removal. Histopathological findings indicated an adamantinomatous CPG. This patient also showed characteristics of Gardner's syndrome. Although this syndrome is associated with intracranial neoplasms, it is unclear whether patients with both Gardner's syndrome and CPG are part of the heterogeneity of Gardner's syndrome.

THINK OUTSIDE THE BOX, COLLAPSE THE BOX, AND TAKE A SHARP KNIFE TO IT!

A 74 year–old woman presented in December 2011 with left sided cerebellar symptoms. She was initially diagnosed as having a posterior circulation infarction and discharged from hospital on appropriate medication....

Mature Teratoma of the Petrous Bone with Extension into the Cerebellopontine Angle: Case Report

Purpose Intracranial teratomas in children involving lateral structures such as the petrous portion of the temporal bone are very uncommon. The authors report a case of a petrous teratoma with significant extension into the cerebellopontine angle with brainstem compression. Case Report An 11-year-old girl presented left-sided facial weakness. Computed tomography (CT) demonstrated a multiloculated lesion expanding the labyrinthine structures in the left petrous temporal bone including the vestibule, semicircular canals, and cochlea, with extension to the left cerebellopontine angle via the expanded left internal auditory canal. The tumor was resected via a transtemporal approach with no evidence of recurrence at nearly 2 years. Conclusion Complete resection should be the primary treatment for these tumors to minimize the risk of recurrence. To the authors' knowledge, this is the first case report of a mature teratoma originating in the petrous bone with extension into the cerebellopontine angle.

Myoepithelioma of the cerebellopontine angle: a previously not documented benign salivary gland-type neoplasm within the cranium

Myoepithelioma is a dimorphic neoplasm with contractile-epithelial phenotype, originally interpreted as deriving from, but not actually restricted to the salivary glands. As a novel addition to the list of exquisitely rare intracranial salivary gland-type tumors and tumor-like lesions, we report on an example of myoepithelioma encountered in the left cerebellopontine angle of a 32-year-old male. Clinically presenting with ataxia and dizziness, this extraaxial mass of 4 × 3.5 × 3 cm was surgically resected, and the patient is alive 6 years postoperatively. Histologically, the tumor exhibited a continuum ranging from compact fascicles of spindle cells to epithelial nests and trabeculae partitioned by hyalinized septa, while lacking tubular differentiation. Regardless of architectural variations, there was robust immunoexpression of S100 protein, smooth muscle actin, GFAP, cytokeratin, and vimentin. Cytologic atypia tended to be modest throughout, and the MIB1 labeling index averaged less than 1%. Fluorescent in situ hybridization indicated no rearrangement of the EWSR1 locus. We interpret these results to suggest that myoepithelioma of the posterior fossa - along with related salivary epithelial tumors in this ostensibly incongruous locale - may possibly represent analogous neoplasms to their orthotopic counterparts, ones arising within aberrant salivary anlagen. The presence of the latter lends itself to being mechanistically accounted for by either postulating placodal remnants in the wake of branchial arch development, or linking them to exocrine glandular nests within endodermal cysts. Alternatively, myoepithelioma at this site could be regarded as a non tissue-specific lesion similar to its relatives ubiquitously occurring in the soft parts.

Obstructive hydrocephalus due to CNS toxocariasis

A 46-year-old man developed intermittent headache, diplopia, and visual obscuration for two months. Funduscopic examination showed optic disk swelling in both eyes. Brain MRI exhibited hydrocephalus and leptomeningeal enhancement at the prepontine cistern, left cerebellopontine angle cistern and bilateral cerebral hemisphere, and hemosiderin deposition along the cerebellar folia. CSF analysis revealed an elevated opening pressure with xanthochromic appearance and small amount of red blood cells. Antibody titer against Toxocariasis using ELISA was elevated both in blood and CSF. Obstructive hydrocephalus and hemosiderin deposition in this case may result from the active inflammatory process due to CNS toxocariasis within the subarachnoid space.

A rare case of an extra.axial cavernous angioma in the cerebellopontine angle

Intracranial extra-axial cavernous angiomas are rare lesions. We report a rare case of extra-axial cavernous angioma in the cerebellopontine angle (CPA) in a 50-year-old male, who presented with lower cranial nerve palsy and gait ataxia. Computed tomography (CT) scan of the brain showed a hyperdense lesion in the left cerebellopontine angle. The lesion was totally excised by the retrosigmoid approach and a pathological examination confirmed the lesion to be a cavernous angioma. Following surgery, the lower cranial nerve palsy recovered significantly.

A rare presentation of intracranial cysticercosis involving the cerebellopontine angle

Intracranial cysticercosis involving the cerebellopontine angle (CPA) is rare. Here we report a patient with intracranial cysticercosis in the left CPA; the initial diagnosis of the lesion was epidermoid cyst or cystic glioma. The patient subsequently underwent total lesion removal through a left suboccipital approach; however, postoperative histopathological examination showed the tumor to be an intracranial cysticercosis. The patient’s symptoms improved dramatically after microsurgery and praziquantel administration. Rare infection caused by cysticercosis involving the CPA should be highlighted in the differential diagnosis of intracranial space-occupying lesions.

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome secondary to an epidermoid tumor in the cerebellopontine angle

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is classified under trigeminal autonomic cephalalgias. This rare headache syndrome is infrequently associated with secondary pathologies. In this paper the authors report on a patient with paroxysmal left retroorbital pain with associated autonomic symptoms of ipsilateral conjunctival injection and lacrimation, suggestive of SUNCT syndrome. After failed medical treatment an MRI sequence was obtained in this patient, demonstrating an epidermoid tumor in the left cerebellopontine angle. The patient's symptoms completely resolved after a gross-total resection of the tumor. This case demonstrates the effectiveness of resection as definitive treatment for SUNCT syndrome associated with tumoral compression of the trigeminal nerve. Early MRI studies should be considered in all patients with SUNCT, especially those with atypical signs and symptoms.

Neurosurgical management of neurenteric cysts in children

Neurenteric cysts (NCs) are endothelium-lined structures of presumed endodermal origin. There have been few pediatric series of intracranial NCs reported previously. The authors present their experience in the management of these lesions. A retrospective chart review of all cases of NCs identified between 1977 and 2007 was carried out. Demographics, details of clinical presentation, surgical therapy, and outcome data were extracted. Eleven cases were identified, involving 6 girls and 5 boys. The patients' average age was 4.6 years (range 1 day-14 years). Limb weakness was the most common presenting symptom. The location of the cysts was cervical in 2 cases, cervicothoracic in 4 cases, and thoracic in 3 cases. One cyst was anterior to the pons with extension to the left cerebellopontine angle and 1 cyst was at the craniocervical junction. Five patients had an apparently complete initial excision, with 1 recurrence. Four patients were initially treated with incomplete excision and/or cyst drainage, usually into an Ommaya reservoir. A neonate with a large cervicothoracic cyst died of multiple congenital anomalies without any intervention. All other patients were alive at last follow-up. One patient with a radiologically identified presumed cyst, which remained stable, was simply followed up. Several patients required multiple procedures and 1 patient developed hydrocephalus after aseptic meningitis and was treated with CSF shunting. Neurenteric cysts are rare in the pediatric population. Total resection, if possible, provides the best long-term outcome. Incomplete excision may lead to multiple procedures, including cyst drainage, but can lead to long-term stability.

Cerebellar Hemorrhage Secondary to Cerebellopontine Angle Metastasis From Thyroid Papillary Carcinoma

A 63-year-old woman presented with a metastatic focus of papillary thyroid carcinoma in the cerebellopontine angle manifesting as lateral gazing nystagmus and slurred speech. Computed tomography demonstrated massive hemorrhage in the left cerebellar hemisphere. She was treated conservatively. Her symptoms resolved completely, but she experienced progressive deterioration in auditory acuity and ataxia over the next 6 months. Magnetic resonance imaging with gadolinium demonstrated an enhanced mass in the left cerebellopontine angle, and she was scheduled for elective resection of the tumor. Left retrosigmoid craniotomy was performed, and the tumor was subtotally removed except for a small amount at the junction of the trigeminal nerve and the pons. Histological examination confirmed a diagnosis of metastatic papillary thyroid carcinoma. The patient then underwent adjuvant gamma knife radiosurgery. Her clinical course was unremarkable, and her hypoacusis and ataxia resolved completely. Postoperative gallium scintigraphy revealed no residual tumor and no other systemic metastases. Hemorrhagic cerebellar metastasis from papillary thyroid carcinoma is extremely rare, but early recognition of metastatic cerebellar tumor should prompt immediate treatment to avoid the development of hearing loss, ataxia, and tonsillar herniation.