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[Clinical analyses of bilateral renal angiomyolipomas associated with tuberous sclerosis complex].
[lymphangioleiomyomatosis]
To
explore
the
clinical
characteristics
,
diagnosis
,
treatment
and
outcomes
of
renal
angiomyolipoma
(
RAML
)
associated
with
tuberous
sclerosis
complex
(
TSC
)
.
The
clinical
data
were
analyzed
for
10
cases
of
renal
angiomyolipomas
associated
with
tuberous
sclerosis
.
There
were
4
males
and
6
females
with
a
mean
age
of
23
.
5
(
9
-
44
)
years
.
Five
patients
were
admitted
for
flank
pain
or
hematuria
.
RAML
was
found
by
TSC
associated
imaging
examinations
for
those
with
extra
renal
organ
involvement
.
Bilateral
multiple
renal
angiomyolipomas
were
all
confirmed
on
imaging
tests
and
consistent
with
clinical
TSC
.
The
maximal
diameter
of
RAMLs
was
from
2
to
15
(
7
.
3
±
3
.
5
)
cm
.
Six
patients
of
tumor
hemorrhage
had
an
average
maximal
diameter
of
9
.
2
cm
.
Selective
arterial
embolization
(
SAE
)
(
n
=
4
)
and
homeostasis
,
antiinflamation
and
immobilization
therapy
(
n
=
2
)
were
performed
.
Four
asymptomatic
cases
accepted
close
observation
.
During
a
follow-up
period
of
9
-
116
months
,
one
of
4
SAE
cases
underwent
secondary
SAE
due
to
rebleeding
12
months
later
while
there
was
no
recurrence
of
flank
pain
in
other
3
cases
.
The
mean
enlargement
of
maximal
diameter
was
1
.
5
cm
/
year
and
serum
creatinine
elevated
by
3
.
1
µmol
/
L
/
year
.
Six
cases
on
conservative
medical
therapy
or
under
observation
had
a
mean
enlargement
of
maximal
diameter
of
0
.
7
cm
/
year
and
serum
creatinine
elevated
by
2
.
2
µmol
/
L
/
year
.
Three
cases
remained
asymptomatic
,
one
died
of
respiratory
failure
from
TSC-associated
pulmonary
lymphangioleiomyomatosis
,
two
had
flank
pain
at
16
and
40
months
respectively
,
one
underwent
secondary
SAE
and
another
received
rapamysin
with
marked
tumor
regression
.
Bilateral
multiple
RAMLs
occur
in
most
TSC
patients
.
After
excluding
malignancy
,
SAE
becomes
an
important
choice
for
its
mini-invasiveness
,
efficacy
and
chances
of
re
-embolization
.
And
rapamysin
provides
ameliorative
treatment
for
TSC
patients
.
