Surgical and radiosurgical outcomes for Koos grade 3 vestibular schwannomas.

This study aimed to reveal the preferred initial treatment for Koos grade 3 vestibular schwannomas (VS). We performed a two-institutional retrospective study on 21 patients with Koos grade 3 VS undergoing resection at Yokohama Medical Center and 37 patients undergoing radiosurgery at Yokohama Rosai Hospital from 2010 to 2021. Tumor control, complications, and functional preservation were compared. The median pre-treatment volume and follow-up duration were 2845 mm3 and 57.0 months, respectively, in the resection group and 2127 mm3 and 81.7 months, respectively, in the radiosurgery group. In the resection group, 16 (76.2%) underwent gross total resection, and three patients (14.3%) experienced regrowth; however, no one required additional treatment. In the radiosurgery group, the tumor control rate was 86.5%, and three cases (8.1%) required surgical resection because of symptomatic brainstem compression. Kaplan-Meier analyses revealed that tumors with delayed continuous enlargement and large thin-walled cysts were significantly associated with poor prognostic factors (p = 0.0027, p < 0.001). The pre-radiosurgery growth rate was also associated with the volume increase (p = 0.013). Two cases (9.5%) required additional operation due to complications such as post-operative hematoma and cerebrospinal fluid leaks in the resection group, whereas temporary cranial neuropathies were observed in the radiosurgery group. Two patients (9.5%) had poor facial nerve function (House-Brackmann grading grade 3) in the resection group, while no one developed facial paresis in the radiosurgery group. Trigeminal neuropathy improved only in the resection group.Radiosurgery can be considered for the treatment of Koos grade 3 VS for functional preservation. However, resection may also be considered for patients with severe trigeminal neuropathy or a high risk of volume increments, such as large thin-walled cysts and rapid pre-treatment growth.

Stereotactic radiosurgery for Koos grade IV vestibular schwannoma: a systematic review and meta-analysis.

BACKGROUND: Stereotactic radiosurgery (SRS) is a well-established treatment option for Koos stage I-III vestibular schwannomas (VS), often used as the first line of treatment or after subtotal resection. However, the optimal treatment for Koos-IV VS remains unclear. Therefore, our study aimed to evaluate the effectiveness of SRS as a primary treatment for large VS classified as Koos-IV. METHODS: A systematic search was performed on December 28th, 2022, based on PubMed, Web of Science, and Scopus according to the PRISMA statement. The review was updated on September 7th, 2023. The risk of bias was assessed using the NIH Quality Assessment Tool. The R software (ver. 4.3.2) was used for all quantitative analyses and preparation of the forest plots. Publication bias and sensitivity analysis were performed to evaluate the reliability of the obtained results. RESULTS: Among 2941 screened records, ten studies (1398 patients) have been included in quantitative synthesis. The overall tumor control rate was 90.7% (95%CI 86.3-94.4). Kaplan-Meier estimates of tumor control at 2, 6, and 10 years were 96.0% (95% CI 92.9-97.6%), 88.8% (95% CI 86.9-89.8%), and 84.5% (95% CI, 81.2-85.8%), respectively. The overall hearing preservation rate was 56.5% (95%CI 37-75.1). Kaplan-Meier estimates of hearing preservation rate at 2, 6, and 10 years were 77.1% (95% CI 67.9-82.5%), 53.5% (95% CI 44.2-58.5%), and 38.1% (95% CI 23.4-40.7%), respectively. The overall facial nerve preservation rate was 100% (95%CI 99.9-100.0). The overall trigeminal neuropathy rate reached 5.7% (95%CI 2.9-9.2). The overall rate of new-onset hydrocephalus was 5.6% (95%CI 3-9). The overall rates of worsening or new-onset tinnitus and vertigo were 6.8% (95%CI 4.2-10.0) and 9.1% (95%CI 2.1-19.6) respectively. No publication bias was detected according to the used methods. CONCLUSIONS: Our systematic review and meta-analysis demonstrated a high overall tumor control rate, excellent facial nerve preservation, and low incidence of new-onset or worsened tinnitus and vertigo. However, several drawbacks associated with SRS should be noted, such as the presence of post-SRS hydrocephalus risk, mediocre long-term hearing preservation, and the lack of immediate tumor decompression. Nevertheless, the use of SRS may be beneficial in appropriately selected cases of Koos-IV VS. Moreover, further prospective studies directly comparing SRS with surgery are necessary to determine the optimal treatment for large VS and verify our results on a higher level of evidence. Registration and protocol: CRD42023389856.

Stereotactic radiosurgery for Koos grade IV vestibular schwannoma in patients ≥ 65 years old: a multi-institutional retrospective study.

BACKGROUND: Surgery is the preferred treatment for large vestibular schwannomas (VS). Good tumor control and cranial nerve outcomes were described in selected Koos IV VS after single-session stereotactic radiosurgery (SRS), but outcomes in elderly patients have never been specifically studied. The aim of this study is to report clinical and radiological outcomes after single-session SRS for Koos IV VS in patients ≥ 65 years old. METHOD: This multicenter, retrospective study included patients ≥ 65 years old, treated with primary, single-session SRS for a Koos IV VS, and at least 12 months of follow-up. Patients with life-threatening or incapacitating symptoms were excluded. Tumor control rate, hearing, trigeminal, and facial nerve function were studied at last follow-up. RESULTS: One-hundred and fifty patients (median age of 71.0 (IQR 9.0) years old with a median tumor volume of 8.3 cc (IQR 4.4)) were included. The median prescription dose was 12.0 Gy (IQR 1.4). The local tumor control rate was 96.0% and 86.2% at 5 and 10 years, respectively. Early tumor expansion occurred in 6.7% and was symptomatic in 40% of cases. A serviceable hearing was present in 16.1% prior to SRS and in 7.4% at a last follow-up of 46.5 months (IQR 55.8). The actuarial serviceable hearing preservation rate was 69.3% and 50.9% at 5 and 10 years, respectively. Facial nerve function preservation or improvement rates at 5 and 10 years were 98.7% and 91.0%, respectively. At last follow-up, the trigeminal nerve function was improved in 14.0%, stable in 80.7%, and worsened in 5.3% of the patients. ARE were noted in 12.7%. New hydrocephalus was seen in 8.0% of patients. CONCLUSION: SRS can be a safe alternative to surgery for selected Koos IV VS in patients ≥ 65 years old. Further follow-up is warranted.

Surgical outcomes in large vestibular schwannomas: should cerebellopontine edema be considered in the grading systems?

PURPOSE: Large (> 3 cm) vestibular schwannomas pose complexity in surgical management because of narrow working corridors and proximity to the cranial nerves, brainstem, and inner ear structures. With current vestibular schwannoma classifications limited in information regarding cerebellopontine edema, our retrospective series examined this radiographic feature relative to clinical outcomes and its possible role in preoperative scoring. METHODS: Of 230 patients who underwent surgical resection of vestibular schwannoma (2014-2020), we identified 107 patients with Koos grades 3 or 4 tumors for radiographic assessment of edema in the middle cerebellar peduncle (MCP), brainstem, or both. Radiographic images were graded and patients grouped into Koos grades 3 or 4 or our proposed grade 5 with edema. Tumor volumes, radiographic features, clinical presentations, and clinical outcomes were evaluated. RESULTS: The 107 patients included 22 patients with grade 3 tumors, 39 with grade 4, and 46 with grade 5. No statistical differences were noted among groups for demographic data or complication rates. Unlike grades 3 and 4 patients, grade 5 patients presented with worse hearing (p < 0.001), larger tumors (p < 0.001), lower rates of gross total resection (GTR), longer hospital stays, and higher rates of balance dysfunction. CONCLUSION: With edema detected in 43% of this cohort, special considerations are warranted for grade 5 vestibular schwannomas given the preoperative findings of worse hearing, lower GTR rates, longer hospital stays, and 96% who pursued postoperative balance therapy. We propose that grade 5 with edema offers a more nuanced interpretation of a radiographic feature that holds relevance to treatment selection and patient outcomes.

Stereotactic radiosurgery ensures an effective and safe long-term control of Koos grade IV vestibular schwannomas: a single-center, retrospective, cohort study.

PURPOSE: Stereotactic radiosurgery (SRS) is a standard treatment modality for vestibular schwannomas (VSs). However, there is a paucity of data on tumor control and neurological preservation for larger VSs. We aimed to investigate the long-term effectiveness of SRS for Koos grade IV compared with I-III VSs. METHODS: We included 452 patients with VSs (50 Koos grade IV and 402 Koos grade I‒III) who were treated with SRS at our institution from 1990 to 2021. Tumor control and functional preservation were calculated using the Kaplan-Meier method and compared between groups with the log-rank test. RESULTS: The median post-SRS follow-up period was 68 months. Progression-free survival rates were 91% at 5 and 10 years for Koos grade IV VSs, and 95% and 92%, respectively, for Koos grade I‒III VSs (p = 0.278). In Koos grade IV VSs, functional preservation rates of the facial and trigeminal nerves were both 96% at 5 years (both 98% for Koos grade I‒III VSs; facial, p = 0.410; trigeminal, p = 0.107). Hearing preservation rates were 61% at 5 years for Koos grade IV VSs and 78% for Koos grade I-III VSs (p = 0.645). Symptomatic transient tumor expansion was more common with Koos grade IV VSs (8.0% vs. 2.5%, p = 0.034), although all related symptoms diminished in accordance with tumor shrinkage. CONCLUSION: SRS may contribute to long-term tumor control and adequate neurological preservation in the treatment of Koos grade IV VSs, comparable to those in the treatment of Koos grade I‒III VSs.

Stereotactic radiosurgery for Koos grade IV vestibular schwannoma in young patients: a multi-institutional study.

PURPOSE: Surgery is the treatment of choice for large vestibular schwannomas (VS). Stereotactic radiosurgery (SRS) has been suggested as an alternative to resection in selected patients. However, the safety and efficacy of SRS in Koos grade IV patients ≤ 45 years old has not been evaluated. The aim of this study is to describe the clinical and radiological outcomes of Koos grade IV in young patient managed with a single-session SRS. METHODS: This retrospective, multicenter analysis included SRS-treated patients, ≤ 45 years old presenting with non-life threatening or incapacitating symptoms due to a Koos Grade IV VS and with follow-up ≥ 12 months. Tumor control and neurological outcomes were evaluated. RESULTS: 176 patients [median age of 36.0 (IQR 9) and median tumor volume of 9.3 cm3 (IQR 4.7)] were included. The median prescription dose was 12 Gy (IQR 0.5). Median follow-up period was 37.5 (IQR 53.5) months. The 5- and 10-year progression-free survival was 90.9% and 86.7%. Early tumor enlargement occurred in 10.9% of cases and was associated with tumor progression at the last follow-up. The probability of serviceable hearing preservation at 5- and 10-years was 56.8% and 45.2%, respectively. The probability of improvement or preservation of facial nerve function was 95.7% at 5 and 10-years. Adverse radiation effects were noted in 19.9%. New-onset hydrocephalus occurred in 4.0%. CONCLUSION: Single-session SRS is a safe and effective alternative to surgical resection in selected patients ≤ 45 years old particularly those with medical co-morbidities and those who decline resection. Longer term follow up is warranted.

Impact of vestibular nerve preservation on facial and hearing outcomes in small vestibular schwannoma surgery: a technical feasibility study.

BACKGROUND: Management of small vestibular schwannomas (VSs) remains controversial. When surgery is chosen, the preservation of facial and cochlear nerve function is a priority. In this report, we introduce and evaluate a technique to anatomically preserve the vestibular nerves to minimize manipulation and preserve the function of the facial and cochlear nerves. METHODS: The vestibular nerve preservation technique was prospectively applied to resect small VS tumors in patients with serviceable preoperative hearing (American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) class A or B). Clinical and radiological data were recorded and analyzed. RESULTS: Ten patients met the inclusion criteria. The mean (SD) age was 40.4 (12.5) years. Follow-up ranged from 6 weeks to 2 years. The maximum tumor diameter parallel to the internal auditory canal ranged from 10 to 20 mm (mean, 14.9 (3.1) mm). There were three Koos grade 3 and seven Koos grade 2 tumors. Gross total resection was achieved in all cases. Both the facial and cochlear nerves were anatomically preserved in all cases. Postoperatively, 7 patients (70%) remained in the AAO-HNS class A or B hearing category. None of the patients had new vestibular symptoms, and all had House-Brackmann grade 1 facial function. Nervus intermedius dysfunction was observed in 1 patient preoperatively, which worsened postoperatively. Two patients had new nervus intermedius symptoms postoperatively. CONCLUSION: Improvement of facial nerve and hearing outcomes is feasible through the intentional preservation of the vestibular nerves in the resection of small VSs. Longer follow-up is required to rule out tumor recurrence.

Subclassification of Koos grade 4 vestibular schwannoma: insights into tumor morphology for predicting postoperative facial nerve function.

OBJECTIVE: Koos grade 4 vestibular schwannoma (KG4VS) is a large tumor that causes brainstem displacement and is generally considered a candidate for surgery. Few studies have examined the relationship between morphological differences in KG4VS other than tumor size and postoperative facial nerve function. The authors have developed a landmark-based subclassification of KG4VS that provides insights into the morphology of this tumor and can predict the risk of facial nerve injury during microsurgery. The aims of this study were to morphologically verify the validity of this subclassification and to clarify the relationship of the position of the center of the vestibular schwannoma within the cerebellopontine angle (CPA) cistern on preoperative MR images to postoperative facial nerve function in patients who underwent microsurgical resection of a vestibular schwannoma. METHODS: In this paper, the authors classified KG4VSs into two subtypes according to the position of the center of the KG4VS within the CPA cistern relative to the perpendicular bisector of the porus acusticus internus, which was the landmark for the subclassification. KG4VSs with ventral centers to the landmark were classified as type 4V, and those with dorsal centers as type 4D. The clinical impact of this subclassification on short- and long-term postoperative facial nerve function was analyzed. RESULTS: In this study, the authors retrospectively reviewed patients with vestibular schwannoma who were treated surgically via a retrosigmoid approach between January 2010 and March 2020. Of the 107 patients with KG4VS who met the inclusion criteria, 45 (42.1%) were classified as having type 4V (KG4VSs with centers ventral to the perpendicular bisector of the porous acusticus internus) and 62 (57.9%) as having type 4D (those with centers dorsal to the perpendicular bisector). Ventral extension to the perpendicular bisector of the porus acusticus internus was significantly greater in the type 4V group than in the type 4D group (p < 0.001), although there was no significant difference in the maximal ventrodorsal diameter. The rate of preservation of favorable facial nerve function (House-Brackmann grades I and II) was significantly lower in the type 4V group than in the type 4D group in terms of both short-term (46.7% vs 85.5%, p < 0.001) and long-term (82.9% vs 96.7%, p = 0.001) outcomes. Type 4V had a significantly negative impact on short-term (OR 7.67, 95% CI 2.90-20.3; p < 0.001) and long-term (OR 6.05, 95% CI 1.04-35.0; p = 0.045) facial nerve function after surgery when age, tumor size, and presence of a fundal fluid cap were taken into account. CONCLUSIONS: The authors have delineated two different morphological subtypes of KG4VS. This subclassification could predict short- and long-term facial nerve function after microsurgical resection of KG4VS via the retrosigmoid approach. The risk of postoperative facial palsy when attempting total resection is greater for type 4V than for type 4D. This classification into types 4V and 4D could help to predict the risk of facial nerve injury and generate more individualized surgical strategies for KG4VSs with better facial nerve outcomes.

Wait-and-scan management in sporadic Koos grade 4 vestibular schwannomas: A longitudinal volumetric study.

Background: Volumetric natural history studies specifically on large vestibular schwannomas (VSs), commonly classified as Koos grade 4, are lacking. The aim of the current study is to present the volumetric tumor evolution in sporadic Koos grade 4 VSs and possible predictors for tumor growth. Methods: Volumetric tumor measurements and tumor evolution patterns from serial MRI studies were analyzed from selected consecutive patients with Koos grade 4 VS undergoing initial wait-and-scan management between January 2001 and July 2020. The significant volumetric threshold was defined as a change in volume of ≥10%. Results: Among 215 tumors with a median size (IQR) of 2.7 cm3 (1.8-4.2), 147 tumors (68%) demonstrated growth and 75 tumors (35%) demonstrated shrinkage during follow-up. Growth-free survival rates (95% CI) at 1, 2, 5, and 10 years were 55% (48-61), 36% (29-42), 29% (23-36), and 28% (21-34), respectively and did not significantly differ in tumors> 20 mm (Chi-square = .40; P-value = .53). Four tumor evolution patterns (% of total) were observed: continued growth (60); initial growth then shrinkage (7); continued shrinkage (27); and stability (5). Good hearing (adjusted HR 2.21, 95% CI 1.48-3.30; P < .001) and peritumoral edema (adjusted HR 2.22, 95% CI 1.18-4.13; P = .01) at diagnosis were significantly associated with an increased likelihood of growth. Conclusions: Koos grade 4 VSs show a wide variety in size and growth. Due to variable growth patterns, an initial wait-and-scan strategy with short scan intervals may be an acceptable option in selected tumors, if no significant clinical symptoms of mass effect that warrant treatment are present.

Stereotactic radiosurgery for Koos grade IV vestibular schwannoma: a multi-institutional study.

OBJECTIVE: Though stereotactic radiosurgery (SRS) is an established safe treatment for small- and medium-sized vestibular schwannomas (VSs), its role in the management of Koos grade IV VS is still unclear. In this retrospective multicenter study, the authors evaluated tumor control and the patient outcomes of primary, single-session SRS treatment for Koos grade IV VS. METHODS: This study included patients treated with primary, single-session SRS for Koos grade IV VS at 10 participating centers. Only those patients presenting with non-life-threatening or incapacitating symptoms and at least 12 months of clinical and neuroimaging follow-up were eligible for inclusion. Relevant data were collected, and the Kaplan-Meier method was used to perform time-dependent analysis for post-SRS tumor control, hearing preservation, and facial nerve function preservation. Univariate and multivariate analyses were performed for outcome measures using Cox regression analysis. RESULTS: Six hundred twenty-seven patients (344 females, median patient age 54 [IQR 22] years) treated with primary SRS were included in this study. The median tumor volume was 8.7 (IQR 5) cm3. Before SRS, serviceable hearing, facial nerve weakness (House-Brackmann grade > I), and trigeminal neuropathy were present in 205 (33%), 48 (7.7%), and 203 (32.4%) patients, respectively. The median prescription dose was 12 (IQR 1) Gy. At a median radiological follow-up of 38 (IQR 54) months, tumor control was achieved in 94.1% of patients. Early tumor expansion occurred in 67 (10.7%) patients and was associated with a loss of tumor control at the last follow-up (p = 0.001). Serviceable hearing preservation rates at the 5- and 10-year follow-ups were 65% and 44.6%, respectively. Gardner-Robertson class > 1 (p = 0.003) and cochlear dose ≥ 4 Gy (p = 0.02) were risk factors for hearing loss. Facial nerve function deterioration occurred in 19 (3.0%) patients at the last follow-up and was associated with margin doses ≥ 13 Gy (p = 0.03) and early tumor expansion (p = 0.04). Post-SRS, 33 patients developed hydrocephalus requiring shunting. Adverse radiation effects occurred in 92 patients and were managed medically or surgically in 34 and 18 cases, respectively. CONCLUSIONS: SRS is a safe and effective method of obtaining tumor control in patients with Koos grade IV VS presenting with non-life-threatening or debilitating symptoms, especially those with surgical comorbidities that contraindicate resection. To decrease the incidence of post-SRS facial palsy, a prescription dose < 13 Gy is recommended.

Long-term tumor control in Koos grade IV vestibular schwannomas without the need for gross-total resection.

OBJECTIVE: The modern management of patients with Koos grade IV vestibular schwannomas (VSs) aims at functional preservation and long-term tumor control. Gross-total resection (GTR) leads to optimal tumor control but frequently also results in permanent facial nerve (FN) palsy. Subtotal resection (STR) or near-total resection (NTR) followed by a wait-and-scan protocol and second-line radiation therapy (RT) in case of progressive residuals yields excellent tumor control rates with less permanent morbidity. METHODS: The authors present the results of their prospective cohort of Koos grade IV VS patients who underwent less-than-total resection followed by a wait-and-scan protocol between January 2009 and December 2019 and discuss the latest evidence on this controversial subject. The cohort was followed up with annual clinical and volumetric outcome analyses after standardized MRI. RESULTS: Forty-eight patients were included in the analysis. The mean extent of resection was 87% (median 91%, range 45%-100%), best fitting into the definition of STR rather than NTR. In 2 cases, the proximal portion of the FN at the brainstem could not be reliably identified and monitored during the initial operation, and a second-stage resection was necessary. At 4.4 years after surgery, 81% (39/48) of the tumor residuals regressed or were stable in size. The percentage of regressive tumor residuals increased over time. Nineteen percent (9/48) of the tumor residuals displayed volumetric progression within a mean time of 35 months (median 36 months, range 14-72 months), resulting in a Kaplan-Meier estimate for progression-free survival of 79% after 4 years; higher postoperative volume showed a linear correlation with higher volumetric progression (factor 1.96, 95% CI 1.67-2.30; p < 0.001). Thirty-four of the 48 (71%) patients continue to undergo a wait-and-scan protocol. Second-line RT was performed in 14 patients (29%) within a mean time of 25 months (median 23 months, range 5-54 months), 12 (86%) of whom responded with post-RT pseudoprogression, resulting in an overall tumor control rate of 96%. At the 4.4-year follow-up from the initial resection, 92% of the patients had a good facial outcome (House-Brackmann [HB] grade I or II), 6% had a fair facial outcome (HB grade III), and 2% had a poor facial outcome (HB grades IV-VI). So far, there has been no need for salvage surgery after RT. CONCLUSIONS: STR followed by observation and second-line RT in cases of progression leads to good facial outcome and an excellent tumor control rate in the longer term.

The longitudinal volumetric response of vestibular schwannomas after Gamma Knife radiosurgery.

OBJECTIVE: Gamma Knife radiosurgery (GKRS) is an effective treatment for vestibular schwannomas (VSs) and has been used in > 100,000 cases worldwide. In the present study the authors sought to define the serial volumetric tumor response of Koos grade I-IV VS after radiosurgery. METHODS: A total of 201 consecutive VS patients underwent GKRS at a single institution between 2015 and 2019. All patients had a minimum follow-up of 18 months and at least 2 interval postprocedure MRI scans. The contrast-enhanced tumor volumes were contoured manually and compared between pre- and post-GKRS imaging. The percentages of tumor volume change at 18 months (short-term follow-up) and up to 5 years after GKRS (long-term follow-up) were compared with the baseline tumor volume. An increase of 20% was considered a significant increase of tumor volume. Trends of tumor volume over time were assessed with linear models using time as a continuous variable. A test for linear trend was evaluated according to the initial Koos tumor classification. RESULTS: Koos grade II VS was the most frequently occurring tumor (n = 74, 36.8%), followed by grade III (n = 57, 28.4%), grade I (n = 41, 20.4%), and grade IV (n = 29, 14.4%). The mean tumor volume at the time of GKRS was 2.12 ± 2.82 cm3 (range 0.12-18.77 cm3) and the median margin dose was 12 Gy. Short-term follow-up revealed that tumor volumes transiently increased in 34.2% and 28.4% of patients at 6 and 18 months, respectively, regardless of Koos grade. Linear regression analysis of Koos grade II, III, and IV tumors showed a significant longitudinal volume decrease on long-term follow-up. At last follow-up (median 30 months, range 18-54 months), 19 patients (9.4%) showed a persistent increase of tumor volume. Five patients received additional management after GKRS. CONCLUSIONS: Although selected VS patients demonstrate an early and measurable transient volumetric increase after GKRS, > 90% have stable or reduced tumor volumes over an observed period of up to 5 years. Volumetric regression is most pronounced in Koos grade II, III, and IV tumors and may not be fully detectable until 3 years after GKRS.

Stereotactic radiosurgery as the first-line treatment for intracanalicular vestibular schwannomas.

OBJECTIVE: This report evaluates the outcomes of stereotactic radiosurgery (SRS) as the first-line treatment of intracanalicular vestibular schwannomas (VSs). METHODS: Between 1987 and 2017, the authors identified 209 patients who underwent SRS as the primary intervention for a unilateral intracanalicular VS. The median patient age was 54 years (range 22-85 years); 94 patients were male and 115 were female. Three patients had facial neuropathy at the time of SRS. One hundred fifty-five patients (74%) had serviceable hearing (Gardner-Robertson [GR] grades I and II) at the time of SRS. The median tumor volume was 0.17 cm3 (range 0.015-0.63 cm3). The median margin dose was 12.5 Gy (range 11.0-25.0 Gy). The median maximum dose was 24.0 Gy (range 15.7-50.0 Gy). RESULTS: The progression-free survival rates of all patients with intracanalicular VS were 97.5% at 3 years, 95.6% at 5 years, and 92.1% at 10 years. The rates of freedom from the need for any additional intervention were 99.4% at 3 years, 98.3% at 5 years, and 98.3% at 10 years. The serviceable hearing preservation rates in GR grade I and II patients at the time of SRS were 76.6% at 3 years, 63.5% at 5 years, and 27.3% at 10 years. In univariate analysis, younger age (< 55 years, p = 0.011), better initial hearing (GR grade I, p < 0.001), and smaller tumor volumes (< 0.14 cm3, p = 0.016) were significantly associated with improved hearing preservation. In multivariate analysis, better hearing (GR grade I, p = 0.001, HR 2.869, 95% CI 1.569-5.248) and smaller tumor volumes (< 0.14 cm3, p = 0.033, HR 2.071, 95% CI 1.059-4.047) at the time of SRS were significantly associated with improved hearing preservation. The hearing preservation rates of patients with GR grade I VS were 88.1% at 3 years, 77.9% at 5 years, and 38.1% at 10 years. The hearing preservation rates of patients with VSs smaller than 0.14 cm3 were 85.5% at 3 years, 77.7% at 5 years, and 42.6% at 10 years. Facial neuropathy developed in 1.4% from 6 to 156 months after SRS. CONCLUSIONS: SRS provided sustained tumor control in more than 90% of patients with intracanalicular VS at 10 years and freedom from the need for additional intervention in more than 98% at 10 years. Patients with initially better hearing and smaller VSs had enhanced serviceable hearing preservation during an observation interval up to 10 years after SRS.

Stereotactic radiosurgery as the primary management for patients with Koos grade IV vestibular schwannomas.

OBJECTIVE: While extensive long-term outcome studies support the role of stereotactic radiosurgery (SRS) for smaller-volume vestibular schwannomas (VSs), its role in the management for larger-volume tumors remains controversial. METHODS: Between 1987 and 2017, the authors performed single-session SRS on 170 patients with previously untreated Koos grade IV VSs (volumes ranged from 5 to 20 cm3). The median tumor volume was 7.4 cm3. The median maximum extracanalicular tumor diameter was 27.5 mm. All tumors compressed the middle cerebellar peduncle and distorted the fourth ventricle. Ninety-three patients were male, 77 were female, and the median age was 61 years. Sixty-two patients had serviceable hearing (Gardner-Robertson [GR] grades I and II). The median margin dose was 12.5 Gy. RESULTS: At a median follow-up of 5.1 years, the progression-free survival rates of VSs treated with a margin dose ≥ 12.0 Gy were 98.4% at 3 years, 95.3% at 5 years, and 90.7% at 10 years. In contrast, the tumor control rate after delivery of a margin dose < 12.0 Gy was 76.9% at 3, 5, and 10 years. The hearing preservation rates in patients with serviceable hearing at the time of SRS were 58.1% at 3 years, 50.3% at 5 years, and 35.9% at 7 years. Younger age (< 60 years, p = 0.036) and initial GR grade I (p = 0.006) were associated with improved serviceable hearing preservation rate. Seven patients (4%) developed facial neuropathy during the follow-up interval. A smaller tumor volume (< 10 cm3, p = 0.002) and a lower margin dose (≤ 13.0 Gy, p < 0.001) were associated with preservation of facial nerve function. The probability of delayed facial neuropathy when the margin dose was ≤ 13.0 Gy was 1.1% at 10 years. Nine patients (5%) required a ventriculoperitoneal shunt because of delayed symptomatic hydrocephalus. Fifteen patients (9%) developed detectable trigeminal neuropathy. Delayed resection was performed in 4% of patients. CONCLUSIONS: Even for larger-volume VSs, single-session SRS prevented the need for delayed resection in almost 90% at 10 years. For patients with minimal symptoms of tumor mass effect, SRS should be considered an effective alternative to surgery in most patients, especially those with advanced age or medical comorbidities.

Intended Near-Total Removal of Koos Grade IV Vestibular Schwannomas: Reconsidering the Treatment Paradigm.

BACKGROUND: The goals of treating Koos grade IV vestibular schwannomas are to relieve brainstem compression, preserve or restore neurological function, and achieve long-term tumor control while minimizing tumor- and treatment-related morbidity. OBJECTIVE: To propose a treatment paradigm involving the intentional near-total removal of Koos grade IV vestibular schwannomas, in which a small amount of residual tumor is not dissected off the cisternal portion of the facial nerve. Patients are then followed by a wait-and-scan approach. Any subsequent volumetric progression of the residual tumor is treated with radiosurgery. METHODS: This is a case series of 44 consecutive unselected patients who underwent intended near-total resection of a Koos grade IV vestibular schwannoma through a retrosigmoid approach from January 2009 to December 2015. Pre- and postoperative volumetric analyses were performed on routine magnetic resonance imaging sequences (constructive interference in steady state and gadolinium-enhanced T1-weighted sequence). RESULTS: The mean preoperative tumor volume was 10.9 cm3. The mean extent of resection was 89%. At the last clinical follow-up, facial nerve function was good [House and Brackmann (HB) I-II] in 89%, fair (HB III) in 9%, and poor (HB IV-VI) in 2% of the patients. At the last radiological follow-up, the residual tumor had become smaller or remained the same size in 84% of patients. Volumetric progression was negatively correlated with the original extent of resection and positively correlated with postoperative residual tumor volume (P = .01, P < .001, respectively). CONCLUSION: Intended near-total removal results in excellent preservation of facial nerve function and has a low recurrence rate. Any progressive residual tumor may be treated by radiosurgery.

Long-term outcome after Gamma Knife radiosurgery for acoustic neuroma of all Koos grades: a single-center study.

OBJECTIVE The authors present long-term follow-up data on patients treated with Gamma Knife radiosurgery (GKRS) for acoustic neuroma. METHODS Six hundred eighteen patients were radiosurgically treated for acoustic neuroma between 1992 and 2016 at the Department of Neurosurgery, Medical University Vienna. Patients with neurofibromatosis and patients treated too recently to attain 1 year of follow-up were excluded from this retrospective study. Thus, data on 557 patients with spontaneous acoustic neuroma of any Koos grade are presented, as are long-term follow-up data on 426 patients with a minimum follow-up of 2 years. Patients were assessed according to the Gardner-Robertson (GR) hearing scale and the House-Brackmann facial nerve function scale, both prior to GKRS and at the times of follow-up. RESULTS Four hundred fifty-two patients (81%) were treated with radiosurgery alone and 105 patients (19%) with combined microsurgery-radiosurgery. While the combined treatment was especially favored before 2002, the percentage of cases treated with radiosurgery alone has significantly increased since then. The overall complication rate after GKRS was low and has declined significantly in the last decade. The risk of developing hydrocephalus after GKRS increased with tumor size. One case (0.2%) of malignant transformation after GKRS was diagnosed. Radiological tumor control rates of 92%, 91%, and 91% at 5, 10, and 15 years after GKRS, regardless of the Koos grade or pretreatment, were observed. The overall tumor control rate without the need for additional treatment was even higher at 98%. At the last follow-up, functional hearing was preserved in 55% of patients who had been classified with GR hearing class I or II prior to GKRS. Hearing preservation rates of 53%, 34%, and 34% at 5, 10, and 15 years after GKRS were observed. The multivariate regression model revealed that the GR hearing class prior to GKRS and the median dose to the cochlea were independent predictors of the GR class at follow-up. CONCLUSIONS In small to medium-sized spontaneous acoustic neuromas, radiosurgery should be recognized as the primary treatment at an early stage. Although minimizing the cochlear dose seems beneficial for hearing preservation, the authors, like others before, do not recommend undertreating intracanalicular tumors in favor of low cochlear doses. For larger acoustic neuromas, radiosurgery remains a reliable management option with tumor control rates similar to those for smaller acoustic neuromas; however, careful patient selection and counseling are recommended given the higher risk of side effects. Microsurgery must be considered in acoustic neuromas with significant brainstem compression or hydrocephalus.