Recurrent Vestibular Schwannoma Research Articles

Surgical salvage for recurrent vestibular schwannoma after primary stereotactic radiosurgery

Background: The management of vestibular schwannoma has evolved over the past hundred years. In the last decades, surgery has been gradually replaced by radiation therapy as a primary treatment modality, particularly for small tumors, due to the less invasive nature and the compared reported outcomes in tumor control and hearing preservation. However, irradiation sometimes fails to stop tumor growth. In a long-term follow-up after primary fractionated stereotactic radiotherapy, the rate of treatment failure was reported as 3% and needed surgical salvage. For single-fraction modality, Hasegawa et al. reported salvage treatment after primary Gamma Knife radiosurgery in 8%, where 90% of these underwent surgery and 50% of those who were treated with a second gamma knife surgery required surgical intervention later. An increase in tumor volume by more than 10–20%, tumor growth after three years, and no return to pretreatment volume after transient swelling have been considered as tumor recurrence rather than pseudoprogression, a transient increase in tumor volume after radiotherapy that occurs up to 30% of cases. It has been reported that microsurgery after radiotherapy is more difficult, with most authors reporting a loss of defined arachnoid planes and worse cranial nerve outcomes, especially for hearing and facial nerve function. Case Description: A 43-year-old female patient was incidentally (asymptomatic) diagnosed on a magnetic resonance imaging (MRI) scan harboring a left vestibular schwannoma, grade T2 (Hannover classification), in 2015. Neurologic examination was unremarkable, and audiometry testing was normal. She was initially treated with observation. Three years later, in 2018, the lesion had enlarged, becoming a grade T3a and reaching the cistern of the cerebellopontine angle. The tumor was then treated with fractionated stereotactic radiosurgery (5 sessions of 5 Gy). MRI scans in 2019 and 2020 showed slight tumor growth. This enlargement was attributed to a pseudoprogression after radiosurgery, and only observation was advocated. In 2022, 4 years later, after radiosurgery, the tumor was still growing, and the patient began to suffer from hearing loss. A failure treatment was considered, and microsurgery was indicated. The patient was counseled about the risk of functional nerve impairment, and surgical consent was obtained. A retro sigmoid approach was planned. A gross total resection was attempted due to the clear subperineural plane during tumor dissection and because it was the only option that would provide a cure for the patient. The adjacent neurovascular structures were firmly adhered to the tumor capsule, which represented a major challenge for microdissection. The tumor was soft, without significant bleeding. A total resection was achieved, and the facial nerve was anatomically preserved. The patient developed facial paresis (House-Brackmann III) in the immediate postoperative period, which improved at the 6-month follow-up. Hearing loss did not improve. Postoperative MRI showed total resection. Conclusion: Microsurgery after radiotherapy for vestibular schwannoma is challenging in terms of indication, when to indicate, resection target, difficulty in dissection due to local changes, and outcome. Gross total resection may be considered, as it is the only treatment that may provide a cure for the patient. However, the patient should be counseled about the risks.

Repeat single-fraction stereotactic radiosurgery for recurrent vestibular schwannoma.

BackgroundData are scarce on the efficacy of a second radiosurgery (SRS) treatment of vestibular schwannoma that has progressed following initial treatment with SRS. We sought to report the outcome of our repeat SRS series with long-term imaging follow-up.Materials and methodsWe retrospectively analyzed 6 patients who met the following criteria: Repeat SRS at our institution between 1995 and 2018; solitary unilateral tumor; no evidence of neurofibromatosis; and magnetic resonance (MR) planning for both SRS treatments. All treatments were delivered with a linear accelerator-based system using head frame immobilization. The prescribed dose to the periphery of the tumor was 12.5 Gy in all initial and repeat SRS treatments, except for one repeat treatment to 10 Gy.ResultsFollow-up with MR scan following the second SRS treatment was a median 8.4 years. The tumor control rate (lack of progression) following the second SRS treatment was 83% (5/6). Actuarial 10-year outcomes following repeat SRS were: tumor control, 80%; absolute survival, 80%; and cause-specific survival, 100%. Of the patients with at least minimal hearing retention before initial SRS, none had ipsilateral hearing preservation after initial radiation treatment. Improvement in any pretreatment cranial nerve deficits was not seen. The only permanent grade ≥ 3 toxicity from repeat SRS was a case of infraorbital nerve deficit. No patient developed a stroke, malignant transformation, induced second tumor, or facial nerve deficit.ConclusionThere was excellent overall survival, tumor control, and low morbidity in our series for recurrent vestibular schwannoma submitted to repeat single-fraction SRS, supporting additional studies of this treatment strategy.

Fate of Residual Tumor After Subtotal Resection of a Previously Irradiated Vestibular Schwannoma: Long-Term Follow-Up of a Single-Institutional Series

The fate of residual tumor after salvage surgery for recurrent vestibular schwannoma (VS) after radiosurgery has not been elucidated so far. We reviewed our surgical series of salvage surgery for recurrent VS, with focus on the natural history of the residual tumor after salvage surgery. This study enrolled 14 patients who underwent salvage surgical resection in our institute and were followed up for >12months. The study included 3 men and 11 women with a median age of 55years (range: 16-70years). The median pre-stereotactic radiosurgery tumor volume was 6591mm3. All patients were treated using gamma knife radiosurgery. The median duration from gamma knife radiosurgery to surgery was 52months (range: 10-116months). Solid tumor growth and cyst formation were observed in 6 (42.9%) and 8 (57.1%) patients, respectively. Subtotal resection and partial resection were performed in 13 (92.8%) patients, and gross total resection was achieved in only one (7.2%) patient. Postoperative facial paresis and surgical complication occurred in 5 (35.7%) and 2 (14.3%) patients, respectively. After salvage resection for irradiated VS, no patient showed tumor progression or recurrence during the follow-up period (13 subtotal/partial resections and 1 total resection). In addition, 2 patients in the subtotal resection group showed residual tumor shrinkage after salvage surgery during the follow-up period. The behavior of residual tumors after salvage surgery for irradiated VS was stable. Adjuvant treatment for these residual tumors may not be necessary.

Collagen Family Genes Associated with Risk of Recurrence after Radiation Therapy for Vestibular Schwannoma and Pan-Cancer Analysis.

Background The safety of radiotherapy techniques in the treatment of vestibular schwannoma (VS) shows a high rate of tumor control with few side effects. Neuropeptide Y (NPY) may have a potential relevance to the recurrence of VS. Further research is still needed on the key genes that determine the sensitivity of VS to radiation therapy. Materials and Methods Transcriptional microarray data and clinical information data from VS patients were downloaded from GSE141801, and vascular-related genes associated with recurrence after radiation therapy for VS were obtained by combining information from MSigDB. Logistics regression was applied to construct a column line graph prediction model for recurrence status after radiation therapy. Pan-cancer analysis was also performed to investigate the cooccurrence of these genes in tumorigenesis. Results We identified eight VS recurrence-related genes from the GSE141801 dataset. All of these genes were highly expressed in the VS recurrence samples. Four collagen family genes (COL5A1, COL3A1, COL4A1, and COL15A1) were further screened, and a model was constructed to predict the risk of recurrence of VS. Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) enrichment analyses revealed that these four collagen family genes play important roles in a variety of biological functions and cellular pathways. Pan-cancer analysis further revealed that the expression of these genes was significantly heterogeneous across immune phenotypes and significantly associated with immune infiltration. Finally, Neuropeptide Y (NPY) was found to be significantly and negatively correlated with the expression of COL5A1, COL3A1, and COL4A1. Conclusions Four collagen family genes have been identified as possible predictors of recurrence after radiation therapy for VS. Pan-cancer analysis reveals potential associations between the pathogenesis of VS and other tumorigenic factors. The relevance of NPY to VS was also revealed for the first time.

Progressive Vestibular Schwannoma following Subtotal or Near-Total Resection: Dose-Escalated versus Standard-Dose Salvage Stereotactic Radiosurgery.

Objective Local failure of incompletely resected vestibular schwannoma (VS) following salvage stereotactic radiosurgery (SRS) using standard doses of 12 to 13 Gy is common. We hypothesized that dose-escalated SRS, corrected for biologically effective dose, would have superior local control of high-grade VS progressing after subtotal or near-total resection compared with standard-dose SRS. Design Retrospective cohort study. Setting Tertiary academic referral center. Participants Adult patients treated with linear accelerator-based SRS for progressive VS following subtotal or near-total resection. Main Outcome Measures Dose-escalated SRS was defined by a biologically effective dose exceeding a single-fraction 13-Gy regimen. Study outcomes were local control and neurologic sequelae of SRS. Binary logistic regression was used to evaluate predictors of study outcomes. Results A total of 18 patients with progressive disease following subtotal (71%) and near-total (39%) resection of Koos grade IV disease (94%) were enrolled. Of the 18 patients, 7 were treated with dose-escalated SRS and 11 with standard-dose SRS. Over a median follow-up of 32 months after SRS, local control was 100% in the dose-escalated cohort and 91% in the standard-dose cohort ( p = 0.95). Neurologic sequelae occurred in 28% of patients, including 60% of dose-escalated cohort and 40% of the standard-dose cohort ( p = 0.12), although permanent neurologic sequelae were low at 6%. Conclusions Dose-escalated SRS has similar local control of recurrent VS following progression after subtotal or near-total resection and does not appear to have higher neurologic sequalae. Larger studies are needed.

Prediction of vestibular schwannoma recurrence using artificial neural network.

ObjectivesTo compare two statistical models, namely logistic regression and artificial neural network (ANN), in prediction of vestibular schwannoma (VS) recurrence.MethodsSeven hundred eighty‐nine patients with VS diagnosis completed an online survey. Potential predictors for recurrence were derived from univariate analysis by reaching the cut off P value of .05. Those nine potential predictors were years since treatment, surgeon's specialty, resection amount, and having incomplete eye closure, dry eye, double vision, facial pain, seizure, and voice/swallowing problem as a complication following treatment. Multivariate binary logistic regression model was compared with a four‐layer 9‐5‐10‐1 feedforward backpropagation ANN for prediction of recurrence.ResultsThe overall recurrence rate was 14.5%. Significant predictors of recurrence in the regression model were years since treatment and resection amount (both P < .001). The regression model did not show an acceptable performance (area under the curve [AUC] = 0.64; P = .27). The regression model's sensitivity and specificity were 44% and 69%, respectively and correctly classified 56% of cases. The ANN showed a superior performance compared to the regression model (AUC = 0.79; P = .001) with higher sensitivity (61%) and specificity (81%), and correctly classified 70% of cases.ConclusionThe constructed ANN model was superior to logistic regression in predicting patient‐answered VS recurrence in an anonymous survey with higher sensitivity and specificity. Since artificial intelligence tools such as neural networks can have higher predictive abilities compared to logistic regression models, continuous investigation into their utility as complementary clinical tools in predicting certain surgical outcomes is warranted.

Contribution of mTOR and PTEN to Radioresistance in Sporadic and NF2-Associated Vestibular Schwannomas: A Microarray and Pathway Analysis.

The use of radiation treatment has increased for both sporadic and neurofibromatosis type 2 (NF2)-associated vestibular schwannoma (VS). However, there are a subset of radioresistant tumors and systemic treatments that are seldom used in these patients. We investigated molecular alterations after radiation in three NF2-associated and five sporadically operated recurrent VS after primary irradiation. We compared these findings with 49 non-irradiated (36 sporadic and 13 NF2-associated) VS through gene-expression profiling and pathway analysis. Furthermore, we stained the key molecules of the distinct pathway by immunohistochemistry. A total of 195 differentially expressed genes in sporadic and NF2-related comparisons showed significant differences based on the criteria of p value < 0.05 and a two-fold change. These genes were involved in pathways that are known to be altered upon irradiation (e.g., mammalian target of rapamycin (mTOR), phosphatase and tensin homolog (PTEN) and vascular endothelial growth factor (VEGF) signaling). We observed a combined downregulation of PTEN signaling and an upregulation of mTOR signaling in progressive NF2-associated VS after irradiation. Immunostainings with mTOR and PTEN antibodies confirmed the respective molecular alterations. Taken together, mTOR inhibition might be a promising therapeutic strategy in NF2-associated VS progress after irradiation.

A rare case of recurrent Vestibular Schwannoma

A vestibular Schwannoma is a non-cancerous primary intracranial tumor of the myelin-forming cells of the vestibulo-cochlear nerve 8th cranial nerve. It grows slowly or not at all. Rarely it may grow rapidly and become large enough to press against the brain and interfere with the vital functions. Treatment includes regular monitoring radiation and surgical removal. Here we report a rare case of recurrent vestibular Schwannoma which recurred after complete removal of tumor.

Multiplatform Molecular Profiling of Vestibular Schwannoma Reveals Immune Enrichment after Radiosurgery and a Methylation-Based Predictor of Recurrence

Vestibular schwannomas (VS) can cause significant morbidity from cranial nerve deficits and mass effect. Broad molecular differences have been described for intracranial versus spinal axis schwannomas, but little is known about the molecular heterogeneity of VS or predictors of VS recurrence. The objective of this study was to understand VS biology and identify molecular predictors of VS recurrence. Sixty-six sporadic VS from 59 consecutive patients with available tissue who underwent initial resection (44), resection for recurrence after prior surgery (5), resection for recurrence after prior stereotactic radiosurgery (SRS) (11), or resection after both prior surgery and prior SRS (6) at a single institution from 2003-2017 were profiled using Illumina 850K DNA methylation arrays (median follow-up: 4.2 years, median volumetric resection: 91%). Twenty-four VS were further characterized using RNA sequencing (RNA-seq), and 7 were analyzed by single nuclei RNA-seq. Molecular subtyping was performed by hierarchical clustering of differentially-methylated DNA probes and validated using transcriptomic data. In vitro functional experiments were performed using human VS cells. Clustering of genome-wide DNA methylation probes revealed that VS are comprised of 2 subgroups that are delineated by enrichment of neural crest or immune genes. Fifteen of 35 immune-enriched VS (43%) had prior SRS, while only 2 of 31 neural crest-enriched VS (6%) had prior SRS (p<0.001). Analysis of 6 patients with matched primary and recurrent VS samples revealed that all recurrent VS after prior SRS exhibited similar methylation profiles and were enriched in immune genes. In contrast, the methylation profile of VS that recurred after prior surgery did not deviate from the primary tumor. Hypomethylation of G protein-coupled estrogen receptor 1 (GPER) promoter was prognostic for local failure in multivariate regression irrespective of primary treatment modality (p<0.0001). Radiation of VS cells in vitro recapitulated gene expression of immune-enriched VS. VS are comprised of 2 molecular subgroups characterized by differential enrichment of neural crest or immune genes. VS with prior SRS are associated with immune-enrichment. GPER promoter hypomethylation is prognostic for VS recurrence. These data illuminate the biology of VS and shed light on potential molecular therapies.

Protein profiling of cerebrospinal fluid from patients undergoing vestibular schwannoma surgery and clinical significance

Vestibular schwannoma (VS) is a common disease in the region of the cerebellopontine angle in the posterior cranial fossa. Large VS and its surgical management usually lead to severe cranial nerve dysfunction and affect the patient’s quality of life. We aimed to find some possible progression markers of VS. Here, we sought to characterize the cerebrospinal fluid (CSF) proteome of patients with different VS grades and recurrence to identify biomarkers predictive of VS growth or recurrence. CSF was collected intraoperatively prior to removal of untreated VS, including grade I–V and recurrence. Isobaric tags for relative and absolute quantitation–based proteomic analysis of CSF from 43 VS patients and 3 control patients was used to identify candidate proteins. Ninety-three overlapping proteins were found to display differential expression in grade I, II, III, IV, and V VS patients compared with the control group. Nine proteins were chosen for validation with enzyme-linked immunosorbent assay. VS was distinguished from control patients based on the expression patterns of six proteins (ATP-binding cassette subfamily A member 3 [ABCA3], secretogranin-1 [SCG1], Krueppel-like factor 11 [KLF11], voltage-dependent calcium channel subunit alpha-2/delta-1 [CA2D1], brain acid soluble protein 1 [BASP1], and peroxiredoxin-2 [PRDX2]. ABCA3 and KLF11 were positively correlated with the size of early-phase of VS, while BASP1 and PRDX2 showed a negative correlation. ABCA3, CA2D1, and KLF11 were upregulated, while BASP1 and PRDX2 were downregulated in the CSF from VS recurrence. But SCG1 was increased only at early-phase. These data suggest that increased ABCA3 and KLF11 and decreased BASP1 and PRDX2 in CSF are associated with VS growth at the early phase or recurrence.

Local Tumor Control and Clinical Symptoms After Gamma Knife Radiosurgery for Residual and Recurrent Vestibular Schwannomas

The use of Gamma Knife radiosurgery (GKRS) for recurrent or residual vestibular schwannoma (VS) after microsurgery (MS) has been investigated in several retrospective studies. The purpose of this study was to identify potential risk factors for both neurologic deterioration and tumor progression after GKRS for previously operated VSs in a prospective setting. Patients who underwent GKRS for previously operated and histopathologically confirmed VS between 1998 and 2015 were prospectively followed-up. Risk factors for therapy side effects and predictors for tumor control were investigated in uni- and multivariate analyses. A total of 160 individuals with a median age of 55 years were included. Median tumor volume prior to GKRS was 1.40 cm3 (range, 0.06-35.80 cm3). After a median follow-up of 36 months, hearing and facial nerve function were serviceable (modified Gardner-Robertson and House-Brackmann grades I and II) in 7 (5%) and 82 (55%) patients, respectively. Deterioration to a nonserviceable facial nerve function after GKRS was found in 3% (3/89) and tended to increase with rising tumor volume (odds ratio, 1.65 per cm3; 95% confidence interval, 1.00-2.71; P= 0.051). Median tumor volume prior to GKRS was higher in patients with radiologic (P= 0.020) or clinical tumor progression (P<0.001). Critical tumor volume prior to GKRS to predict clinical and radiologic tumor progression was 1.30 cm3 (P< 0.001) and 3.30 cm3 (P= 0.019), respectively. However, in multivariate analyses, none of the analyzed variables were found to independently predict tumor progression. Intended submaximal resection followed by GKRS is a viable treatment for VS. Because tumor remnant size after MS is an important predictor for recurrence after adjuvant GKRS, both brainstem and cerebellar decompression and maximal safely achievable resection should remain major goals of microsurgery.

Progression of vestibular schawnnoma after GammaKnife radiosurgery: A challenge for microsurgical resection

ObjectiveWe aimed to evaluate the outcome of patients who underwent salvage microsurgery for vestibular schwannoma (VS) that failed primary Gammaknife radiosurgery (GKS). Patients and MethodsAmong the 1098 patients who received GKS for the treatment of VS in our center between January 2004 and December 2012, the follow-up was organized in our institution for 290 patients who lived in our recruitment area. Tumor progression was noted in 23 patients. A salvage microsurgical resection was performed in 11 patients, who were included in our study. Grading of facial function was done according to the House & Brackman scale. ResultsThe mean age at diagnosis was 50.2 years (19–68 years) and the mean follow-up was 9.4 years (4–13 years). The mean dose was 11.8 Gy (11–12 Gy) and the mean volume was 922 mm3 (208–2500 mm3). The mean period between GKS and diagnosis of tumor progression was 32 months (18–72 months). Concerning salvage microsurgery, complete resection was obtained in 8 patients. Small residual tumor on the facial nerve was deliberately left in 3 patients and no tumor progression was noted with a mean follow-up of 26 months. At last follow-up, facial nerve function was grade 1 in 4 patients, grade 2 in 3 patients, grade 3 in 1 patient and grade 4 in 3 patients. ConclusionSalvage surgery of recurrent vestibular schwannoma after failed initial GKS remains a good treatment. However, facial nerve preservation is more challenging in this case and small tumor remnant could be sometimes deliberately left.

Utility of Noncontrast Magnetic Resonance Imaging for Detection of Recurrent Vestibular Schwannoma.

Gadolinium-enhanced T1-weighted magnetic resonance imaging (MRI) (T1WI) is the conventional imaging technique of choice to detect vestibular schwannoma (VS) recurrence or regrowth, despite suboptimal specificity secondary to enhancing postoperative changes. Furthermore, recent concerns regarding the accumulation of gadolinium in body tissues have led for a call to reduce the number of contrast-enhanced examinations. The objective of the current study is to evaluate the diagnostic accuracy of high-resolution noncontrast (three-dimensional [3D] T2) MRI relative to gadolinium-enhanced T1WI in the detection of VS recurrence after resection. Following Institutional Review Board approval, 13 consecutive postoperative patients with VS recurrence or regrowth were identified from a prospectively maintained clinical database in which recurrence was determined by progression on serial postoperative MRI examination. Three blinded neuroradiologists retrospectively evaluated a total of 41 postoperative MRI examinations from these patients using only gadolinium-enhanced T1WI and 3D T2 MRI for recurrence or regrowth. Interobserver agreement, differences in detection between the two sequences, and the sensitivity and specificity of 3D T2 MRI were assessed. Fifteen of the 41 postoperative MRIs demonstrated progression, as determined by examiner consensus on the gadolinium-enhanced T1WI. Agreement, measured using Krippendorff's alpha, was 0.82 for the 3D T2 images and 0.83 for the contrast-enhanced T1WI. All the three examiners demonstrated no difference in the detection of progression between the two sequences (McNemar's test p values 0.69 for examiner 1, 0.63 for examiner 2, and 0.99 for examiner 3). The sensitivity of 3D T2 was 0.78 (CI 0.60-0.96), while the specificity was 0.94 (CI 0.86-1.00). Noncontrast high-resolution 3D T2 MRI seems sufficient to assess for recurrence or regrowth after VS resection. The results of this study have implications for reducing cost, time, and adverse events associated with gadolinium administration in this population requiring serial follow-up examinations. These promising, yet preliminary findings warrant confirmation with a larger prospective cohort of patients.

Utility of Noncontrast Magnetic Resonance Imaging for Detection of Recurrent Vestibular Schwannoma

Utility of Noncontrast Magnetic Resonance Imaging for Detection of Recurrent Vestibular Schwannoma

Microsurgery for Recurrent Vestibular Schwannoma after Previous Gross Total Resection

Microsurgery for Recurrent Vestibular Schwannoma after Previous Gross Total Resection

Trapped Fourth Ventricle in Recurrent Acoustic Schwannoma - A Rarity

AbstractFourth ventricular dilatation is usually seen along with tri-ventriculomegaly in patients with communicating hydrocephalus. Isolated fourth ventricular dilatation is uncommon, especially as a sequelae following infective or post hemorrhagic communicating hydrocephalus. Communicating hydrocephalus is reported in vestibular schwannoma with an incidence of 3.7 to 23.5%, but 4th ventricular dilatation following its treatment has not been reported in the literature. We report a novel case of isolated fourth ventricular obstruction following surgery for recurrent vestibular schwannoma and ventriculoperitoneal shunt placement for communicating hydrocephalus. Management strategies range from endoscopic procedures to ventricular shunt placement. We describe the surgical technique for the placement of a fourth ventricular shunt with the use of a Y connector.

再発を繰り返す難治性聴神経鞘腫に対する手術戦略

再発を繰り返す難治性聴神経鞘腫に対する手術戦略

Gamma Knife Radiosurgery for Residual and Recurrent Vestibular Schwannomas After Previous Surgery: Clinical Results in a Series of 90 Patients and Review of the Literature

Complete removal of vestibular schwannomas (VS) is not always achievable without any risk of disabling postoperative complications, especially in terms of facial nerve function. Moreover, even after gross total removal, a relevant rate of recurrence has been reported. The aim of this study is to validate Gamma Knife radiosurgery (GKRS) as an effective strategy to treat tumor regrowth after previous surgery. Ninety patients treated with GKRS for VS after previous microsurgery were included in the present study. GKRS was performed at a median of 31 months (range, 4-174 months) postoperatively. Mean tumor volume was 3.35 cm3 (median, 2.5 cm3; range, 0.027-13 cm3) and median marginal dose was 13 Gy. At a mean follow-up of 77.2 months, tumor control was achieved in 90% of patients: 2 patients underwent repeated GKRS, and 7 patients underwent further microsurgery. Tumor shrinkage at last follow-up was recorded in 80.3% of cases. The complication rate was low and many consisted of a transient worsening of preexisting symptoms. The overall incidence of persisting facial nerve deficit and trigeminal nerve impairment was, in both cases, 3.3%. Two of 5 patients (40%) preserved functional hearing at last follow-up. One patient (1.1%) underwent ventriculoperitoneal shunting 12 months after GKRS. GKRS is a safe and effective treatment for growing residual and recurrent VSs, with tumor control obtained in 90% of cases and a low morbidity rate. Moreover, the possibility of treating patients with major medical comorbidities constitutes a significant advantage over repeated surgery.

Malignant Transformation of Vestibular Schwannoma 13 Years after stereotactic radiation

Methods: In 2002, a 39-year-old female patient underwent stereotactic radiation (27 × 2 Gy) of a left sided vestibular schwannoma. After an uneventful period of 13 years, she was readmitted in an acute emergency situation with increased intracranial pressure. Initially, recurrent vestibular schwannoma was suspected as the cause of occlusive hydrocephalus.

Stereotactic radiosurgery for recurrent vestibular schwannoma after previous resection

OBJECTIVE The goal of this retrospective cohort study was to assess long-term outcomes in patients with vestibular schwannoma (VS) who underwent stereotactic radiosurgery (SRS) after initial microsurgical resection. METHODS From the authors' database of 1770 patients with VS, the authors retrospectively analyzed data from 173 Gamma Knife SRS procedures for VS after 1 (128 procedures) or multiple (45 procedures) microsurgical resections. The median length of the interval between the last resection and SRS was 42 months (range 2-329 months). The median length of clinical follow-up was 74 months (range 6-285 months). Progression-free survival after SRS was determined with Kaplan-Meier analysis. RESULTS At the time of SRS, the hearing of 161 patients (93%) was Gardner-Robertson Class V, and 81 patients (47%) had facial neuropathy (i.e., facial function with House-Brackmann [HB] grades of III-VI), 87 (50%) had trigeminal neuropathy, and 71 (41%) reported imbalance or disequilibrium disorders. The median tumor volume was 2.7 cm3 (range 0.2-21.6 cm3), and the median dose to the tumor margin was 13 Gy (range 11-20 Gy). Radiosurgery controlled growth of 163 (94%) tumors. Progression-free survival after SRS was 97% at 3 years, 95% at 5 years, and 90% at 10 years. Four patients with delayed tumor progression underwent repeat SRS at a median of 35 months (range 23-64 months) after the first SRS. Four patients (2.3%) with tumor progression underwent repeat resection at a median of 25 months (range 19-33 months). Among the patients with any facial dysfunction (indicated by HB grades of II-VI), 19% had improvement in this condition after SRS, and 5.5% with some facial function (indicated by HB grades of I-V) developed more facial weakness. Among patients with trigeminal neuropathy, 20% had improvement in this condition, and 5.8% developed or had worsened trigeminal neuropathy after SRS. CONCLUSIONS Stereotactic radiosurgery offered a safe and effective long-term management strategy for VS patients whose tumors remained or recurred after initial microsurgery.