%0 Journal Article
%T Radiation treatment of benign tumors in NF2-related-schwannomatosis: A national study of 266 irradiated patients showing a significant increase in malignancy/malignant progression.
%A Evans DG
%A Halliday D
%A Obholzer R
%A Afridi S
%A Forde C
%A Rutherford SA
%A Hammerbeck-Ward C
%A Lloyd SK
%A Freeman SM
%A Pathmanaban ON
%A Thomas OM
%A Laitt RD
%A Stivaros S
%A Kilday JP
%A Vassallo G
%A McBain C
%A Lavin T
%A Paterson C
%A Whitfield G
%A McCabe MG
%A Axon PR
%A Halliday J
%A Mackeith S
%A Parry A
%A  
%A Harkness EF
%A Buttimore J
%A King AT
%J Neurooncol Adv
%V 5
%N 1
%D Jan-Dec 2023
%M 37051330
暂无%R 10.1093/noajnl/vdad025
%X <B>UNASSIGNED: </B>Radiation treatment of benign tumors in tumor predisposition syndromes is controversial, but short-term studies from treatment centers suggest safety despite apparent radiation-associated malignancy being reported. We determined whether radiation treatment in NF2-related schwannomatosis patients is associated with increased rates of subsequent malignancy (M)/malignant progression (MP).<BR><B>UNASSIGNED: </B>All UK patients with NF2 were eligible if they had a clinical/molecular diagnosis. Cases were NF2 patients treated with radiation for benign tumors. Controls were matched for treatment location with surgical/medical treatments based on age and year of treatment. Prospective data collection began in 1990 with addition of retrospective cases in 1969. Kaplan-Meier analysis was performed for malignancy incidence and survival. Outcomes were central nervous system (CNS) M/MP (2cm annualized diameter growth) and survival from index tumor treatment.<BR><B>UNASSIGNED: </B>In total, 1345 NF2 patients, 266 (133-Male) underwent radiation treatments between 1969 and 2021 with median first radiotherapy age of 32.9 (IQR = 22.4-46.0). Nine subsequent CNS malignancies/MPs were identified in cases with only 4 in 1079 untreated (P < .001). Lifetime and 20-year CNS M/MP was ~6% in all irradiated patients-(4.9% for vestibular schwannomas [VS] radiotherapy) versus <1% in the non-irradiated population (P < .001/.01). Controls were well matched for age at NF2 diagnosis and treatment (Males = 133%-50%) and had no M/MP in the CNS post-index tumor treatment (P = .0016). Thirty-year survival from index tumor treatment was 45.62% (95% CI = 34.0-56.5) for cases and 66.4% (57.3-74.0) for controls (P = .02), but was nonsignificantly worse for VS radiotherapy.<BR><B>UNASSIGNED: </B>NF2 patients should not be offered radiotherapy as first-line treatment of benign tumors and should be given a frank discussion of the potential 5% excess absolute risk of M/MP.