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Sunitinib treatment for metastatic renal cell carcinoma in patients with von hippel-lindau disease.
[von hippel-lindau disease]
Von
Hippel-
Lindau
(
VHL
)
disease
is
an
autosomal
dominant
disease
that
produces
a
variety
of
tumors
and
cysts
in
the
central
nervous
system
and
visceral
organs
,
including
renal
cell
carcinoma
(
RCC
)
.
RCC
in
patients
with
VHL
disease
does
not
frequently
metastasize
,
therefore
,
the
response
to
treatment
and
prognosis
of
metastatic
RCC
developed
in
patients
with
VHL
disease
has
not
been
reported
.
Sunitinib
is
an
oral
,
multitargeted
receptor
tyrosine
kinase
inhibitor
with
antiangiogenic
and
antitumor
activity
.
Here
,
we
report
on
four
patients
with
metastatic
RCC
in
VHL
disease
who
received
sunitinib
and
achieved
partial
responses
that
have
lasted
for
a
prolonged
period
of
time
.
