Primary glioblastoma of the cerebellopontine angle in adults

Gliomas are rare entities in the cerebellopontine angle (CPA) in adults. The authors present clinical, neuroradiological, serological, and neuropathological findings in a 60-year-old man with an extraaxial CPA glioblastoma arising from the proximal portion of cranial nerve VIII. The patient presented with progressive left-sided deafness and left-sided facial palsy lasting less than 2 months and progressive dysarthria and dysphagia lasting 2 weeks. Preoperative neuroimaging suggested the diagnosis of CPA meningioma with “dural-tail” sign and involvement of the internal auditory canal. Serological examination showed an increase in the malignant markers of ferritin and neuron-specific enolase, which suggested underlying malignancy. The tumor was subtotally removed, and it was confirmed to be completely separated from the brainstem and cerebellum. Cranial nerves VII and VIII were destroyed and sacrificed. Transient severe bradycardia occurred during surgery due to entrapment of the caudal cranial nerve complex by the tumor in such an infiltrative way. The neuropathological examination revealed a glioblastoma. The patient underwent no further treatment and died of cachexia 2 months postoperatively. To the authors' knowledge, this represents the first case of a primary glioblastoma in the CPA in an adult. A high index of suspicion along with reliance on clinical assessment, radiological findings, and serum detection of specific malignant markers is essential to diagnose such uncommon CPA lesions.

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Glioblastoma of the cerebellopontine angle and internal auditory canal mimicking a peripheral nerve sheath tumor: case report

Journal of Neurosurgery ◽

10.3171/2018.8.jns181702 ◽

2019 ◽

Vol 131 (6) ◽

pp. 1835-1839 ◽

Cited By ~ 1

Author(s):

Hirokazu Takami ◽

Christoph M. Prummer ◽

Christopher S. Graffeo ◽

Maria Peris-Celda ◽

Caterina Giannini ◽

...

Keyword(s):

Peripheral Nerve ◽

Cerebellopontine Angle ◽

Frozen Section ◽

Cranial Nerves ◽

Internal Auditory Canal ◽

Molecular Testing ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath ◽

First Case

Glioblastoma (GBM) of the internal auditory canal (IAC) is exceedingly rare, with only 3 prior cases reported in the literature. The authors present the fourth case of cerebellopontine angle (CPA) and IAC GBM, and the first in which the lesion mimicked a vestibular schwannoma (VS) early in its natural history. A 55-year-old man presented with tinnitus, hearing loss, and imbalance. MRI identified a left IAC/CPA lesion measuring 8 mm, most consistent with a benign VS. Over the subsequent 4 months he developed facial weakness. The tumor grew remarkably to 24 mm and surgery was recommended; the main preoperative diagnosis was malignant peripheral nerve sheath tumor (MPNST). Resection proceeded via a translabyrinthine approach with resection of cranial nerves VII and VIII, followed by facial-hypoglossal nerve anastomosis. Intraoperative frozen section suggested malignant spindle cell neoplasm, but final histopathological and molecular testing confirmed the lesion to be a GBM. The authors report the first case in which absence of any brainstem interface effectively excluded a primary parenchymal tumor, in particular GBM, from the differential diagnosis. Given the dramatic differences in treatment and prognoses between malignant glioma and MPNST, this case emphasizes the importance of surgical intervention on an aggressively growing lesion, which provides both the best probability of local control and the critical tissue diagnosis.

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Trigeminal Nerve Schwannoma of the Cerebellopontine Angle

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0038-1669966 ◽

2018 ◽

Vol 79 (S 05) ◽

pp. S389-S390

Author(s):

Maria Peris-Celda ◽

Christopher Graffeo ◽

Avital Perry ◽

Lucas Carlstrom ◽

Michael Link

Keyword(s):

Trigeminal Nerve ◽

Cerebellopontine Angle ◽

Incidental Finding ◽

Cranial Nerves ◽

Internal Auditory Canal ◽

Clinical History ◽

Sensory Loss ◽

Meckel’S Cave ◽

Neurologic Deficits ◽

Meckel's Cave

Introduction Large and even moderate sized, extra-axial cerebellopontine angle (CPA) tumors may fill this restricted space and distort the regional anatomy. It may be difficult to determine even with high resolution magnetic resonance imaging (MRI) if the tumor is dural-based, or what the nerve of origin is if a schwannoma. While clinical history and exam are helpful, they are not unequivocal, particularly since many patients present with a myriad of symptoms, or conversely an incidental finding. We present an atypical appearing, asymptomatic CPA tumor, ultimately identified at surgery to be a trigeminal schwannoma. Case History A 40-year-old man presented with new-onset seizure. MRI identified an incidental heterogeneously contrast-enhancing CPA lesion (Fig. 1A–D). The tumor was centered on the internal auditory canal (IAC) with no tumor extension into Meckel's cave, IAC or jugular foramen. Audiometry demonstrated 10db of relative left-sided hearing loss with 100% word recognition. Physical examination was negative for focal neurologic deficits. A retrosigmoid craniotomy was performed and an extra-axial, yellow-hued mass was encountered and resected, which was ultimately confirmed to originate from the trigeminal nerve (Video 1). Gross total resection was achieved, and the patient recovered from surgery with partial ipsilateral trigeminal sensory loss and no other new neurologic deficits. Conclusion Pure CPA trigeminal schwannomas are rare, but should be considered in the differential for enhancing CPA lesions. Although, Meckel's cave involvement is frequently observed, it is not universal, and pure CPA schwannomas of all cranial nerves IV–XII have been reported in the literature.The link to the video can be found at: https://youtu.be/AlodYCu70F8.

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Intratumoral Bleeding in a Vestibular Schwannoma

Arquivos Brasileiros de Neurocirurgia Brazilian Neurosurgery ◽

10.1055/s-0037-1615287 ◽

2017 ◽

Vol 37 (01) ◽

pp. 47-49

Author(s):

Emanuelle Braga ◽

Luiza Köhler ◽

Marcelo de Cesaro ◽

Tasso Barreto ◽

Richard Giacomelli ◽

...

Keyword(s):

Hearing Loss ◽

Case Report ◽

Cranial Nerve ◽

Cerebellopontine Angle ◽

Clinical Presentation ◽

Cranial Nerves ◽

Altered Consciousness ◽

Progressive Hearing Loss ◽

Asymptomatic Patients ◽

Intratumoral Bleeding

AbstractVestibular schwannomas (VSs) account for ∼ 70% of all tumors of the cerebellopontine angle (CPA). Their clinical presentation is often insidious, with progressive hearing loss and involvement of other cranial nerves. Spontaneous hemorrhage in those tumors is very unusual, and generally presents with acute clinical features such as nausea, vomiting, headache and altered consciousness, usually with marked dysfunction of the cranial nerve involved, and with new deficits of neighboring cranial nerves. Asymptomatic patients are extremely rare. We present a case report of an incidental VS with asymptomatic bleeding, which evolved to death after surgery.

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SLE pachymeningitis and multiple cranial nerve palsies: a case report and review of the literature

Lupus ◽

10.1177/0961203319862867 ◽

2019 ◽

Vol 28 (9) ◽

pp. 1154-1157 ◽

Cited By ~ 1

Author(s):

T J John ◽

K John ◽

L du Plessis ◽

M Manie

Keyword(s):

Lupus Erythematosus ◽

Cranial Nerve ◽

Neurological Dysfunction ◽

Hypertrophic Pachymeningitis ◽

First Case ◽

Cranial Nerve Palsies ◽

Uncommon Disease ◽

High Index Of Suspicion ◽

Multiple Cranial Nerve

Hypertrophic pachymeningitis (HP) is a relatively uncommon disease associated with focal or diffuse thickening of the dura mater secondary to underlying chronic inflammation. The link between systemic lupus erythematosus (SLE) and hypertrophic pachymeningitis (HP) is extremely rare, with only six other cases reported in the literature. We, however, report the first case of SLE pachymeningitis presenting with multiple cranial nerve palsies. The patient showed good response to steroids and cyclophosphamide therapy. One should maintain a high index of suspicion to make the diagnosis in patients with SLE presenting with neurological dysfunction. Prompt therapy prevents long-term neurological sequelae.

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Continuous electromyography monitoring of motor cranial nerves during cerebellopontine angle surgery

Journal of Neurosurgery ◽

10.3171/jns.2000.93.4.0586 ◽

2000 ◽

Vol 93 (4) ◽

pp. 586-593 ◽

Cited By ~ 156

Author(s):

Johann Romstöck ◽

Christian Strauss ◽

Rudolf Fahlbusch

Keyword(s):

Detailed Analysis ◽

Cranial Nerve ◽

Cerebellopontine Angle ◽

Operative Procedure ◽

Cranial Nerves ◽

Postoperative Outcome ◽

Lower Cranial Nerve ◽

Content Type ◽

Lower Cranial Nerves ◽

Fine Print

Object. Electromyography (EMG) monitoring is expected to reduce the incidence of motor cranial nerve deficits in cerebellopontine angle surgery. The aim of this study was to provide a detailed analysis of intraoperative EMG phenomena with respect to their surgical significance.Methods. Using a system that continuously records facial and lower cranial nerve EMG signals during the entire operative procedure, the authors examined 30 patients undergoing surgery on acoustic neuroma (24 patients) or meningioma (six patients). Free-running EMG signals were recorded from muscles targeted by the facial, trigeminal, and lower cranial nerves, and were analyzed off-line with respect to waveform characteristics, frequencies, and amplitudes. Intraoperative measurements were correlated with typical surgical maneuvers and postoperative outcomes.Characteristic EMG discharges were obtained: spikes and bursts were recorded immediately following the direct manipulation of a dissecting instrument near the cranial nerve, but also during periods when the nerve had not yet been exposed. Bursts could be precisely attributed to contact activity. Three distinct types of trains were identified: A, B, and C trains. Whereas B and C trains are irrelevant with respect to postoperative outcome, the A train—a sinusoidal, symmetrical sequence of high-frequency and low-amplitude signals—was observed in 19 patients and could be well correlated with additional postoperative facial nerve paresis (in 18 patients).Conclusions. It could be demonstrated that the occurrence of A trains is a highly reliable predictor for postoperative facial palsy. Although some degree of functional worsening is to be expected postoperatively, there is a good chance of avoiding major deficits by warning the surgeon early. Continuous EMG monitoring is superior to electrical nerve stimulation or acoustic loudspeaker monitoring alone. The detailed analysis of EMG-waveform characteristics is able to provide more accurate warning criteria during surgery.

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BILATERAL OSTEOMAS ARISING FROM THE INTERNAL AUDITORY CANAL

Neurosurgery ◽

10.1227/01.neu.0000316023.81786.b6 ◽

2008 ◽

Vol 62 (2) ◽

pp. E528-E529 ◽

Cited By ~ 15

Author(s):

Venelin M. Gerganov ◽

Amir Samii ◽

Vincenzo Paterno ◽

Alexandru C. Stan ◽

Madjid Samii

Keyword(s):

Cerebellopontine Angle ◽

Management Strategy ◽

Clinical Presentation ◽

Internal Auditory Canal ◽

Brain Magnetic Resonance Imaging ◽

Surgical Removal ◽

Vestibulocochlear Nerve ◽

Computed Tomographic ◽

First Case ◽

Symptomatic Side

Abstract OBJECTIVE Osteomas arising from the internal auditory canal and developing in the cerebellopontine angle have rarely been reported. We present the first case of bilateral osteomas in this region and describe our management strategy. CLINICAL PRESENTATION A 30-year-old woman presented with strong vertigo, tinnitus, and hypacusis on the left side. Brain magnetic resonance imaging and computed tomographic scans displayed bilateral cerebellopontine angle osteomas arising from the internal auditory canal. The larger tumor on the left side was found to be compressing the vestibulocochlear nerve. INTERVENTION Surgery was performed on the symptomatic side via the retrosigmoid approach, and the tumor was removed completely. The vertigo resolved completely after surgery, and the patient had no further tinnitus attacks. An audiogram showed slightly improved hearing with a mean of 20 dB in the main speech area. CONCLUSION Osteomas should be considered in patients with bilateral cerebellopontine angle tumors. Surgical removal might provide resolution of symptoms.

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Transapical extension in difficult cerebellopontine angle tumours: preliminary report

The Journal of Laryngology & Otology ◽

10.1258/002221503770716214 ◽

2003 ◽

Vol 117 (10) ◽

pp. 788-792 ◽

Cited By ~ 11

Author(s):

Mario Sanna ◽

Manoj Agarwal ◽

Yogesh Jain ◽

Alessandra Russo ◽

Abdel Kader Taibah

Keyword(s):

Vestibular Schwannoma ◽

Cerebellopontine Angle ◽

Cranial Nerves ◽

Internal Auditory Canal ◽

Petrous Bone ◽

Vestibular Schwannomas ◽

Type I ◽

Posterior Surface ◽

Prepontine Cistern ◽

Anterior Extension

Difficult cerebellopontine angle (CPA) tumours namely large/giant vestibular schwannomas, vestibular schwannomas with a significant anterior extension and meningiomas of the posterior surface of the petrous bone extending anterior to the internal auditory canal (IAC) have always posed a problem for the otoneurosurgeon. Modifications of the enlarged translabyrinthine approach (ETLA) specifically aimed at dealing with these tumours are not reported. The aim of this paper is to introduce the transapical extension of ETLA which involves increased circumferential drilling around the IAC beyond 270°C. The extension allows enhanced surgical control over the tumour as well as the anterior aspect of the CPA including the prepontine cistern, the Vth and VIth cranial nerves. The extension is further classified into Type I and II depending upon the extent of drilling. Type I extension entails drilling around the IAC for 300–320° and is indicated for large/giant vestibular schwannomas (large vestibular schwannoma extrameatal diameter 3–3.9 cm, giant vestibular schwannoma extrameatal diameter [ges ]4 cm) and vestibular schwannomas with significant anterior extension. Type II extension involves complete drilling around the canal for 360° and is indicated for meningiomas of the posterior surface of the petrous bone extending anterior to the IAC.

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Microsurgical Resection of the Epidermoid Tumor in the Cerebellopontine Angle

Journal of Neurological Surgery Part B Skull Base ◽

10.1055/s-0038-1677499 ◽

2019 ◽

Vol 80 (S 03) ◽

pp. S327-S328

Author(s):

Mirza Pojskić ◽

Kenan I. Arnautović

Keyword(s):

Brain Stem ◽

Aseptic Meningitis ◽

Cranial Nerve ◽

Cerebellopontine Angle ◽

Cranial Nerves ◽

Senior Author ◽

Epidermoid Tumor ◽

Postoperative Mri ◽

Suprasellar Region ◽

Microsurgical Resection

In this video, we demonstrate epidermoid tumor microsurgical resection of the cerebellopontine angle (CPA) performed by the senior author (K.I.A.). Epidermoid tumors arise from ectoderm trapped within/displaced into the central nervous system. They show predilection for CPA Angle (up to 40%), 4th ventricle, suprasellar region, and spinal cord.1 They are the 3rd most common CPA tumor, comprising approximately 7% of CPA pathology. CPA lesions can produce 5th and 7–12th cranial nerve neuropathies.2 3 4 Recurrent episodes of aseptic meningitis caused by cyst content rupture may occur. Symptoms include fever, meningeal irritation, and hydrocephalus. A 26-year-old female presented with headaches. Head magnetic resonance imaging (MRI) revealed right CPA tumor with brain stem compression (Fig. 1, A–C). There was evidence of restricted diffusion in diffusion-weighted imaging, typical of epidermoid tumor. Surgery was performed in prone position with head turned 25 degrees to the ipsilateral side using retrosigmoid craniotomy.5 Tumor was ventral to the 7th and 8th cranial nerve complexes, between the 5th nerve as well as toward the brainstem. The surgical plan was gross total resection with tumor capsule resection to prevent recurrence.6 (Small residuals can be left behind when capsule is adherent to critical structures.) Tumor was adherent to brain stem perforators which were preserved using meticulous dissection. Cranial nerves and vascular structures were also left intact. We irrigated with antibiotic saline and used perioperative treatment to prevent aseptic meningitis. The pathohistological diagnosis revealed epidermoid tumor cyst. Postoperative MRI revealed complete resection (Fig. 1, D–F). The patient recovered fully and was neurologically intact.The link to the video can be found at: https://youtu.be/LyWl-KZUSGY.

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Parésia do Nervo Motor Ocular Comum em Contexto de Sinusite Esfenoidal

Acta Médica Portuguesa ◽

10.20344/amp.5229 ◽

2014 ◽

Vol 27 (6) ◽

pp. 782 ◽

Cited By ~ 2

Author(s):

Luís Almeida Dores ◽

Marco Alveirinho Simão ◽

Marta Canas Marques ◽

Óscar Dias

Keyword(s):

Cranial Nerve ◽

Cranial Nerve Palsy ◽

Sphenoid Sinus ◽

Nerve Palsy ◽

Cranial Nerves ◽

Sinus Surgery ◽

Intravenous Antibiotics ◽

Motor Nerves ◽

Third Cranial Nerve Palsy ◽

High Index Of Suspicion

Sphenoid sinus disease is particular not only for its clinical presentation, as well as their complications. Although rare, these may present as cranial nerve deficits, so it is important to have a high index of suspicion and be familiar with its diagnosis and management. Symptoms are often nonspecific, but the most common are headache, changes in visual acuity and diplopia due to dysfunction of one or more ocular motor nerves. The authors report a case of a 59 years-old male, who was referred to the ENT emergency department with frontal headaches for one week which had progressively worsened and were associated, since the last 12 hours, with diplopia caused by left third cranial nerve palsy. Neurologic examination was normal aside from the left third cranial nerve palsy. Anterior and posterior rhinoscopy excluded the presence of nasal masses and purulent rhinorrhea. The CT scan revealed a soft tissue component and erosion of the roof of the left sphenoid sinus. Patient was admitted for intravenous antibiotics and steroids treatment without any benefit after 48 hours. He was submitted to endoscopic sinus surgery with resolution of the symptoms 10 days after surgery. The authors present this case for its rarity focusing on the importance of differential diagnosis in patients with headaches and cranial nerves palsies.<br /><strong>Keywords:</strong> Sphenoid Sinusitis; Oculomotor Nerve Diseases.

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Complete 3rd cranial nerve dysfunction postdeflation/ excision of an encasing pituitary macroadenoma intrasellular cyst: A Case Report

Romanian Neurosurgery ◽

10.1515/romneu-2016-0058 ◽

2016 ◽

Vol 30 (3) ◽

pp. 382-386

Author(s):

C. S. Ng ◽

S. Norlela

Keyword(s):

Cranial Nerve ◽

Cranial Nerves ◽

Fluid Pressure ◽

Residual Tumour ◽

Pituitary Macroadenoma ◽

Cystic Fluid ◽

First Case ◽

Cranial Nerve Dysfunction ◽

Cystic Component ◽

Cranial Nerve Palsies

Abstract Central nervous system injury in particular cranial nerve palsy has been reported to be as high as 2%. Such prevalence of palsy generally attributed to surgical manipulation at the cavernous sinus, especially incurring the abducens nerve. We report the first case of acute oculomotor nerve sequel to the release of cystic fluid wrapping the nerve following a transsphenoidal excision of pituitary macroadenoma in a 57-year-old woman. She attended with the presentation of acute excruciating headache associated with partial drooping of right eye. The computed tomography and magnetic resonance imaging (MRI) were consistent with pituitary apoplexy of an underlying pituitary macroadenoma. Urgent transsphenoidal hypophysectomy was done. Intra-operatively, cystic fluid was aspirated during pituitary tumour dissection. At the same time, curettage was employed to removal residual tumour after the tumour biopsy. Immediate post-operative assessment noted complete right eye ptosis, with clinical evidence of complete right third and fourth nerve palsies. MRI was repeated a week later in view of such palsy non-resolution. However, no local compression or edema noted. Observation and monitoring were opted versus surgical revision. Propitiously the aforementioned cranial nerve palsies persist for a month and subsequently subsided. In this case, we highlight the potential deleterious impact of aspirating cystic component and curettaging during pituitary surgery. Likely postulated accounts for such occurrence include sudden release of fluid pressure with resultant cystic traction on its enfolding cranial nerves and subsequent neuropraxia. We aim to invite comments that could enlighten us on this gray area.

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