Stereotactic radiosurgery for vestibular schwannomas in neurofibromatosis type 2 patients: a systematic review and meta-analysis

Tosi, Umberto; Maayan, Omri; An, Anjile; Lavieri, Miguel E. Tusa; Guadix, Sergio W.; DeRosa, Antonio; Christos, Paul J.; Pannullo, Susan C.; Stieg, Philip E.; Brandmaier, Andrew; Knisely, Jonathan; Ramakrishna, Rohan

doi:10.1007/s11060-021-03910-8

Cited by 15 publications

(14 citation statements)

References 77 publications

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“…However, a significantly lower prior surgery rate of 23% was reported. A recently published systematic review and meta-analysis also covered the effect of photon SRS for VS in NF2 [ 13 ]. A total of 750 tumors were irradiated with a mean dose of 13.2 up to 25.0 Gy using a variety of radiation techniques.…”

Section: Discussionmentioning

confidence: 99%

“…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 ]. Therefore, it remains challenging to find a treatment that achieves good tumor control with minimal treatment-induced side effects.…”

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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“…In recent studies, tumor progression rate after radiosurgery remained indeed low in NF2-SWN (progression of more than 10% of initial tumor volume in 11% of cases [31], progression of more than 20% at 3 years in 4.9% cases [32], and progression of more than 25% at 10 years in 23% of cases [33]). The need for further intervention was evaluated between 3% [32] and 12% [31,33]. Of note, most radiosurgery studies do not describe the natural history of tumors before treatment, in a disease where many tumors are spontaneously stable.…”

Section: Radiosurgerymentioning

confidence: 98%

“…A recent meta-analysis with the largest cohort of patients with NF2-SWN treated by radiosurgery (780 tumors) found a clinical tumor control rate of 88% [31]. In recent studies, tumor progression rate after radiosurgery remained indeed low in NF2-SWN (progression of more than 10% of initial tumor volume in 11% of cases [31], progression of more than 20% at 3 years in 4.9% cases [32], and progression of more than 25% at 10 years in 23% of cases [33]). The need for further intervention was evaluated between 3% [32] and 12% [31,33].…”

Section: Radiosurgerymentioning

confidence: 99%

“…Of note, most radiosurgery studies do not describe the natural history of tumors before treatment, in a disease where many tumors are spontaneously stable. On the other hand, morbidity after radiosurgery remained significant with worsening of serviceable hearing observed between 65% [33] and 74% [31] of patients, and increased facial nerve impairment observed in 62% of patients [31]. With a median follow up of 5.2 years, none of the patients with NF2 -related vestibular schwannomas (VS) developed a new radiation-related neoplasm or malignant transformation after SRS.…”

Section: Therapeutic Aspects Of Schwannomatosismentioning

confidence: 99%

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Schwannomatosis: a Realm Reborn: year one

Planet,

Kalamarides,

Peyre

2023

Current Opinion in Oncology

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Purpose of review In 2022, an international consensus recommendation revised the nomenclature for neurofibromatosis type 2 (NF2) and Schwannomatosis (SWN), now grouped under the umbrella term Schwannomatosis, and defined new diagnostic criteria. Recent findings This review describes the molecular criteria for diagnosis of schwannomatosis and the subsequent diagnosis strategy, while setting out the most recent advances in our understanding of the natural history, pathology, molecular biology and treatment of schwannomatosis-associated tumors, including schwannomas, meningiomas and ependymomas. Summary Somatic mutation screening should become a new standard for the diagnosis of NF2-, LTZTR1-, SMARCB1- and 22q-schwannomatosis to discriminate those conditions. Constitutional events in NF2-Schwannomatosis have a major influence on disease severity and justifiably motivate ongoing efforts on gene replacement therapy research. On the other hand, underlying mechanisms of disease severity and associated pain remain largely unknown in non-NF2-SWN and independent of germline mutation. Research efforts therefore focus on pain relief in ongoing trials and the discovery of new molecular mechanisms underlying schwannoma tumorigenesis/pain/neuropathies.

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Stereotactic Radiosurgery and Radiotherapy for Vestibular Schwannoma in NF2‐Related Schwannomatosis

Shrivastava,

Emmanouil,

Mathew

et al. 2023

The Laryngoscope

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ObjectivesTo determine the long‐term control rates and hearing outcomes for growing vestibular schwannoma in NF2‐related schwannomatosis (NF2) treated with stereotactic radiosurgery (SRS) and fractionated radiotherapy (FRT).MethodsRetrospective review of all patients treated with SRS/FRT between 1986 and2021 from a tertiary NF2 unit. Overall tumor control was defined as: (1) growth control (growth failure was defined as growth in any dimension of 3 millimetres or more from baseline post‐SRS/FRT), and (2) treatment control (no need for further intervention). Loss of serviceable hearing was defined as a drop in speech discrimination score below 50% after SRS/FRT.ResultsThere were 81 cases, with a mean duration of follow‐up of 125 months. Overall control rate was 72% (58/81), with 80% (65/81) growth control and 74% (60/81) treatment control. There was a 5‐year actuarial survival of 77% and 10‐year survival of 71%. Forty‐three percent (30/69) of cases did not have serviceable hearing at baseline. Of those remaining, 49% (19/39) preserved serviceable hearing during follow‐up at a mean of 106 months. Actuarial survival for preservation of serviceable hearing at 5 and 10 years was 69% and 53%. There were poorer outcomes with increasing genetic severity, and with baseline tumor size >3 cm. No cases of SRS/FRT‐related malignancy were identified at a mean follow‐up of 10 years.ConclusionStereotactic radiosurgery/fractionated radiotherapy are an effective option to treat growing vestibular schwannoma in patients with NF2 with the potential for hearing preservation in a proportion of patients.Level of EvidenceLevel 4—Case Series Laryngoscope, 2023

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Stereotactic radiosurgery for vestibular schwannomas in neurofibromatosis type 2 patients: a systematic review and meta-analysis

Cited by 15 publications

References 77 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

Schwannomatosis: a Realm Reborn: year one

Stereotactic Radiosurgery and Radiotherapy for Vestibular Schwannoma in NF2‐Related Schwannomatosis

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