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Nephron sparing surgery for unilateral Wilms tumor in children with predisposing syndromes: single center experience over 10 years.
[aniridia]
Unilateral
Wilms
tumors
associated
with
predisposing
syndromes
are
treated
with
preoperative
chemotherapy
followed
by
surgical
resection
.
We
describe
our
experience
with
nephron
sparing
surgery
for
Wilms
tumor
in
this
population
at
risk
for
metachronous
lesions
.
We
conducted
a
retrospective
review
of
all
children
with
a
predisposing
syndrome
who
underwent
nephrectomy
for
malignancy
during
a
10
-
year
period
(
2000
to
2010
)
.
Data
collected
included
age
,
mode
of
detection
,
tumor
size
,
treatment
,
pathology
results
,
followup
time
and
recurrence
episodes
.
From
2000
to
2010
,
13
of
75
(
19
%
)
patients
treated
for
Wilms
tumor
were
diagnosed
with
predisposing
syndrome
(
s
)
.
Eight
patients
with
unilateral
tumors
were
treated
and
had
a
mean
age
at
diagnosis
of
27
months
(
range
7
months
to
9
years
)
.
Beckwith-
Wiedemann
syndrome
,
isolated
hemihyperplasia
,
WAGR
(
Wilms
tumor
,
Aniridia
,
Genitourinary
abnormalities
,
mental
Retardation
)
syndrome
and
isolated
11
p
13
deletion
were
the
underlying
diagnoses
in
3
,
2
,
2
and
1
patient
,
respectively
.
All
but
2
patients
were
diagnosed
by
screening
ultrasound
and
5
underwent
preoperative
chemotherapy
.
Median
tumor
size
at
surgery
was
2
.
5
cm
(
range
1
to
13
)
.
Nephron
sparing
surgery
was
performed
in
6
of
8
patients
.
Pathological
study
showed
favorable
histology
Wilms
tumor
and
nephrogenic
rests
in
6
and
2
patients
,
respectively
.
After
a
mean
followup
of
36
months
(
range
6
to
72
)
no
recurrences
were
documented
and
all
children
had
normal
creatinine
levels
.
Nephron
sparing
surgery
appears
safe
for
patients
with
unilateral
Wilms
tumor
associated
with
predisposing
syndrome
(
s
)
,
allowing
for
the
preservation
of
renal
function
and
good
oncologic
outcomes
for
the
available
followup
time
.
If
more
studies
confirm
our
observation
,
current
recommendations
for
the
surgical
treatment
of
Wilms
tumor
may
need
to
reemphasize
the
value
of
attempting
nephron
sparing
surgery
in
this
patient
population
.
Diseases
Validation
Diseases presenting
"mental retardation"
symptom
achondroplasia
alexander disease
alpha-thalassemia
aniridia
aromatase deficiency
canavan disease
classical phenylketonuria
coats disease
cohen syndrome
cowden syndrome
cystinuria
dentin dysplasia
familial hypocalciuric hypercalcemia
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
kabuki syndrome
kallmann syndrome
lamellar ichthyosis
lymphangioleiomyomatosis
monosomy 21
phenylketonuria
primary hyperoxaluria type 1
proteus syndrome
pyruvate dehydrogenase deficiency
sneddon syndrome
triple a syndrome
wolf-hirschhorn syndrome
zellweger syndrome
This symptom has already been validated