Leksell Top 25 - Vestibular Schwannoma

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Wen-Yuh Chung, Kang-Du Liu, Cheng-Ying Shiau, Hsiu-Mei Wu, Ling-Wei Wang, Wan-Yuo Guo, Donald Ming-Tak Ho, and David Hung-Chi Pan

Object. The authors conducted a study to determine the optimal radiation dose for vestibular schwannoma (VS) and to examine the histopathology in cases of treatment failure for better understanding of the effects of irradiation.

Methods. A retrospective study was performed of 195 patients with VS; there were 113 female and 82 male patients whose mean age was 51 years (range 11–82 years). Seventy-two patients (37%) had undergone partial or total excision of their tumor prior to gamma knife surgery (GKS). The mean tumor volume was 4.1 cm3 (range 0.04–23.1 cm3). Multiisocenter dose planning placed a prescription dose of 11 to 18.2 Gy on the 50 to 94% isodose located at the tumor margin. Clinical and magnetic resonance (MR) imaging follow-up evaluations were performed every 6 months.

A loss of central enhancement was demonstrated on MR imaging in 69.5% of the patients. At the latest MR imaging assessment decreased or stable tumor volume was demonstrated in 93.6% of the patients. During a median follow-up period of 31 months resection was avoided in 96.8% of cases. Uncontrolled tumor swelling was noted in five patients at 3.5, 17, 24, 33, and 62 months after GKS, respectively. Twelve of 20 patients retained serviceable hearing. Two patients experienced a temporary facial palsy. Two patients developed a new trigeminal neuralgia. There was no treatment-related death. Histopathological examination of specimens in three cases (one at 62 months after GKS) revealed a long-lasting radiation effect on vessels inside the tumor.

Conclusions. Radiosurgery had a long-term radiation effect on VSs for up to 5 years. A margin 12-Gy dose with homogeneous distribution is effective in preventing tumor progression, while posing no serious threat to normal cranial nerve function.

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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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Toshinori Hasegawa, Yoshihisa Kida, Tatsuya Kobayashi, Masayuki Yoshimoto, Yoshimasa Mori, and Jun Yoshida

Object. Gamma knife surgery (GKS) has been a safe and effective treatment for vestibular schwannomas in both the short and long term, although less is known about long-term outcomes in the past 10 years. The aim of this study was to clarify long-term outcomes in patients with vestibular schwannomas treated using GKS based on techniques in place in the early 1990s.

Methods. Eighty patients harboring a vestibular schwannoma (excluding neurofibromatosis Type 2) were treated using GKS between May 1991 and December 1993. Among these, 73 patients were assessed; seven were lost to follow up. The median duration of follow up was 135 months. The mean patient age at the time of GKS was 56 years old. The mean tumor volume was 6.3 cm3, and the mean maximal and marginal radiation doses applied to the tumor were 28.4 and 14.6 Gy, respectively. Follow-up magnetic resonance images were obtained in 71 patients. Forty-eight patients demonstrated partial tumor remission, 14 had tumors that remained stable, and nine demonstrated tumor enlargement or radiation-induced edema requiring resection. Patients with larger tumors did not fare as well as those with smaller lesions. The actuarial 10-year progression-free survival rate was 87% overall, and 93% in patients with tumor volumes less than 10 cm3. No patient experienced malignant transformation.

Conclusions. Gamma knife surgery remained an effective treatment for vestibular schwannomas for longer than 10 years. Although treatment failures usually occurred within 3 years after GKS, it is necessary to continue follow up in patients to reveal delayed tumor recurrence.

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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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Berndt Wowra, Alexander Muacevic, Anja Jess-Hempen, John-Martin Hempel, Stefanie Müller-Schunk, and Jörg-Christian Tonn

Object. The purpose of the study was to define the therapeutic profile of outpatient gamma knife surgery (GKS) for vestibular schwannoma (VS) by using sequential tumor volumetry to quantify changes following treatment.

Methods. A total of 111 patients met the inclusion criteria. The median follow-up duration was 7 years (range 5–9.6 years). Thirty-seven patients (33%) had undergone surgery before GKS and 10 (9%) had neurofibromatosis Type 2 (NF2). The median VS volume was 1.6 cm3 (range 0.08–8.7 cm3).

The actuarial 6-year tumor control rate after a single GKS treatment was 95%. Tumor swelling was observed in 43 patients (38.7%). Recurrence was significantly associated with NF2 (p < 0.003) and the reduced dose (p < 0.03) delivered to these tumors. The incidence of facial nerve neuropathy was mainly determined by surgery prior to GKS (p < 0.0001). Facial nerve radiation toxicity was mild and transient. No permanent facial nerve toxicity was observed. Trigeminal neuropathy occurred in 13 patients, and this was correlated with the VS volume (p < 0.02). The median hearing loss was −10 dB (range + 20 dB to −70 dB). The risk of hearing loss was correlated with age and transient tumor swelling (p < 0.05) but not with dose parameters or NF2.

Conclusions. Outpatient GKS is feasible, effective, and safe. Its therapeutic profile compares favorably with that of microsurgery.

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L. Dade Lunsford, Ajay Niranjan, John C. Flickinger, Ann Maitz, and Douglas Kondziolka

Object. Management options for vestibular schwannomas (VSs) have greatly expanded since the introduction of stereotactic radiosurgery. Optimal outcomes reflect long-term tumor control, preservation of cranial nerve function, and retention of quality of life. The authors review their 15-year experience.

Methods. Between 1987 and 2002, some 829 patients with VSs underwent gamma knife surgery (GKS). Dose selection, imaging, and dose planning techniques evolved between 1987 and 1992 but thereafter remained stable for 10 years. The average tumor volume was 2.5 cm3. The median margin dose to the tumor was 13 Gy (range 10–20 Gy).

No patient sustained significant perioperative morbidity. The average duration of hospital stay was less than 1 day. Unchanged hearing preservation was possible in 50 to 77% of patients (up to 90% in those with intracanalicular tumors). Facial neuropathy risks were reduced to less than 1%. Trigeminal symptoms were detected in less than 3% of patients whose tumors reached the level of the trigeminal nerve. Tumor control rates at 10 years were 97% (no additional treatment needed).

Conclusions. Superior imaging, multiple isocenter volumetric conformal dose planning, and optimal precision and dose delivery contributed to the long-term success of GKS, including in those patients in whom initial microsurgery had failed. Gamma knife surgery provides a low risk, minimally invasive treatment option for patients with newly diagnosed or residual VS. Cranial nerve preservation and quality of life maintenance are possible in long-term follow up.

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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.

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Jean Régis, William Pellet, Christine Delsanti, Henry Dufour, Pierre Hughes Roche, Jean Marc Thomassin, Michel Zanaret, and Jean Claude Peragut

Object. Microsurgical excision is an established treatment for vestibular schwannoma (VS). In 1992 the authors used a patient questionnaire to evaluate the functional outcome and quality of life in a series of 224 consecutive patients. In addition, starting with gamma knife surgery (GKS) in 1992, the authors decided to use the same methodology to evaluate prospectively the results of this modality to compare the two alternatives.

Methods. Among the 500 patients who were included prospectively, the authors only evaluated patients in whom GKS was the primary treatment for unilateral VS. Four years of follow up was available for the first 104 consecutive patients. Statistical analysis of the GKS and microsurgery populations has shown that only a comparison of Stage II and III (according to the Koos classification) was meaningful in terms of group size and preoperative risk factor distribution. Objective results and questionnaire answers from the first 97 consecutive patients were compared with the 110 patients in the microsurgery group who fulfilled the inclusion criteria.

Questionnaire answers indicated that 100% of patients who underwent GKS compared with 63% of patients who underwent microsurgery had no new facial motor disturbance. Forty-nine percent of patients who underwent GKS (17% in the microsurgery study) had no ocular symptoms, and 91% of patients treated with GKS (61% in the microsurgery study) had no functional deterioration after treatment. The mean hospitalization stay was 3 days after GKS and 23 days after microsurgery. All the patients who underwent GKS who had been employed, except one, had kept the same professional activity (56% in the microsurgery study). The mean time away from work was 7 days for GKS (130 days in the microsurgery study). Among patients whose preoperative hearing level was Class 1 according to the Gardner and Robertson scale, 70% preserved functional hearing after GKS (Class 1 or 2) compared with only 37.5% in the microsurgery group.

Conclusions. Functional side effects happen during the first 2 years after radiosurgery. Findings after 4 years of follow up indicated that GKS provided better functional outcomes than microsurgery in this patient series.

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Abdalla Shamisa, Manohar Bance, Sukriti Nag, Charles Tator, Shun Wong, Georg Norén, and Abhijit Guha

✓ Stereotactic radiosurgery is being increasingly advocated as the primary modality for treatment of vestibular schwannomas (VS). This modality has been shown to arrest tumor growth, with few associated short-term morbidities, and with possibly better hearing and facial nerve preservation rates than microsurgery. Radiation-induced oncogenesis has long been recognized, although stereotactic radiosurgery de novo induction of a secondary tumor has never been clearly described. The authors report on a patient with a VS who did not have neurofibromatosis Type 2 and who underwent gamma knife surgery (GKS). This patient required microsurgical removal of the VS within 8 months because of development of a tumor cyst with associated brainstem compression and progressive hydrocephalus. The operation resulted in clinical stabilization and freedom from tumor recurrence.

Seven and a half years after undergoing GKS, the patient presented with symptoms of raised intracranial pressure. Magnetic resonance imaging demonstrated a new ring-enhancing lesion in the inferior temporal lobe adjacent to the area of radiosurgery, which on craniotomy was confirmed to be a glioblastoma multiforme (GBM). Despite additional conventional external-beam radiation to the temporal lobe, the GBM has progressed. Whereas this first reported case of a GBM within the scatter field of GKS does not conclusively prove a direct causal link, it does fulfill all of Cahan's criteria for radiation-induced neoplasia, and demands increased vigilance for the potential long-term complications of stereotactic radiosurgery, and reporting of any similar cases.

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John C. Flickinger, Douglas Kondziolka, Ajay Niranjan, and L. Dade Lunsford

Object. The goal of this study was to define tumor control and complications of radiosurgery encountered using current treatment methods for the initial management of patients with unilateral acoustic neuroma.

Methods. One hundred ninety patients with previously untreated unilateral acoustic neuromas (vestibular schwannomas) underwent gamma knife radiosurgery between 1992 and 1997. The median follow-up period in these patients was 30 months (maximum 85 months). The marginal radiation doses were 11 to 18 Gy (median 13 Gy), the maximum doses were 22 to 36 Gy (median 26 Gy), and the treatment volumes were 0.1 to 33 cm3 (median 2.7 cm3).

The actuarial 5-year clinical tumor-control rate (no requirement for surgical intervention) for the entire series was 97.1 ± 1.9%. Five-year actuarial rates for any new facial weakness, facial numbness, hearing-level preservation, and preservation of testable speech discrimination were 1.1 ± 0.8%, 2.6 ± 1.2%, 71 ± 4.7%, and 91 ± 2.6%, respectively. Facial weakness did not develop in any patient who received a marginal dose of less than 15 Gy (163 patients). Hearing levels improved in 10 (7%) of 141 patients who exhibited decreased hearing (Gardner-Robertson Classes II–V) before undergoing radiosurgery. According to multivariate analysis, increasing marginal dose correlated with increased development of facial weakness (p = 0.0342) and decreased preservation of testable speech discrimination (p = 0.0122).

Conclusions. Radiosurgery for acoustic neuroma performed using current procedures is associated with a continued high rate of tumor control and lower rates of posttreatment morbidity than those published in earlier reports.