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@ARTICLE{Cloughesy:305604,
author = {T. F. Cloughesy and M. J. van den Bent and M. Touat and D.
T. Blumenthal and K. B. Peters and B. M. Ellingson and J. L.
Clarke and J. Mendez and S. Yust-Katz and L. Welsh and W. P.
Mason and F. Ducray and Y. Umemura and B. Nabors and M.
Holdhoff and A. F. Hottinger and Y. Arakawa and J. M.
Sepulveda and W. Wick$^*$ and R. Soffietti and J. Perry and
P. Giglio and M. de la Fuente and E. Maher and A. Bottomley
and A. E. Tron and D. Yi and D. Zhao and S. S. Pandya and L.
Steelman and I. Hassan and P. Y. Wen and I. K. Mellinghoff},
collaboration = {I. t. investigators},
title = {{V}orasidenib in {IDH}1-mutant or {IDH}2-mutant low-grade
glioma ({INDIGO}): secondary and exploratory endpoints from
a randomised, double-blind, placebo-controlled, phase 3
trial.},
journal = {The lancet / Oncology},
volume = {nn},
issn = {1470-2045},
address = {London},
publisher = {The Lancet Publ. Group},
reportid = {DKFZ-2025-02254},
pages = {nn},
year = {2025},
note = {epub},
abstract = {In a phase 3 trial, vorasidenib, an oral brain-penetrant
inhibitor of mutant isocitrate dehydrogenase 1 and 2
(IDH1/2), resulted in improved progression-free survival
(primary endpoint) and time to next intervention (key
secondary endpoint) at second interim analysis, resulting in
study unblinding. We report 6 months of additional
double-blind data, from second interim analysis (Sept 6,
2022) to unblinding (March 7, 2023), and the effect of
vorasidenib on volumetric tumour growth rate, health-related
quality of life (HRQOL), neurocognitive function, and
seizure control.INDIGO was a randomised, double-blind,
placebo-controlled, phase 3 trial done in 92 hospitals in
Canada, France, Germany, Israel, Italy, Japan, the
Netherlands, Spain, Switzerland, the UK, and the USA.
Patients aged 12 years or older with residual or recurrent
grade 2 IDH1/2-mutant diffuse glioma, a Karnofsky
performance-status score of 80 or higher, at least one
previous surgery, and no other previous anticancer treatment
were eligible. Patients were randomly assigned (1:1;
stratified according to locally determined chromosome 1p/19q
codeletion status and baseline tumour size) to oral
vorasidenib (40 mg) or placebo once a day in continuous
28-day cycles until disease progression or unacceptable
toxicity. Progression-free survival per masked independent
review committee was the primary endpoint, and time to next
intervention was the key secondary endpoint. Prespecified
secondary endpoints included tumour growth rate (6-monthly
change in tumour volume) and HRQOL (Functional Assessment of
Cancer Therapy-Brain [FACT-Br]). Prespecified exploratory
endpoints included neurocognitive function (cognitive
performance instruments) and seizure activity
(self-reported). The full analysis set was used for all
efficacy analyses and included all randomly assigned
patients, and the safety analysis set was used for all
safety analyses and included all patients who received one
or more doses of vorasidenib or placebo. The trial is
registered with ClinicalTrials.gov, NCT04164901. Recruitment
is complete and the trial is ongoing.Between Jan 9, 2020,
and Feb 22, 2022, 331 patients were enrolled and randomly
assigned to vorasidenib (n=168) or placebo (n=163). 187
$(56\%)$ patients were male, 144 $(44\%)$ were female, and
257 $(78\%)$ were White. Median follow-up was 20·1 months
(IQR 15·9 to 23·8). With an additional 6 months of
follow-up, median progression-free survival (not reached
$[95\%$ CI 22·1 to not estimated] vs 11·4 months $[95\%$
CI 11·1 to 13·9]; hazard ratio [HR] 0·35 $[95\%$ CI 0·25
to 0·49]) and time to next intervention (not estimated [not
estimated to not estimated] vs 20·1 months [17·5 to
27·1]; HR 0·25 [0·16 to 0·40]) remained substantially
improved with vorasidenib versus placebo. Tumour growth rate
was $-1·3\%$ $(95\%$ CI -3·2 to 0·7) with vorasidenib and
$14·4\%$ $(95\%$ CI 12·0 to 16·8) with placebo
(difference $15·9\%$ $[95\%$ CI 12·6 to 19·3]). Mean
FACT-Br total scores were similar between the vorasidenib
and placebo groups (158·2 [SD 26·4] and 158·8 [23·3]) at
baseline and remained high (154·2 [29·8] and 153·2
[29·4]) by the end of treatment. There was no difference
between vorasidenib or placebo in neurocognitive functions
of verbal learning, executive function, attention, working
memory, and psychomotor function from baseline through to
end of treatment. The vorasidenib group had lower rates of
seizures than the placebo group (18·2 seizures per
person-year $[95\%$ CI 8·4 to 39·5] vs 51·2 seizures per
person-year [22·9 to 114·8]). The most common grade 3 or
worse treatment-emergent adverse events (TEAEs) in the
vorasidenib and placebo groups, respectively, were increased
alanine aminotransferase (17 $[10\%]$ and two $[1\%]),$
increased aspartate aminotransferase (eight $[5\%]$ and
none), seizures (seven $[4\%]$ and five $[3\%]),$ and
increased γ-glutamyltransferase (five $[3\%]$ and two
$[1\%]).$ Serious TEAEs occurred in 20 $(12\%)$ patients in
the vorasidenib group and ten $(6\%)$ in the placebo group;
the most common were seizures. There were no
treatment-related deaths.Vorasidenib reduced tumour growth
rate and improved seizure control compared with placebo,
with no observed negative effects on HRQOL or
neurocognition. Additional follow-up supported the
robustness of progression-free survival and time to next
intervention in patients with grade 2 IDH1/2-mutant diffuse
glioma. These findings support the use of vorasidenib in
patients with grade 2 IDH1/2-mutant gliomas who only had
surgical intervention and are not in immediate need of
radiotherapy or chemotherapy.Servier.},
cin = {B320},
ddc = {610},
cid = {I:(DE-He78)B320-20160331},
pnm = {312 - Funktionelle und strukturelle Genomforschung
(POF4-312)},
pid = {G:(DE-HGF)POF4-312},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:41175888},
doi = {10.1016/S1470-2045(25)00472-3},
url = {https://inrepo02.dkfz.de/record/305604},
}