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\begin{document}

\title{A HOMOGENEOUS TREATMENT FOR NON-DIPG DIFFUSE MIDLINE GLIOMA}



\author[1]{Elisabetta Schiavello}%
\author[1]{Veronica Biassoni}%
\author[1]{Giovanna Gattuso}%
\author[1]{Marta Podda}%
\author[1]{Stefano Chiaravalli}%
\author[1]{Francesco Barretta}%
\author[2]{Manila Antonelli}%
\author[1]{Loris De Cecco}%
\author[1]{emilia pecori}%
\author[1]{lorenza gandola}%
\author[1]{Maura Massimino}%
\affil[1]{Fondazione IRCCS Istituto Nazionale dei Tumori}%
\affil[2]{University of Rome La Sapienza}%


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  \date{May 25, 2024}


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\begin{abstract}
Introduction. The H3K27M-mutant diffuse midline glioma (DMG) was first
included in the WHO Classification of CNS (central nervous system)
tumors in 2016, and confirmed in its fifth edition. The biological
behavior and dismal prognosis of this tumor resemble DIPG (diffuse
intrinsic pontine gliomas). Homogeneously-treated series are rarely
reported. Methods. From 2016 onwards, we treated patients with DMG with
radiotherapy and concomitant/adjuvant nimotuzumab/vinorelbine, plus
re-irradiation at relapse, as already done for DIPG
(DOI10.1007/s11060-014-1428-z). Results. We treated nine patients, seven
females, median age at diagnosis of 13 years-old. Tumor sites were:
thalamic in five cases, pontocerebellar in two, pineal in one, and
paratrigonal with nodular/leptomeningeal dissemination in one. Three
patients were biopsied, and six had partial tumor resections. Central
review of the pathologists' diagnoses was performed. The median time to
local progression was 12.7 months, and the median overall survival was
17.8 months. Six patients died of tumor progression, one of cerebral
bleeding whose tumor was progressing. Two were alive, one in continuous
remission, the other after a relapse, at 38.6 and 46.3 months after
diagnosis, respectively. Progression-free survival was 33.3\% at one
year. Overall survival was 88.9\%, 33.3\% and 22.2\% at 1, 2 and 3
years, respectively. Conclusions. This is one of only a handful of
reports on homogeneously-treated series. The results obtained are
comparable with those seen in patients with DIPG. Given the
phenotypically- and molecularly-defined setting of DMG and severe
outcome in this orphan population, they should be treated and included
in registries and protocols of DIPG.%
\end{abstract}%



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