Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Preclinical and clinical evidence of activity of pazopanib in solitary fibrous tumour.
[severe combined immunodeficiency]
To
explore
the
activity
of
pazopanib
in
solitary
fibrous
tumour
(
SFT
)
.
In
a
preclinical
study
,
we
compared
the
activity
of
pazopanib
,
sorafenib
,
sunitinib
,
regorafenib
,
axitinib
and
bevacizumab
in
a
dedifferentiated-
SFT
(
DSFT
)
xenotransplanted
into
Severe
Combined
Immunodeficiency
(
SCID
)
mice
.
Antiangiogenics
were
administered
at
their
reported
optimal
doses
when
mean
tumour
volume
(
TV
)
was
80
mm
(
3
)
.
Drug
activity
was
assessed
as
TV
inhibition
percentage
(
TVI
%
)
.
From
May
2012
,
six
consecutive
patients
with
advanced
SFT
received
pazopanib
,
on
a
national
name-based
programme
.
In
one
case
sunitinib
was
administered
after
pazopanib
failure
.
In
the
xenograft
model
,
pazopanib
showed
the
lowest
antitumour
activity
(
21
%
TVI
)
,
while
regorafenib
was
the
most
active
(
95
%
TVI
)
.
Sorafenib
,
bevacizumab
,
sunitinib
were
markedly
active
(
78
/
70
/
65
%
TVI
)
.
Axitinib
was
marginally
active
(
51
%
TVI
)
.
In
the
retrospective
case-series
,
three
patients
carried
malignant
-
SFT
(
MSFT
)
,
three
DSFT
.
Best
Response
Evaluation
Criteria
in
Solid
Tumour
(
RECIST
)
responses
were
:
three
stable
disease
(
SD
)
,
all
MSFT
,
three
progressive
disease
(
PD
)
,
all
DSFT
,
corresponding
to
one
partial
response
(
PR
)
,
two
SD
,
three
PD
by
Choi
criteria
.
Median-progression-free
survival
was
3
months
(
range
1
-
15
)
.
In
one
patient
,
sunitinib
was
started
after
pazopanib
failure
,
with
a
response
.
In
dedifferentiated-
SFT
xenograft
pazopanib
induced
a
marginal
antitumour
activity
,
while
regorafenib
appeared
the
most
active
and
promising
agent
.
When
administered
in
patients
,
pazopanib
showed
a
modest
activity
in
terms
of
tumour
growth
stabilisation
,
observed
only
in
non-dedifferentiated
cases
.
Diseases
Validation
Diseases presenting
"immunodeficiency"
symptom
adrenal incidentaloma
allergic bronchopulmonary aspergillosis
cushing syndrome
dracunculiasis
hirschsprung disease
hodgkin lymphoma, classical
homocystinuria without methylmalonic aciduria
kabuki syndrome
legionellosis
malignant atrophic papulosis
oculocutaneous albinism
omenn syndrome
papillon-lefèvre syndrome
primary effusion lymphoma
primary hyperoxaluria type 1
pyomyositis
severe combined immunodeficiency
sneddon syndrome
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated