Neurofibromatosis Type 2 Vestibular Schwannoma Treatment

Maniakas, Anastasios; Saliba, Issam

doi:10.1097/mao.0000000000000272

Cited by 26 publications

(10 citation statements)

References 49 publications

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“…Physicians are faced with difficult dilemmas when managing VS in NF2 patients [ 9 , 10 , 11 ]. The available active interventions—in addition to the sequelae of the disease itself—may have significant side effects and could result in the (further) losses of hearing, balance, and facial nerve and trigeminal nerve function.…”

Section: Discussionmentioning

confidence: 99%

“…The management of VS in NF2 is challenging. Current strategies include active surveillance, surgery, PhRT, and pharmacotherapy with bevacizumab [ 9 , 10 , 11 ]. Photon radiotherapy achieves a high tumor control, either as fractionated PhRT or single fraction stereotactic radiosurgery (SRS), but side effects, such as decreased hearing, vestibular complaints, or the increased impairment of facial nerve and trigeminal nerve function, are not uncommon [ 12 , 13 ].…”

Section: Introductionmentioning

confidence: 99%

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Proton Radiotherapy for Vestibular Schwannomas in Patients with NF2-Related Schwannomatosis: A Case Series

et al. 2023

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(1) Background: This study aimed to evaluate the efficacy and treatment-related toxicity of proton radiotherapy (PRT) for vestibular schwannoma (VS) in patients with neurofibromatosis type 2-related schwannomatosis (NF2). (2) Methods: Consecutive NF2 patients treated with PRT for VS between 2004 and 2016 were retrospectively evaluated, focusing on tumor volume, facial and trigeminal nerve function, hearing, tinnitus, vestibular symptoms, and the need for salvage therapy after PRT. (3) Results: Eight patients were included (median age 36 years, 50% female). Median follow-up was 71 months. Five (63%) patients received fractionated PRT and three (38%) received PRT radiosurgery for VS. Six patients (75%) received prior VS surgery; three also received bevacizumab. Six patients (75%) did not require salvage therapy after PRT. Two patients (25%) with residual hearing lost it after PRT, and six had already lost ipsilateral hearing prior to PRT. Tumor and treatment-related morbidity could be evaluated in six patients. Following PRT, conditions that occurred or worsened were: facial paresis in five (83%), trigeminal hypoesthesia in two (33%), tinnitus in two (33%), and vestibular symptoms in four patients (67%). (4) Conclusion: After PRT for VS, the majority of the NF2 patients in the cohort did not require additional therapy. Tumor and/or treatment-related cranial nerve deficits were common. This is at least partly explained by the use of PRT as a salvage treatment after surgery or bevacizumab, in the majority of cases. There remains the further opportunity to elucidate the efficacy and toxicity of proton radiotherapy as a primary treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Proton Radiotherapy for Vestibular Schwannomas in Patients with NF2-Related Schwannomatosis: A Case Series

et al. 2023

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“…Radiation might be the alternative choice for residual tumor. In the Maniakas and Saliba [ 14 ] study in 2014, SR have better tumor control than microsurgery; however, microsurgery had better hearing preservation than radiation. Mathieu et al [ 15 ] reported that gamma knife had a high local tumor control rate of 81% in 15 years, [ 16 ] but the hearing preservation at 5 years was 48%.…”

Section: Discussionmentioning

confidence: 99%

Microsurgery for patients diagnosed with neurofibromatosis type 2 complicated by vestibular schwannomas

Chen

Zhang²,

Xu³

et al. 2018

Medicine

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Most patients diagnosed with neurofibromatosis type 2 (NF2) have bilateral vestibular schwannomas (VS). Through reviewing surgical method and clinical outcomes, we tried to find out a strategy for treatments in NF2 patients with VS.We retrospectively reviewed patients diagnosed pathological NF2 and have had microsurgery (MS) for VS in the PLA Army General Hospital. Seventeen patients were included from January 2000 to December 2016. Fifteen patients had progressive hearing impairment, and 7 ears were totally deaf. Computed tomography and magnetic resonance imaging were used for preoperative and postoperative evaluation. House–Brackmann (H-B) classification was used to evaluate facial function, and the hearing outcome was classified according to American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) hearing classification system. The outcomes included functional hearing, facial function, and complications.In the 17 patients, 9 were men, and the mean age was 27.2 years old. The mean duration of disease was 38.4 months. Twenty-six VS were excised. Nine patients with bilateral VS and unilateral surgery had repeated surgery for the contralateral tumor after 3 to 12 months. The hearing preservation rate was 41.6%. In the 26 excisions for VS, 24 had intact facial nerve. In the other 2 tumor excision, damaged facial nerves had head-to-head adhesion using biological fibrin glue. The rate of facial nerve function preservation was 60%. No mortality or major complication was reported. The follow-up time ranged from 11 to 78 months with a mean value of 39 months.MS is an effective treatment for NF2 patients with VS. The operation for bilateral VS should be staged according to tumor size and bilateral hearing function. However, methods on how to preserve functional hearing and facial function remain the issue. Further randomized controlled studies are needed to find out a better treatment for NF2 patients with VS according to the overall condition.

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“…[4][5][6][19][20][21] By contrast, in a systematic review of NF2-associated VSs, surgical resection alone resulted in a 45.4% recurrence rate during an average follow-up time of 4.15 years. 22 Comparatively, of tumors that had prior surgery (N = 96) in this study, the type of SRS (adjuvant or salvage after recurrence) was specified in 72 tumors. Of these 72 tumors, SRS was used as salvage therapy after recurrence in 90.3%.…”

Section: Tumor Control and Serviceable Hearing Preservationmentioning

confidence: 99%

Stereotactic Radiosurgery for Vestibular Schwannoma in Neurofibromatosis Type 2: An International Multicenter Case Series of Response and Malignant Transformation Risk

et al. 2023

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BACKGROUND:Vestibular schwannomas (VSs) related to neurofibromatosis type 2 (NF2) are challenging tumors. The increasing use of stereotactic radiosurgery (SRS) necessitates further investigations of its role and safety.OBJECTIVE:To evaluate tumor control, freedom from additional treatment (FFAT), serviceable hearing preservation, and radiation-related risks of patients with NF2 after SRS for VS.METHODS:We performed a retrospective study of 267 patients with NF2 (328 VSs) who underwent single-session SRS at 12 centers participating in the International Radiosurgery Research Foundation. The median patient age was 31 years (IQR, 21-45 years), and 52% were male.RESULTS:A total of 328 tumors underwent SRS during a median follow-up time of 59 months (IQR, 23-112 months). At 10 and 15 years, the tumor control rates were 77% (95% CI: 69%-84%) and 52% (95% CI: 40%-64%), respectively, and the FFAT rate were 85% (95% CI: 79%-90%) and 75% (95% CI: 65%-86%), respectively. At 5 and 10 years, the serviceable hearing preservation rates were 64% (95% CI: 55%-75%) and 35% (95% CI: 25%-54%), respectively. In the multivariate analysis, age (hazards ratio: 1.03 [95% CI: 1.01-1.05]; P = .02) and bilateral VSs (hazards ratio: 4.56 [95% CI: 1.05-19.78]; P = .04) were predictors for serviceable hearing loss. Neither radiation-induced tumors nor malignant transformation were encountered in this cohort.CONCLUSION:Although the absolute volumetric tumor progression rate was 48% at 15 years, the rate of FFAT related to VS was 75% at 15 years after SRS. None of the patients with NF2-related VS developed a new radiation-related neoplasm or malignant transformation after SRS.

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Neurofibromatosis Type 2 Vestibular Schwannoma Treatment

Cited by 26 publications

References 49 publications

Proton Radiotherapy for Vestibular Schwannomas in Patients with NF2-Related Schwannomatosis: A Case Series

Proton Radiotherapy for Vestibular Schwannomas in Patients with NF2-Related Schwannomatosis: A Case Series

Microsurgery for patients diagnosed with neurofibromatosis type 2 complicated by vestibular schwannomas

Stereotactic Radiosurgery for Vestibular Schwannoma in Neurofibromatosis Type 2: An International Multicenter Case Series of Response and Malignant Transformation Risk

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