A vestibular schwannoma (also known as acoustic neuroma, acoustic neurinoma, or acoustic neurilemoma) is a benign, usually slow-growing cerebellopontine angle tumor that develops from the balance and hearing nerves supplying the inner ear. The tumor comes from an overproduction of Schwann cells.
Gao S et al., reported a cerebellar glioblastoma multiforme patient, with his clinical presentations and imaging characteristics mimicking a vestibular schwannoma. To the best of authors knowledge, this is the first reported patient with cGBM mimicking a vestibular schwannoma 1).
Case-control study of 37 patients who underwent surgical resection of sporadic VS following prior SRS at two tertiary academic referral centers between 2003 and 2015. A cohort of nonirradiated control subjects, matched according to tumor size, age, and treatment center, were used as comparison.
Thirty-seven patients were included. The median time from radiation to surgical salvage was 36 months (range 9.6-153 months). Following tumor progression after SRS, 18 (49%) patients underwent gross total resection, 10 (27%) underwent near-total resection, and nine (24%) underwent subtotal resection. Postoperative complications following salvage surgery included one (3%) case of stroke, four (11%) cases of cerebrospinal fluid leak, and two (5%) cases of meningitis. Twenty-seven (73%) patients had good postoperative facial nerve outcome (House-Brackmann Score I-II) at long-term follow-up. There were no cases of tumor recurrence or regrowth after a median length of 26 months following microsurgical salvage (range 3-114 months). The rate of satisfactory postoperative facial nerve function was not different between study and control subjects (73% vs. 76%; P = 0.8); however, less-than-complete resection was utilized more frequently among previously radiated patients (P = 0.01).
Microsurgical salvage of VS following primary radiation therapy is challenging. Less-than-complete resection is required in a greater percentage of patients to preserve facial nerve integrity and prevent neurological complications. Long-term follow-up is needed to determine the risk of delayed progression following incomplete tumor removal 2).
A total of 106 patients with unilateral VS were enrolled in this study prospectively. Each patient received a caloric reflex test, vestibular evoked myogenic potential (VEMP) test, and cochlear nerve function test (hearing) before the operation and 1 week, 3, and 6 months, postoperatively. All patients underwent surgical removal of the VS using the suboccipital approach. During the operation, the nerve of tumor origin (SVN or IVN) was identified by the surgeon. Tumor size was measured by preoperative magnetic resonance imaging.
The nerve of tumor origin could not be unequivocally identified in 38 patients (38/106, 35.80%). These patients were not subsequently evaluated. In 26 patients (nine females, seventeen males), tumors arose from the SVN and in 42 patients (18 females, 24 males), tumors arose from the IVN. Comparing with the nerve of origins (SVN and IVN) of tumors, the results of the caloric tests and VEMP tests were significantly different in tumors originating from the SVN and the IVN in our study. Hearing was preserved in 16 of 26 patients (61.54%) with SVN-originating tumors, whereas hearing was preserved in only seven of 42 patients (16.67%) with IVN-originating tumors.
The data suggest that caloric and VEMP tests might help to identify whether VS tumors originate from the SVN or IVN. These tests could also be used to evaluate the residual function of the nerves after surgery. Using this information, we might better predict the preservation of hearing for patients 3).
A retrospective cohort study of 489 patients who underwent vestibular schwannoma resection at the Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota between 2000 and 2014. Delayed facial palsy was defined as deterioration in facial function of at least 2 House-Brackmann (HB) grades between postoperative days 5 to 30. Only patients with a HB grade of I to III by postoperative day 5 were eligible for study inclusion.
One hundred twenty-one patients with HB grade IV to VI facial weakness at postoperative day 5 were excluded from analysis. Of the remaining 368, 60 (16%) patients developed DFP (mean 12 days postoperatively, range: 5-25 days). All patients recovered function to HB grade I to II by a mean of 33 days (range: 7-86 days). Patients that developed DFP had higher rates of gross total resections (83% vs 71%, P = .05) and retrosigmoid approaches (72% vs 52%, P < .01). There was no difference in recovery time between patients who received treatment with steroids, steroids with antivirals, or no treatment at all (P = .530).
Patients with a gross total tumor resection or undergoing a retrosigmoid approach may be at higher risk of DFP. The prognosis is favorable, with patients likely recovering to normal or near-normal facial function within 1 month of onset 4).
Thirty-two patients diagnosed with acoustic neuroma received contrast-enhanced magnetic resonance imaging of brain were recruited. The volume was calculated by the ABC/2 equation and planimetry method (defined as exact volume) at the same time. The 32 patients were divided into three groups by tumor volume to avoid volume-dependent overestimation (<3 ml, 3-6 ml and >6 ml).
The tumor volume by ABC/2 method was highly correlated to that calculated by planimetry method using linear regression analysis (R2=0.985). Pearson correlation coefficient (r=0.993, p<0.001) demonstrates nearly perfect association between two methods.
The ABC/2 formula is an easy method in estimating the tumor volume of acoustic neuromas that is not inferior to planimetry method 5).
Thirty-eight VS patients were split in three groups according to caloric vestibular test results before surgery; nine had a symmetrical vestibular response (vestibular normoreflexy), 19 with a decreased response of more than 20% of the affected side (vestibular hyporeflexy) and 10 with an absent caloric response on the side of the affected labyrinth (vestibular areflexy). They underwent pendular rotary vestibular testing (RVT), allowing to evaluate gain and directional preponderance of the vestibulo-ocular reflex, and a sensory organisation test (SOT), evaluating balance control in six conditions (C1 to C6). These tests were performed shortly before, and 8 and 90 days after surgery. Directional preponderance performances of patients with vestibular normoreflexy or hyporeflexy followed a classical time-course with a huge asymmetry just after surgery and a recovery to pre-operative performances at 90 days; patients with vestibular areflexy were relatively stable in time. Variation in SOT performances of patients with vestibular normoreflexy, especially in the more complex C4 to C6, followed a classical time-course with an important postural degradation just after surgery and a recovery to pre-operative performances at 90 days. Patients with vestibular areflexy showed no balance degradation just after surgery and a marked increase in performances at 90 days after surgery, especially in C5 and C6. Performances of patients with vestibular hyporeflexy were intermediate, close to performances of patients with vestibular normoreflexy before surgery and close to performances of patients with vestibular areflexy at 8 and 90 days after surgery. Pre-operative vestibular function alteration triggers an adaptive process, characterized by a restoration of the symmetry of the vestibular nuclei activity and by sensory substitution and new behavioural strategies, allowing the anticipation of unilateral vestibular deafferentation effects 6).
Seventy-six patients underwent the primary removal of an acoustic neurinoma at the Mayo Clinic from 1978 through 1980. Hearing loss was present in 97% of the patients, and tinnitus and dysequilibrium occurred in 70% of the patients. The most common signs were a decreased corneal reflex, nystagmus, and facial hypesthesia. In these patients, pure tone and speech audiometry are used to define the hearing loss. When hearing is still present, the speech discrimination is often disproportionately low. Acoustic reflex testing and brain stem evoked response are used to determine whether the hearing loss is cochlear or retrocochlear. When these tests could be performed in this series of patients, they were accurate in 85 to 95%. The vestibular response to caloric testing is expected to be decreased or absent in about 90% of patients, and this was so in 86% of our patients. Radiographic studies are the most important tests currently used for the diagnosis of acoustic neurinoma. Tomography of the internal auditory canal shows abnormalities in 80% of patients. Computed tomography with contrast enhancement demonstrates abnormalities in 90% of patients. The computed tomographic (CT) scan may reveal the location, the size, and often the consistency of the tumor. In cases still questionable after CT scanning, positive contrast rhombencephalography is used for clarification. In this series, no single symptom, sign, abnormal audiometric test result, or abnormal radiographic finding was present in all patients; therefore, the most important factor in diagnosis is an alert physician 7).
126 tumors have been operated by Sterkers from 1966 to 1978. The surgery was done either by the middle fossa, or the translabyrinthine, or the retrosigmoid approach. The perservation of the Facial nerve is obtained in 93%, the facial function is normal in 70%. The hearing is preserved after removal of the tumor in 50% of the intracanalar neuromas, and in 35% after removal of tumors expanded in the angle 8)
Temporal bone invasion by VS is extremely rare. A 51-year-old man who revealed temporal bone destruction beyond IAC by unilateral VS. The bony destruction extended anteriorly to the carotid canal and inferiorly to the jugular foramen. On histopathologic examination, the tumor showed typical benign schwannoma and did not show any unusual vascularity or malignant feature. Facial nerve was severely compressed and distorted by tumor, which unevenly eroded temporal bone in surgical field. Vestibular schwannoma with atypical invasion of temporal bone can be successfully treated with combined translabyrinthine and lateral suboccipiral approach without facial nerve dysfunction. Early detection and careful dissection of facial nerve with intraoperative monitoring should be considered during operation due to severe adhesion and distortion of facial nerve by tumor and eroded temporal bone 9)
A 15-year-old male who presented with hearing loss due to a small left-sided vestibular schwannoma (VS) not associated with neurofibromatosis type 2 (NF2), which had been apparent for six months. Magnetic resonance imaging with gadolinium diethylenetriamine penta-acetic acid revealed a mass, 10 mm in diameter, located in the left inner auditory canal. The patient had no family history of NF2 and gene mutation analysis showed no signs of the condition. Small sporadic or non-NF2 VS is extremely rare and the treatment decision-making process is complicated in children when considering the implications for the impairment of childhood development and lifelong disability. Following careful consideration, the patient in the present study underwent treatment with stereotactic radiosurgery. The five-year post-operative follow-up examination showed tumor stability without additional neurological deficits and at the time of writing the patient was alive and well 10).