Leksell Top 25 - Vestibular Schwannoma

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Huai-che Yang, Hideyuki Kano, Nasir Raza Awan, L. Dade Lunsford, Ajay Niranjan, John C. Flickinger, Josef Novotny Jr., Jagdish P. Bhatnagar and Douglas Kondziolka

Object

Stereotactic radiosurgery (SRS) is an important management option for patients with small- and medium-sized vestibular schwannomas. To assess the potential role of SRS in larger tumors, the authors reviewed their recent experience.

Methods

Between 1994 and 2008, 65 patients with vestibular schwannomas between 3 and 4 cm in one extracanalicular maximum diameter (median tumor volume 9 ml) underwent Gamma Knife surgery. Seventeen patients (26%) had previously undergone resection.

Results

The median follow-up duration was 36 months (range 1–146 months). At the first planned imaging follow-up at 6 months, 5 tumors (8%) were slightly expanded, 53 (82%) were stable in size, and 7 (11%) were smaller. Two patients (3%) underwent resection within 6 months due to progressive symptoms. Two years later, with 63 tumors overall after the 2 post-SRS resections, 16 tumors (25%) had a volume reduction of more than 50%, 22 (35%) tumors had a volume reduction of 10–50%, 18 (29%) were stable in volume (volume change < 10%), and 7 (11%) had larger volumes (5 of the 7 patients underwent resection and 1 of the 7 underwent repeat SRS). Eighteen (82%) of 22 patients with serviceable hearing before SRS still had serviceable hearing after SRS more than 2 years later. Three patients (5%) developed symptomatic hydrocephalus and underwent placement of a ventriculoperitoneal shunt. In 4 patients (6%) trigeminal sensory dysfunction developed, and in 1 patient (2%) mild facial weakness (House-Brackmann Grade II) developed after SRS. In univariate analysis, patients who had a previous resection (p = 0.010), those with a tumor volume exceeding 10 ml (p = 0.05), and those with Koos Grade 4 tumors (p = 0.02) had less likelihood of tumor control after SRS.

Conclusions

Although microsurgical resection remains the primary management choice in patients with low comorbidities, most vestibular schwannomas with a maximum diameter less than 4 cm and without significant mass effect can be managed satisfactorily with Gamma Knife radiosurgery.

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Jean Régis, Romain Carron, Michael C. Park, Outouma Soumare, Christine Delsanti, Jean Marc Thomassin and Pierre-Hugues Roche

Object

The roles of the wait-and-see strategy and proactive Gamma Knife surgery (GKS) in the treatment paradigm for small intracanalicular vestibular schwannomas (VSs) is still a matter of debate, especially when patients present with functional hearing. The authors compare these 2 methods.

Methods

Forty-seven patients (22 men and 25 women) harboring an intracanalicular VS were followed prospectively. The mean age of the patients at the time of inclusion was 54.4 years (range 20–71 years). The mean follow-up period was 43.8 ± 40 months (range 9–222 months). Failure was defined as significant tumor growth and/or hearing deterioration that required microsurgical or radiosurgical treatment. This population was compared with a control group of 34 patients harboring a unilateral intracanalicular VS who were consecutively treated by GKS and had functional hearing at the time of radiosurgery.

Results

Of the 47 patients in the wait-and-see group, treatment failure (tumor growth requiring treatment) was observed in 35 patients (74%), although conservative treatment is still ongoing for 12 patients. Treatment failure in the control (GKS) group occurred in only 1 (3%) of 34 patients. In the wait-and-see group, there was no change in tumor size in 10 patients (21%), tumor growth in 36 patients (77%), and a mild decrease in tumor size in 1 patient (2%). Forty patients in the wait-and-see group were available for a hearing level study, which demonstrated no change in Gardner-Robertson hearing class for 24 patients (60%). Fifteen patients (38%) experienced more than 10 db of hearing loss and 2 of them became deaf. At 3, 4, and 5 years, the useful hearing preservation rates were 75%, 52%, and 41% in the wait-and-see group and 77%, 70%, and 64% in the control group, respectively. Thus, the chances of maintaining functional hearing and avoiding further intervention were much higher in cases treated by GKS (79% and 60% at 2 and 5 years, respectively) than in cases managed by the wait-and-see strategy (43% and 14% at 2 and 5 years, respectively).

Conclusions

These data indicate that the wait-and-see policy exposes the patient to elevated risks of tumor growth and degradation of hearing. Both events may occur independently in the mid-term period. This information must be presented to the patient. A careful sequential follow-up may be adopted when the wait-and-see strategy is chosen, but proactive GKS is recommended when hearing is still useful at the time of diagnosis. This recommendation may be a main paradigm shift in the practice of treating intracanalicular VSs.

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Isaac Yang, Michael E. Sughrue, Seunggu J. Han, Derick Aranda, Lawrence H. Pitts, Steven W. Cheung and Andrew T. Parsa

Object

Gamma Knife surgery (GKS) has evolved into a practical alternative to open microsurgical resection in the treatment of patients with vestibular schwannoma (VS). Hearing preservation rates in GKS series suggest very favorable outcomes without the possible acute morbidity associated with open microsurgery. To mitigate institutional and practitioner bias, the authors performed an analytical review of the published literature on the GKS treatment of vestibular schwannoma patients. Their aim was to objectively characterize the prognostic factors that contribute to hearing preservation after GKS, as well as methodically summarize the reported literature describing hearing preservation after GKS for VS.

Methods

A comprehensive search of the English-language literature revealed a total of 254 published studies reporting assessable and quantifiable outcome data obtained in patients who underwent radiosurgery for VSs. Inclusion criteria for articles were 4-fold: 1) hearing preservation rates reported specifically for VS; 2) hearing status reported using the American Association of Otolaryngology–Head and Neck Surgery (AAO-HNS) or Gardner-Robertson classification; 3) documentation of initial tumor size; and 4) GKS was the only radiosurgical modality in the treatment. In the analysis only patients with AAO-HNS Class A or B or Gardner-Robertson Grade I or II status at the last follow-up visit were defined as having preserved hearing. Hearing preservation and outcome data were then aggregated and analyzed based on the radiation dose, tumor volume, and patient age.

Results

The 45 articles that met the authors' inclusion criteria represented 4234 patients in whom an overall hearing preservation rate was 51%, irrespective of radiation dose, patient age, or tumor volume. Practitioners who delivered an average ≤ 13-Gy dose of radiation reported a higher hearing preservation rate (60.5% at ≤ 13 Gy vs 50.4% at > 13 Gy; p = 0.0005). Patients with smaller tumors (average tumor volume ≤ 1.5 cm3) had a hearing preservation rate (62%) comparable with patients harboring larger tumors (61%) (p = 0.8968). Age was not a significant prognostic factor for hearing preservation rates as in older patients there was a trend toward improved hearing preservation rates (56% at < 65 years vs 71% at ≥ 65 years of age; p < 0.1134). The average overall follow-up in the studies reviewed was 44.4 ± 32 months (median 35 months).

Conclusions

These data provide a methodical overview of the literature regarding hearing preservation with GKS for VS and a less biased assessment of outcomes than single-institution studies. This objective analysis provides insight into advising patients of hearing preservation rates for GKS treatment of VSs that have been reported, as aggregated in the published literature. Analysis of the data suggests that an overall hearing preservation rate of ~ 51% can be expected approaching 3–4 years after radiosurgical treatment, and the analysis reveals that patients treated with ≤ 13 Gy were more likely to have preserved hearing than patients receiving larger doses of radiation. Furthermore, larger tumors and older patients do not appear to be at any increased risk for hearing loss after GKS for VS than younger patients or patients with smaller tumors.

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Bruce E. Pollock, Michael J. Link and Robert L. Foote

Object

The decline in cranial nerve morbidity after radiosurgery for vestibular schwannoma (VS) correlates with dose reduction and other technical changes to this procedure. The effect these changes have had on tumor control has not been well documented.

Methods

The authors performed a retrospective review of 293 patients with VSs who underwent radiosurgery between 1990 and 2004 and had a minimum of 24 months of imaging follow-up (90% of the entire series). The median radiation dose to the tumor margin was 13 Gy. Treatment failure was defined as progressive tumor enlargement noted on 2 or more imaging studies. The mean postradiosurgical follow-up was 60.9 ± 32.5 months.

Results

Tumor growth was noted in 15 patients (5%) at a median of 32 months after radiosurgery. Radiographically demonstrated tumor control was 96% at 3 years and 94% at 7 years after radiosurgery. Univariate analysis revealed 2 factors that correlated with failed radiosurgery for VS: an increasing number of isocenters (p = 0.03) and tumor margin radiation doses ≤ 13 Gy (p = 0.02). Multivariate analysis showed that only an increasing number of isocenters correlated with failed VS radiosurgery (hazard ratio 1.1, 95% CI 1.02–1.32, p < 0.05). The tumor margin radiation dose (p = 0.22) was not associated with tumor growth after radiosurgery.

Conclusions

Distortion of stereotactic MR imaging coupled with increased radiosurgical conformality and progressive dose reduction likely caused some VSs to receive less than the prescribed radiation dose to the entire tumor volume.

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Mark E. Linskey

Stereotactic radiosurgery (SRS) for vestibular schwannomas has evolved and improved over time. Although early short-term follow-up reports suggest that fractionation yields hearing preservation rates equivalent to modern single-dose SRS techniques, significant questions remain regarding long-term tumor control after the use of fractionation in a late responding tumor with a low proliferative index and α/β ratio. With single-dose SRS, critical hearing preservation variables include: 1) strict attention to prescription dose 3D conformality so that the ventral cochlear nucleus (VCN) receives ≤ 9 Gy; 2) careful delineation of the 3D tumor margin to exclude the cochlear nerve when visualizable with contrast-enhanced T2-weighted MR volumetric imaging techniques and exclusion the dura mater of the anterior border of the internal auditory canal; 3) a tumor margin dose prescription ≤ 12 Gy; 4) optimization of the tumor treatment gradient index without sacrificing coverage and conformality; and 5) strict attention to prescription dose 3D conformality so that the modiolus and the basal turn of the cochlea receive the lowest possible dose (ideally < 4–5.33 Gy). Testable correlates for the relative importance of the VCN versus cochlear dose given the tonotopic organization of each structure suggests that VCN toxicity should lead to preferential loss of low hearing frequencies, while cochlear toxicity should lead to preferential loss of high hearing frequencies. The potential after SRS for hearing toxicity from altered endolymph and/or perilymph fluid dynamics either via impaired fluid production and/or absorption has yet to be explored. Serous otitis media, ossicular or temporal bone osteonecrosis, and chondromalacia are not likely to be relevant factors or considerations for hearing preservation after SRS.

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Nicolas Massager, Ouzi Nissim, Carine Delbrouck, Isabelle Delpierre, Daniel Devriendt, Françoise Desmedt, David Wikler, Jacques Brotchi and Marc Levivier

Object

The purpose of this study was to measure the dose of radiation delivered to the cochlea during a Gamma knife surgery (GKS) procedure for treatment of patients with vestibular schwannomas (VSs), and to analyze the relationship between cochlear irradiation and the hearing outcome of these patients.

Methods

Eighty-two patients with VSs were treated with GKS using a marginal dose of 12 Gy. No patient had neurofibromatosis Type 2 disease, and all had a Gardner–Robertson hearing class of I to IV before treatment, and a radiological and audiological follow-up of at least 1-year after GKS. The dosimetric data of the volume of the cochlea were retrospectively analyzed and were correlated with the auditory outcome of patients.

Results

The mean radiation dose delivered to the cochlear volume ranged from 1.30 to 10.00 Gy (median 4.15 Gy). The cochlea received significantly higher radiation doses in patients with worsening of hearing after GKS. A highly significant association between the cochlear and the intracanalicular dose of radiation delivered during GKS was found.

Conclusions

During GKS for VSs, relatively high doses of radiation can be delivered to the cochlea. Worsening of hearing after GKS can be the consequence of either radiation injury to the cochlea or the irradiation dose delivered into the auditory canal, or both.

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Ania G. Pollack, MaryAnne H. Marymont, John A. Kalapurakal, Alan Kepka, Vythialingam Sathiaseelan and James P. Chandler

✓ The authors describe an acute facial and acoustic neuropathy following gamma knife surgery (GKS) for vestibular schwannoma (VS). This 39-year-old woman presenting with tinnitus underwent GKS for a small right-sided intracanalicular VS, receiving a maximal dose of 26 Gy and a tumor margin dose of 13 Gy to the 50% isodose line. Thirty-six hours following treatment she presented with nausea, vomiting, vertigo, diminished hearing, and a House—Brackmann Grade III facial palsy. She was started on intravenous glucocorticosteroid agents, and over the course of 2 weeks her facial function returned to House—Brackmann Grade I. Unfortunately, her hearing loss persisted. A magnetic resonance (MR) image obtained at the time of initial deterioration demonstrated a significant decrease in tumor enhancement but no change in tumor size or peritumoral edema. Subsequently, the patient experienced severe hemifacial spasms, which persisted for a period of 3 weeks and then progressed to a House—Brackmann Grade V facial palsy. During the next 3 months, the patient was treated with steroids and in time her facial function and hearing returned to baseline levels. Results of MR imaging revealed transient enlargement (3 mm) of the tumor, which subsequently returned to its baseline size. This change corresponded to the tumor volume increase from 270 to 336 mm3. The patient remains radiologically and neurologically stable at 10 months posttreatment.

This is the first detailed report of acute facial and vestibulocochlear neurotoxicity following GKS for VS that improved with time. In addition, MR imaging findings were indicative of early neurotoxic changes. A review of possible risk factors and explanations of causative mechanisms is provided.

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Albertus T. C. J. van Eck and Gerhard A. Horstmann

Object. Gamma knife surgery (GKS) for vestibular schwannoma is still associated with an additional hearing loss of approximately 30%. The purpose of this study was to record the effect on hearing preservation of maintaining a margin dose of 13 Gy while reducing the maximum dose to 20 Gy.

Methods. Seventy-eight of 95 patients who entered a prospective protocol with a follow up of at least 12 months (mean 22 months) were evaluated. The mean tumor volume was 2.28 cm3. After a mean follow-up duration of 22 months, the magnetic resonance imaging—based tumor control rate was 87%. In two cases a second procedure (surgery) was necessary. Thus, the clinical control rate was 97.5%. In two cases there was an increase in trigeminal dysesthesia. One patient suffered transient facial nerve impairment. Functional hearing was preserved in 83.4% of the patients with functional hearing preoperatively.

Conclusions. Reducing the maximum dose to 20 Gy seems to be an effective treatment, which probably increases preservation of functional hearing without sacrificing the high tumor control rates achieved in radiosurgery. Post-radiosurgery tumor swelling occurred in 25% of the cases and was not correlated with hearing deterioration.

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Hiroshi K. Inoue

Object. The author conducted a study to assess the long-term results obtained in patients who underwent GKS for large vestibular schwannomas (> cochlear nerve functions were evaluated.

Methods. Twenty consecutive large tumors in 18 patients (including two cases of neurofibromatosus Type 2 [NF2]) were followed for more than 6 years. There were eight tumors that were more than 4 cm in maximum diameter. Microsurgery had already been performed prior to GKS in 11 patients (nine recurrent and two residual tumors).

Four patients (including one with NF2) died during the follow-up period of other diseases or by accident. Fourteen of 15 tumors were stable or decreased in size. Microsurgery was performed in one patient 2 years after radiosurgery. Facial nerve function was preserved in all patients and hearing preserved in four of five patients with cochlear nerve function prior to radiosurgery. No adverse effects of radiosurgery have been observed to date.

Conclusions. Gamma knife surgery seems to have a place in the low-dose treatment of selected large vestibular schwannoma in patients with a reasonable chance of retaining facial function and pretreatment hearing level. Patients with severe brainstem compression should first be undergo microsurgery.

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Mark E. Linskey, Peter A. S. Johnstone, Michael O'Leary and Steven Goetsch

Object. The dosimetry of radiation exposure of healthy inner, middle, and external ear structures that leads to hearing loss, tinnitus, facial weakness, dizziness, vertigo, and imbalance after gamma knife surgery (GKS) for vestibular schwannomas (VSs) is unknown. The authors quantified the dose of radiation received by these structures after GKS for VS to assess the likelihood that these doses contributed to postradiosurgery complications.

Methods. A retrospective study was performed using a prospectively acquired database of a consecutive series of 54 patients with VS who were treated with GKS during a 3.5-year period at an “open unit” gamma knife center. Point doses were measured for 18 healthy temporal bone structures in each patient, with the anatomical position of each sampling point confirmed by a fellowship-trained neurootologist. These values were compared against single-dose equivalents for the 5-year tolerance dose for a 5% risk of complications and the 5-year tolerance dose for a 50% risk of complications, which were calculated using known 2-Gy/fraction thresholds for chronic otitis, chondromalacia, and osseous necrosis, as well as the tumor margin dose and typical tumor margin prescription doses for patients in whom hearing preservation was attempted.

External and middle ear doses were uniformly low. The intratemporal facial nerve is susceptible to unintentionally high radiation exposure at the fundus of the internal auditory canal, with higher than tumor margin doses detected in 26% of cases. In the cochlea, the basal turn near the modiolus and its inferior portion are most susceptible, with doses greater than 12 Gy detected in 10.8 and 14.8% of cases. In the vestibular labyrinth, the ampulated ends of the lateral and posterior semicircular canals are most susceptible, with doses greater than 12 Gy detected in 7.4 and 5.1% of cases.

Conclusions. Doses delivered to middle and external ear structures are unlikely to contribute to post-GKS complications, but unexpectedly high doses may be delivered to sensitive areas of the intratemporal facial nerve and inner ear. Unintentional delivery of high doses to the stria vascularis, the sensory neuroepithelium of the inner ear organs and/or their ganglia, may play a role in the development of post-GKS tinnitus, hearing loss, dizziness, vertigo, and imbalance. Minimizing treatment complications post-GKS for VS requires precise dose planning conformality with the three-dimensional surface of the tumor.