Stereotactic radio-neurosurgery for jugular foramen schwannomas.

BACKGROUND: Stereotactic radiosurgery (SRS) represents a minimally invasive and valuable alternative for jugular foramen schwannomas (JFS), both as upfront and/or adjuvant treatment (in hybrid approaches). METHODS: We conducted a retrospective review of our cases treated at the Lausanne University Hospital (CHUV) from June 2010 to October 2023. Eleven patients underwent SRS, among whom three had prior surgery, two in our center in the frame of a planned combined approach and one in another center. Two patients received "volume-staged" SRS. The mean age at SRS was 60 years (median 68; range 29-83). Cranial nerve (CN) symptoms were present in six patients, while five were asymptomatic. The mean tumor volume at SRS was 2.1 cc (median 1.2; range 0.068-7.3 cc), with a 12 Gy marginal dose prescribed in all cases. RESULTS: The mean follow-up period was 3.9 years (median 2, range 1-7). Cranial nerve function improved after SRS in six patients, while five remained stable. At the last follow-up, all tumors showed a decrease in volume, except for one patient, who underwent surgery at 18 months after SRS, for volumetric increase at 6 and 12 months, with further XII-th CN palsy and medulla oblongata compression. Although tumor decreased at 18 months, such patient needed microsurgical resection for symptom persistence and was further controlled. The mean tumor volume at 1 year post-SRS was 1.6 cc (median 0.55; range 0.028-7.77 cc), at 2 years was 1.31 cc (median 0.76; range 0.19-5), and at 3 years was 1.32 cc (median 0.59; range 0.23-4.8). No adverse radiation events were observed. CONCLUSIONS: Stereotactic radiosurgery is considered a safe and effective treatment for jugular foramen schwannomas, ensuring high rates of tumor control in all patients over the long term. The cranial nerve function improved after SRS in the 6 patients who had deficits and the other 5 patients who had no deficits remained asymptomatic. For larger tumors, combined/hybrid approaches can be a valuable alternative, to obtain tumor control and to preserve neurological function.

Stereotactic radiosurgery for the treatment of hypoglossal schwannoma: a multi-institutional retrospective study.

BACKGROUND: Surgical removal has been performed as the first line treatment for symptomatic or enlarging hypoglossal schwannomas (HS). Stereotactic radiosurgery (SRS) offers a minimally invasive approach that may afford long-term tumor control for patients with HS particularly those who refuse or are unfit for surgery. This study evaluates outcomes after SRS performed for both newly diagnosed and residual tumors after incomplete resection. METHODS: This retrospective, multi-institutional study involved patients treated with adjuvant or primary SRS for HS. The study end-points included local tumor response, clinical outcomes, and procedure-related complications. All the patients had Gamma Knife SRS. RESULTS: The cohort included 12 patients (five females), median age at SRS 49.5 years (range, 37-76)]. The median tumor target volume was 5.9 cm3 (range, 0.7-27.23). At median imaging follow-up of 37 months (range, 6-153), tumor control was achieved in 11 patients. Tumor enlargement that was managed with surgical resection was noted at the 6-month follow-up in one patient. At median clinical follow-up of 30.5 months (range, 6-157), stability, or improvement of all pre-SRS signs and symptoms was noted in nine patients. Two patients experienced worsening of at least one pre-existing symptoms or sign. New-onset trapezius weakness was noted in one patient and tongue atrophy in two patients. CONCLUSION: Single-fraction SRS appears to be a safe and effective upfront and adjuvant treatment option for HS. SRS may be recommended as an alternative to surgery for patients presenting with HS or as an adjuvant treatment following subtotal resection and at HS recurrence.

Targeting the cMET pathway augments radiation response without adverse effect on hearing in NF2 schwannoma models.

Neurofibromatosis type II (NF2) is a disease that needs new solutions. Vestibular schwannoma (VS) growth causes progressive hearing loss, and the standard treatment, including surgery and radiotherapy, can further damage the nerve. There is an urgent need to identify an adjunct therapy that, by enhancing the efficacy of radiation, can help lower the radiation dose and preserve hearing. The mechanisms underlying deafness in NF2 are still unclear. One of the major limitations in studying tumor-induced hearing loss is the lack of mouse models that allow hearing testing. Here, we developed a cerebellopontine angle (CPA) schwannoma model that faithfully recapitulates the tumor-induced hearing loss. Using this model, we discovered that cMET blockade by crizotinib (CRZ) enhanced schwannoma radiosensitivity by enhancing DNA damage, and CRZ treatment combined with low-dose radiation was as effective as high-dose radiation. CRZ treatment had no adverse effect on hearing; however, it did not affect tumor-induced hearing loss, presumably because cMET blockade did not change tumor hepatocyte growth factor (HGF) levels. This cMET gene knockdown study independently confirmed the role of the cMET pathway in mediating the effect of CRZ. Furthermore, we evaluated the translational potential of cMET blockade in human schwannomas. We found that human NF2-associated and sporadic VSs showed significantly elevated HGF expression and cMET activation compared with normal nerves, which correlated with tumor growth and cyst formation. Using organoid brain slice culture, cMET blockade inhibited the growth of patient-derived schwannomas. Our findings provide the rationale and necessary data for the clinical translation of combined cMET blockade with radiation therapy in patients with NF2.

[Progress in the surgical treatment of trigeminal schwannoma].

Trigeminal schwannomas(TSs) is the second most common intracranial schwannomas next to acoustic neuroma. These uncommon, slow-growing tumors, which prove to originated from Schwann cells of the fifth brain nerve, can achieve their development into various compartments.Previous to the era of microsurgery, the total tumor resection rate was low, and the postoperative neurological function was seriously damaged.With the development of microsurgery and skull base approach, the microsurgery effect of TSs has been improved. Besides, neuroendoscopy is also used in the operation of some types of trigeminal schwannomas. Radiation therapy can be performed to control tumor growth for the patients who cannot tolerate surgery, have small tumor volume, have residual tumor tissue or relapse.

Gamma Knife radiosurgery for intravestibular and intracochlear schwannomas.

BACKGROUND: Schwannomas of the VIIIth cranial nerve are benign tumours, with vast majority occurring in vestibular division. Rarely, they can also arise from distal branches of cochlear, superior or inferior vestibular. We review our experience with Gamma Knife radiosurgery (GKR), as first intention treatment for intracochlear (ICS) and intravestibular (IVS) schwannomas. METHODS: A total number of five patients were analysed, treated over 8 years, between June 2010 and September 2018, with Leksell Gamma Knife Perfexion or Icon (Elekta Instruments, AB, Sweden). The marginal dose prescribed was 12 Gy at a mean prescription isodose line of 61.4% (range 50-70). Clinical evaluation included auditory and facial function. RESULTS: The mean age was 49.9 (range 34-63). The mean follow-up period was 52.8 months (range 12-84). The mean target volume (TV) was 0.087 ml (range 0.014-0.281). The mean maximal dose received by the cochlea was 11.2 Gy (range 2.6-20.3). The mean marginal dose received by the vestibule (e.g. utricula) was 14.2 Gy (range 3.8-17.5). No patient experienced an acute or subacute clinical adverse radiation effect after GKR. Four cases had overall symptom stability. In one patient (1/5), the vertigo, which was the main clinical complain, disappeared 1 year after GKR. However, it reappeared 3 years later, with same pretherapeutic characteristics and is currently fluctuating. One patient experienced hearing decrease after GKR, during the first 12 months. This case received 11.2 Gy to the cochlea. Follow-up MRI course showed a decrease in size in four patients, and stability in one. CONCLUSIONS: Gamma Knife radiosurgery is a valuable first intention treatment for ICS or IVS, in selected cases. Special attention should be paid for the dose delivered to the cochlea and the vestibular apparatus. Acute and subacute clinical effects are exceptional, while tumour control was achieved in all cases in our small series.

Radiotherapy for parapharyngeal space tumors.

A wide variety of tumors, both benign and malignant, occur in the parapharyngeal space. Depending on histology and extent, treatment may include surgery and/or radiotherapy (RT). Herein we discuss the role of RT in the management of some of the more commonly encountered neoplasms, including salivary gland tumors, paragangliomas, schwannomas, and soft-tissue sarcomas.

Feasibility of MR-only radiation planning for hypofractionated stereotactic radiotherapy of schwannomas using non-coplanar volumetric modulated arc therapy.

PURPOSE: To compare the dose calculation accuracy of plans done on a CT density-assigned MR image set for hypofractionated stereotactic radiotherapy (HSRT) using volumetric modulated radiation therapy containing non-coplanar beams. METHODS: Eighteen patients diagnosed with schwannoma treated with HSRT were selected retrospectively. These patients underwent planning CT (pCT) for radiation therapy (RT) and contrast-enhanced three-dimensional fast-spoiled gradient-echo image (3D FSPGR) to assist tumor delineation. CTplan is plan done on pCT. The structures body, bone, and air are contoured exclusively on MR image and assigned Hounsfield units of 25, + 1000, and - 1000, respectively. This is termed as MRCT. After registration, original plans from pCT are pasted on the MRCT. Dose calculation is done in two ways: (1) with preset MU values (DDC) and (2) with optimization (OPT_DC). Conformity indices and Dmax and D0.5cc of brainstem, gamma agreement index and correlation coefficient are analyzed. ANOVA test is carried out to find the significance of difference between plans. RESULTS: The mean deviations of Dmax and D0.5cc of brainstem for CTplan versus DDC are 2.49% and 1.45% respectively. The mean deviations of Dmax and D0.5cc of brainstem for CTplan versus OPT_DC are - 1.56% and - 1.97%, respectively. Mean deviations of conformity index for DDC and OPT_DC are 0.84% and 0.89%, respectively. No significant difference was found with ANOVA test. CONCLUSION: Results show that there is no difference between plans generated with actual CT data and MRCT data. Thus MR scans could be employed for radiation planning provided the verification image is available. This gives us confidence to reduce treatment margins where image registration process is avoided.

Radiosurgery in trochlear and abducens nerve schwannomas: case series and systematic review.

INTRODUCTION: Schwannomas involving the occulomotor cranial nerves  (CNs; III, IV and VI), can be disabling, due to the associated diplopia and decreased quality of life and are extremely rare. We evaluated the role of Gamma Knife surgery (GKS) in these cases. METHODS: Five patients with CN IV and VI schwannomas (three and two, respectively) were treated in Lausanne University Hospital between 2010 and 2015. Four benefitted from upfront GKS and one from a combined approach (planned subtotal resection followed by GKS), due to a large preoperative tumour volume (size, 3 × 2 × 2.5 cm; volume, 7.9 ml), with symptomatic mass effect and oedema, as well as an entrapement cyst at the brainstem interface, in a young patient. Neuro-ophtalmological evaluation was performed at baseline and during each follow-up time-point. A systematic literature review is presented and compared to the present report. RESULTS: The mean follow-up was 44.4 months (12-54). Initial clinical presentation was diplopia in four cases and cavernous sinus syndrome in one. The marginal dose was 12 Gy in all cases. The mean target volume was 1.51 cm3 (0.086-5.8). The mean prescription isodose volume (PIV) was 1.71 cm3 (0.131-6.7). At last follow-up, all patients presented with disappearance of the baseline symptoms. Tumour control was achieved in 100%, with decrease in volume in all cases. The systematic review analysed 11 peer-reviewed studies, with a total of 35 patients. For uniformly reported CN VI, the mean marginal radiation dose ranged between 12 and 12.5 Gy, with disappearance of symptoms in 12.5%, improvement in 31.25%, stabilisation in 6.25%, worsening in 12.5%. Tumour volume decreased in all cases. CONCLUSIONS: Our data suggest that first intention GKS is a safe and effective option for patients with small to medium size oculomotor schwannomas, providing a high rate of clinical alleviation and tumour control. When the initial tumour volume is too large for first intention GKS, a combined approach with planned subtotal resection followed by GKS can be performed, with favourable and comparable outcomes as in upfront GKS.

Uncontrolled spread following radiosurgery for a skull base aspergilloma misdiagnosed as schwannoma: is radiosurgery responsible?

BACKGROUND: Little is known about the natural history of skull base fungal lesions in immunocompetent individuals and the effect of high-dose radiation on fungal lesions. METHOD AND RESULTS: We report a case where radiosurgery was given to a skull base aspergilloma, mistaking it to be a trigeminal schwannoma. There was dramatic spread of the lesion to periventricular region with significant increase in the skull base lesion. The patient never received steroids. A stereotactic biopsy established the diagnosis, and treatment with voriconazole helped. CONCLUSIONS: It is possible that radiosurgery decreases local immunity due to vascular sclerosis and aids in spread of the fungal lesion, though it is only speculative. This again highlights the importance of establishing the diagnosis prior to radiosurgery.

A patient with a large intrathoracic malignant schwannoma who showed a complete clinical response to rAd-p53-combined with radiotherapy.

The prognosis of postoperatively recurred malignant schwannoma is poor and there is no effective treatment. We had a patient who was found to have a large intrathoracic tumor 1 year after surgery and could not tolerate an operation for the second time. We then decided to evaluate the synergistic effect of recombinant adenovirus-p53 (rAd-p53) combined with radiotherapy for the patient. rAd-p53 was injected intratumorally twice a week before radiotherapy, a total of 10 times, over a course of treatment. Radiotherapy then followed gene therapy at five fractions a week for 5 weeks, with a total dosage of 80.6 Gy/31f in the center part of the tumor and 62 Gy/31f in other locations. The pathological diagnosis of malignant schwannoma indicated that the p53 expression was strongly positive and vascular endothelial growth factor and Bcl-2 were positive before treatment on protein immunohistochemical staining. After treatment, the diameter of the tumor was noticeably reduced and the center part of the tumor presented as a fluid anechoic area and cavities on computed tomographic scanning. The result of the puncture biopsy showed that there were many fibronecrotic tissues and no significant tumor cells. The p53 expression was weakly positive, Vascular endothelial growth factor was negative, and Bcl-2 was weakly positive after treatment on protein immunohistochemical staining.

Preclinical evaluation of the combination of mTOR and proteasome inhibitors with radiotherapy in malignant peripheral nerve sheath tumors.

About one half of malignant peripheral nerve sheath tumors (MPNST) have Neurofibromin 1 (NF1) mutations. NF1 is a tumor suppressor gene essential for negative regulation of RAS signaling. Survival for MPNST patients is poor and we sought to identify an effective combination therapy. Starting with the mTOR inhibitors rapamycin and everolimus, we screened for synergy in 542 FDA approved compounds using MPNST cells with a native NF1 loss in both alleles. We further analyzed the cell cycle and signal transduction. In vivo growth effects of the drug combination with local radiation therapy (RT) were assessed in MPNST xenografts. The synergistic combination of mTOR inhibitors with bortezomib yielded a reduction in MPNST cell proliferation. The combination of mTOR inhibitors and bortezomib also enhanced the anti-proliferative effect of radiation in vitro. In vivo, the combination of mTOR inhibitor (everolimus) and bortezomib with RT decreased tumor growth and proliferation, and augmented apoptosis. The combination of approved mTOR and proteasome inhibitors with radiation showed a significant reduction of tumor growth in an animal model and should be investigated and optimized further for MPNST therapy.

Clinical and Imaging Outcomes After Trigeminal Schwannoma Radiosurgery: Results From a Multicenter, International Cohort Study.

BACKGROUND AND OBJECTIVES: An international, multicenter, retrospective study was conducted to evaluate the long-term clinical outcomes and tumor control rates after stereotactic radiosurgery (SRS) for trigeminal schwannoma. METHODS: Patient data (N = 309) were collected from 14 international radiosurgery centers. The median patient age was 50 years (range 11-87 years). Sixty patients (19%) had prior resections. Abnormal facial sensation was the commonest complaint (49%). The anatomic locations were root (N = 40), ganglion (N = 141), or dumbbell type (N = 128). The median tumor volume was 4 cc (range, 0.2-30.1 cc), and median margin dose was 13 Gy (range, 10-20 Gy). Factors associated with tumor control, symptom improvement, and adverse radiation events were assessed. RESULTS: The median and mean time to last follow-up was 49 and 65 months (range 6-242 months). Greater than 5-year follow-up was available for 139 patients (45%), and 50 patients (16%) had longer than 10-year follow-up. The overall tumor control rate was 94.5%. Tumors regressed in 146 patients (47.2%), remained unchanged in 128 patients (41.4%), and stabilized after initial expansion in 20 patients (6.5%). Progression-free survival rates at 3 years, 5 years, and 10 years were 91%, 86%, and 80 %. Smaller tumor volume (less than 8 cc) was associated with significantly better progression-free survival ( P = .02). Seventeen patients with sustained growth underwent further intervention at a median of 27 months (3-144 months). Symptom improvement was noted in 140 patients (45%) at a median of 7 months. In multivariate analysis primary, SRS ( P = .003) and smaller tumor volume ( P = .01) were associated with better symptom improvement. Adverse radiation events were documented in 29 patients (9%). CONCLUSION: SRS was associated with long-term freedom (10 year) from additional management in 80% of patients. SRS proved to be a valuable salvage option after resection. When used as a primary management for smaller volume tumors, both clinical improvement and prevention of new deficits were optimized.

Intra-parotid facial nerve schwannoma with intra-temporal extension; a case report. Is there a role for stereotactic radiotherapy?

Facial nerve schwannoma (FNS) is an extremely rare benign tumour that may arise anywhere along the course of the facial nerve; the standard treatment is total removal via microsurgery. Stereotactic radiotherapy has been shown to be effective in the treatment of skull base tumours, in particular for acoustic neuromas; it is interesting to notice that also the few data existing in literature about the use of radiotherapy for non acoustic schwannomas show an excellent local control rate and few adverse effects. Here we report a case of facial nerve neuroma, involving the nerve sheath from the geniculate ganglion to the parotid gland, treated with fractionated stereotactic radiotherapy after debulking surgery.

Schwannoma of the facial nerve: indications for surgical treatment.

There is no preoperative diagnostic modality that can identify facial nerve schwannoma with certainty. Difficulty in locating the facial nerve intraoperatively raises suspicion of a neurogenous tumor of the facial nerve, and this may prevent unnecessary damage to the nerve. Not every facial nerve schwannoma should be resected.

The Treatment Outcome of CyberKnife-Based Stereotactic Radiotherapy for Intracranial and Extracranial Nonvestibular Schwannomas: A Single-Center Experience.

BACKGROUND: Data on the outcomes of CyberKnife-based hypofractionated stereotactic radiosurgery (hSRS) for intracranial and extracranial nonvestibular schwannomas (nVSs) are not sufficient. METHODS: Patients who underwent hSRS for nVSs between 2010 and 2019 were retrospectively reviewed. RESULTS: A total of 39 patients with 39 nVSs were identified. The mean age was 53 (±18) years, and 20 patients (51%) were women. Twenty-five patients (64%) had previous surgeries. Seventeen patients (44%) had nVSs extending outside the cranium. The mean prescribed dose covering 95% of the planning target volume was 22 Gy (±3.7 Gy), the mean fractionation was 4 (±2), and the mean target volume was 13 cm3 (±16 cm3). The radiological tumor control rate was 100% during the mean follow-up period of 67 months (±37 months). Thirty-seven patients (95%) were clinically stable during the mean follow-up period of 72 months (±35 months). Nine patients (23%) suffered from transient adverse radiation effects (AREs), including transient tumor expansion, and 2 (5%) suffered from permanent AREs. CONCLUSIONS: We summarized the treatment outcomes of hSRS for nVSs. Although all patients achieved radiological tumor control, the risk of either transient or permanent ARE was high. Therefore, it is necessary to monitor patients for clinical deterioration due to AREs.

Can Trigeminal Schwannoma-Related Symptoms be Controlled With Gamma Knife Radiosurgery?

OBJECTIVE: The effectiveness of gamma knife radiosurgery (GKRS) for the management of trigeminal schwannomas (TS) has been established. Although GKRS has been shown to inhibit tumor growth, the effect of GKRS on the symptoms caused by TS has not been sufficiently studied. In this study, symptomatic changes following GKRS for the management of symptomatic TS were investigated using long-term follow-up results. METHODS: A retrospective analysis was performed on 32 patients with TS who underwent GKRS between May 1994 and December 2016. Clinical charts, radiographic results, and surgical records were reviewed. To evaluate whether symptoms improved after GKRS, patient demographics, GKRS profile, radiological tumor size change, and tumor location were analyzed. RESULTS: Tumor control after GKRS for symptomatic TS was 87%. The improvement rates for facial pain at 6, 12, and 24 months after GKRS were 46%, 72%, and 86%, respectively. For the same time intervals, facial hypesthesia improved by 12%, 46%, and 52%, respectively. Of the patients with diplopia, 17% had improved symptoms 12 months after GKRS, and 50% of the patients improved after 24 months. CONCLUSIONS: GKRS can be an effective treatment modality for TS tumor control and shows favorable results in improving TS-related symptom, especially facial pain.

Stereotactic radiosurgery for hypoglossal schwannoma.

Hypoglossal schwannomas (HS) are extremely rare neoplasms. Surgical resection has historically been the treatment of choice but carries a significant risk of postoperative neurological deficits and mortality. Stereotactic radiosurgery (SRS) is a minimally invasive approach that may afford long-term tumour growth. However, literature to determine the safety and effectiveness of SRS in the treatment of HS is scarce. We report on a patient who presented with progressive headache and dysphagia as well as tongue deviation to the left, due to a space-occupying lesion, consistent on brain MRI with a left HS. Primary SRS using a prescription dose of 12 Gy in a single fraction was used to treat the tumour without complications. By last follow-up, the tumour regressed, and the patient's symptoms improved. Our case shows that radiosurgery can be safe and effective for the management of HS.

Thirty-year clinical experience in gamma knife radiosurgery for trigeminal schwannomas.

We aimed to evaluate the radiographic and clinical outcomes after gamma knife radiosurgery (GKRS) for trigeminal schwannomas (TSs). A total of 87 patients who underwent GKRS for TSs between 1990 and 2020 were enrolled. The mean tumor volume was 4.3 cm3. The median prescribed dose for the margins of the tumor was 13 Gy. The median follow-up duration was 64.3 months (range 12.0-311.5 months). The overall local tumor control rate was 90%, and the symptom response rate was 93%. The response rate for each symptom was 88% for facial pain, 97% for facial sensory change, and 86% for cranial nerve deficits. Nineteen (22%) patients showed transient swelling, which had regressed at the time of the last follow-up. Cystic tumors were associated with transient swelling (p = 0.04). A tumor volume of < 2.7 cm3 was associated with local tumor control in univariable analysis. Transient swelling was associated with symptom control failure in both univariable and multivariable analyses (p = 0.04, odds ratio 14.538). GKRS is an effective treatment for TSs, both for local control and symptom control.

Safety and Efficacy of Hypofractionated Stereotactic Radiosurgery in Facial Nerve Schwannoma.

OBJECTIVE: In the current era of modern neurosurgery, the treatment strategies have been shifted to "nerve-preservation approaches" for achieving a higher facial and hearing function preservation rate following facial nerve tumors. We have conducted this novel report on determining the outcome of patients with facial nerve schwannomas (FNS) treated with hypofractionated stereotactic radiosurgery (hfSRS). PATIENTS: Retrospective chart review of a prospectively maintained database search was conducted. INTERVENTION: Patients who underwent hfSRS CyberKnife (Accuray Inc, Sunnyvale, CA, U.S.A.) for FNS were included. MAIN OUTCOME MEASURES: Outcomes consisted of tumor control, facial and hearing nerve function as graded by House-Brackmann and American Academy of Otolaryngology-Head and Neck Surgery recommendations, and adverse radiation effects. RESULTS: With an institutional board review approval, we retrospectively identified five patients with FNS (four intracranial [80%] and one extracranial [20%]) treated with hfSRS (2011-2019). Patients received definitive SRS in three patients (60.0%), whereas adjuvant to surgical resection in two patients (40.0%). A median tumor volume of 7.5 cm 3 (range, 1.5-19.6 cm 3 ) received a median prescription dose of 23.2 Gy (range, 21-25 Gy) administered in median of three fractions (range, three to five sessions). With a median radiographic follow-up of 31.4 months (range, 13.0-71.0 mo) and clinical follow-up of 32.6 months (range, 15.1-72.0 mo), the local tumor control was 100.0%. At the last clinical follow-up, the facial nerve function improved or remained unchanged House-Brackmann I-II in 80.0% of the patients, whereas the hearing nerve function improved or remained stable in 100.0% of the patients. Temporary clinical toxicity was observed in three patients (60.0%), which resolved. None of the patients developed adverse radiation effect. CONCLUSION: From our case series, hfSRS in FNS seems to be safe and efficacious in terms of local tumor control, and improved facial and hearing nerve function.

Malignant peripheral nerve sheath tumour of the trigeminal nerve: case report and literature review.

Although all trigeminal nerve schwannomas are uncommon, malignant schwannomas are extraordinarily rare. We describe a patient who presented with clinical and radiological features of a trigeminal schwannoma; however, pathological analysis showed a malignant peripheral nerve sheath tumour (WHO Grade IV). We discuss these extremely rare tumours and their management.