Leksell Top 25 - Vestibular Schwannoma

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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.

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Dheerendra Prasad, Melita Steiner and Ladislau Steiner

Object. The goal of this study was to assess the results of gamma surgery (GS) for vestibular schwannoma (VS) in 200 cases treated over the last 10 years and to review the role of this neurosurgical procedure in the management of VS.

Methods. Follow-up reviews ranging from 1 to 10 years were available in 153 of these patients. Follow-up images in these cases were analyzed using computer software that we developed to obtain volume measurements for the tumors, and the clinical condition of the patients was assessed using questionnaires.

Gamma surgery was the primary treatment modality in 96 cases and followed microsurgery in 57 cases. Tumors ranged in volume from 0.02 to 18.3 cm3. In the group in which GS was the primary treatment, a decrease in volume was observed in 78 cases (81%), no change in 12 (12%), and an increase in volume in six cases (6%). The decrease was more than 75% in seven cases. In the group treated following microsurgery, a decrease in volume was observed in 37 cases (65%), no change in 14 (25%), and an increase in volume in six (11%). The decrease was more than 75% in eight cases. Five patients experienced trigeminal dysfunction; in three cases this was transient and in the other two it was persistent, although there has been improvement. Three patients had facial paresis (in one case this was transient, lasting 6 weeks; in one case there was 80% recovery at 18 months posttreatment; and in one case surgery was performed after the onset of facial paresis for presumed increase in tumor size). Over a 6-year period, hearing deteriorated in 60% of the patients. Three patients showed an improvement in hearing. No hearing deterioration was observed during the first 2 years of follow-up review.

Conclusions. Gamma surgery should be used to treat postoperative residual tumors as well as tumors in patients with medical conditions that preclude surgery. Microsurgery should be performed whenever a surgeon is confident of extirpating the tumor with a risk—benefit ratio superior to that presented in this study.

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Brian R. Subach, Douglas Kondziolka, L. Dade Lunsford, David J. Bissonette, John C. Flickinger and Ann H. Maitz

Object. Stereotactically guided radiosurgery is one of the primary treatment modalities for patients with acoustic neuromas (vestibular schwannomas). The goal of radiosurgery is to arrest tumor growth while preserving neurological function. Patients with acoustic neuromas associated with neurofibromatosis Type 2 (NF2) represent a special challenge because of the risk of complete deafness. To define better the tumor control rate and long-term functional outcome, the authors reviewed their 10-year experience in treating these lesions.

Methods. Forty patients underwent stereotactic radiosurgery at the University of Pittsburgh, 35 of them for solitary tumors. The other five underwent staged procedures for bilateral lesions (10 tumors, 45 total). Thirteen patients (with 29% of tumors) had undergone a median of two prior resections. The mean tumor volume at radiosurgery was 4.8 ml, and the mean tumor margin dose was 15 Gy (range 12–20 Gy).

The overall tumor control rate was 98%. During the median follow-up period of 36 months, 16 tumors (36%) regressed, 28 (62%) remained unchanged, and one (2%) grew. In the 10 patients for whom more than 5 years of clinical and neuroimaging follow-up results were available (median 92 months), five tumors were smaller and five remained unchanged. Surgical resection was performed in three patients (7%) after radiosurgery; only one showed radiographic evidence of progression. Useful hearing (Gardner—Robertson Class I or II) was preserved in six (43%) of 14 patients, and this rate improved to 67% after modifications made in 1992. Normal facial nerve function (House—Brackmann Grade 1) was preserved in 25 (81%) of 31 patients. Normal trigeminal nerve function was preserved in 34 (94%) of 36 patients.

Conclusions. Stereotactically guided radiosurgery is a safe and effective treatment for patients with acoustic tumors in the setting of NF2. The rate of hearing preservation may be better with radiosurgery than with other available techniques.

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Vestibular schwannoma management

Part II. Failed radiosurgery and the role of delayed microsurgery

Bruce E. Pollock, L. Dade Lunsford, Douglas Kondziolka, Raymond Sekula, Brian R. Subach, Robert L. Foote and John C. Flickinger

Object. The indications, operative findings, and outcomes of vestibular schwannoma microsurgery are controversial when it is performed after stereotactic radiosurgery. To address these issues, the authors reviewed the experience at two academic medical centers.

Methods. During a 10-year interval, 452 patients with unilateral vestibular schwannomas underwent gamma knife radiosurgery. Thirteen patients (2.9%) underwent delayed microsurgery at a median of 27 months (range 7–72 months) after they had undergone radiosurgery. Six of the 13 patients had undergone one or more microsurgical procedures before they underwent radiosurgery. The indications for surgery were tumor enlargement with stable symptoms in five patients, tumor enlargement with new or increased symptoms in five patients, and increased symptoms without evidence of tumor growth in three patients. Gross-total resection was achieved in seven patients and near-gross-total resection in four patients. The surgery was described as more difficult than that typically performed for schwannoma in eight patients, no different in four patients, and easier in one patient. At the last follow-up evaluation, three patients had normal or near-normal facial function, three patients had moderate facial dysfunction, and seven had facial palsies. Three patients were incapable of caring for themselves, and one patient died of progression of a malignant triton tumor.

Conclusions. Failed radiosurgery in cases of vestibular schwannoma was rare. No clear relationship was demonstrated between the use of radiosurgery and the subsequent ease or difficulty of delayed microsurgery. Because some patients have temporary enlargement of their tumor after radiosurgery, the need for surgical resection after radiosurgery should be reviewed with the neurosurgeon who performed the radiosurgery and should be delayed until sustained tumor growth is confirmed. A subtotal tumor resection should be considered for patients who require surgical resection of their tumor after vestibular schwannoma radiosurgery.