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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
.
Diseases
Validation
Diseases presenting
"central nervous system"
symptom
22q11.2 deletion syndrome
adrenomyeloneuropathy
alexander disease
aniridia
aromatase deficiency
canavan disease
child syndrome
classical phenylketonuria
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cystinuria
dracunculiasis
erdheim-chester disease
fabry disease
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
hirschsprung disease
hodgkin lymphoma, classical
kabuki syndrome
kallmann syndrome
kindler syndrome
krabbe disease
lamellar ichthyosis
legionellosis
liposarcoma
malignant atrophic papulosis
monosomy 21
neonatal adrenoleukodystrophy
phenylketonuria
proteus syndrome
scrub typhus
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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