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Molecular genetics of meningiomas: Building the roadmap towards personalized therapy.
[proteus syndrome]
New
advances
have
recently
been
made
in
the
field
of
molecular
genetics
and
mouse
modeling
of
meningiomas
,
opening
new
perspectives
for
future
treatments
.
Recent
genome-
wide
genotyping
and
exome
sequencing
studies
have
confirmed
the
pivotal
role
of
NF
2
in
meningioma
tumorigenesis
,
concerning
roughly
half
of
the
tumors
,
and
unraveled
new
mutations
in
non-
NF
2
meningiomas
concerning
AKT
1
,
SMO
,
KLF
4
and
TRAF
7
.
The
molecular
mechanisms
underlying
tumorigenesis
of
high
histological
grades
have
been
progressively
deciphered
with
the
recent
discovery
of
TERT
promoter
mutations
in
progressing
tumors
.
A
better
understanding
of
the
genetics
and
clinical
behavior
of
high
-grade
meningiomas
is
mandatory
in
order
to
better
design
future
clinical
trials
.
New
genetically
engineered
mouse
models
of
benign
and
histologically
aggressive
meningioma
represent
a
substantial
resource
for
the
establishment
of
relevant
pre-clinical
trials
.
By
studying
the
mechanisms
underlying
these
new
tumorigenesis
pathways
and
the
corresponding
mouse
models
,
we
should
be
able
to
offer
personalized
chemotherapy
to
patients
with
surgery-
and
radiation-refractory
meningiomas
in
the
near
future
.
