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

Bin-Alamer, Othman; Abou‐Al‐Shaar, Hussam; Mallela, Arka N.; Niranjan, Ajay; Sheehan, Jason P.; Lunsford, L. Dade

doi:10.1227/neu.0000000000002614

Cited by 3 publications

(5 citation statements)

References 16 publications

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“…Recent data following 266 NF2 patients with benign intracranial tumors treated with SRS/FRT may suggest a 20‐year malignancy rate of 6% 13 . Other publications have demonstrated conflicting results but with limited follow‐up 27 …”

Section: Discussionmentioning

confidence: 99%

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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Section: Discussionmentioning

confidence: 99%

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

Shrivastava,

Emmanouil,

Mathew

et al. 2023

The Laryngoscope

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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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“…3 Given the minimally invasive nature of stereotactic radiosurgery (SRS) and its safety and efficacy in the management of VS, we sought to better understand the outcomes when VS SRS was performed in octogenarians. [3][4][5] In this study, we examined tumor control, need for additional tumor management and survival after SRS for VS in octogenarians.…”

mentioning

confidence: 99%

Vestibular Schwannoma Stereotactic Radiosurgery in Octogenarians: Case Series

et al. 2023

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BACKGROUND AND OBJECTIVES: The management of octogenarians with vestibular schwannomas (VS) has received little attention. However, with the increase in octogenarian population, more effort is needed to clarify the value of stereotactic radiosurgery (SRS) in this population. The aim of this study was to evaluate the safety and efficacy of SRS in this patient age group. METHODS: A retrospective study of 62 patients aged 80 years or older who underwent single-session SRS for symptomatic VS during a 35-year interval was performed. The median patient age was 82 years, and 61.3% were male. SRS was performed as planned adjuvant management or for delayed progression after prior partial resection in 5 patients. RESULTS: SRS resulted in a 5-year tumor control rate of 95.6% with a 4.8% risk of adverse radiation effects (ARE). Tumor control was unrelated to patient age, tumor volume, Koos grade, sex, SRS margin dose, or prior surgical management. Four patients underwent additional management including 1 patient with symptomatic progression requiring surgical resection, 2 patients with symptomatic hydrocephalus requiring cerebrospinal fluid diversion, and 1 patient whose tumor-related cyst required delayed cyst aspiration. Three patients developed ARE, including 1 patient with permanent facial weakness (House-Brackmann grade II), 1 who developed trigeminal neuropathy, and 1 who had worsening gait disorder. Six patients had serviceable hearing preservation before SRS, and 2 maintained serviceable hearing preservation after 4 years. A total of 44 (71%) patients died at an interval ranging from 6 to 244 months after SRS. CONCLUSION: SRS resulted in tumor and symptom control in most octogenarian patients with VS.

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

Cited by 3 publications

References 16 publications

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

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

Schwannomatosis: a Realm Reborn: year one

Vestibular Schwannoma Stereotactic Radiosurgery in Octogenarians: Case Series

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