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Diagnostic utility of NCOA2 fluorescence in situ hybridization and Stat6 immunohistochemistry staining for soft tissue angiofibroma and morphologically similar fibrovascular tumors.
[liposarcoma]
Soft
tissue
angiofibroma
(
STA
)
,
a
recently
suggested
new
histologic
entity
,
is
a
benign
fibrovascular
soft
tissue
tumor
composed
of
bland
spindle-shaped
tumor
cells
with
abundant
collagenous
to
myxoid
stroma
and
branching
small
vessels
.
The
lesion
has
a
characteristic
AHRR
-
NCOA
2
fusion
gene
derived
from
chromosomal
translocation
of
t
(
5
;
8
)
(
p
15
;
q
13
)
.
However
,
morphologically
similar
tumors
containing
abundant
fibrovascular
and
myxoid
stroma
can
complicate
diagnosis
.
We
designed
an
original
DNA
probe
for
detecting
NCOA
2
split
signals
on
fluorescence
in
situ
hybridization
(
FISH
)
and
estimated
its
utility
with
20
fibrovascular
tumors
:
4
each
of
STAs
,
solitary
fibrous
tumors
(
SFTs
)
,
and
cellular
angiofibromas
and
3
each
of
low
-grade
myxofibrosarcomas
,
myxoid
liposarcomas
,
and
low
-grade
fibromyxoid
sarcomas
.
We
also
performed
FISH
for
13
q
14
deletion
and
immunohistochemistry
(
IHC
)
staining
for
estrogen
receptor
,
progesterone
receptor
,
retinoblastoma
protein
,
and
MUC-
4
expression
.
Furthermore
,
IHC
for
Stat
6
was
conducted
in
the
20
cases
analyzed
by
FISH
and
in
an
additional
26
SFTs
.
We
found
moderate
to
strong
nuclear
Stat
6
expression
in
all
SFTs
but
no
expression
in
the
other
tumors
.
Both
estrogen
receptor
and
progesterone
receptor
expressions
were
observed
in
STAs
,
SFTs
,
and
cellular
angiofibromas
.
Expression
of
retinoblastoma
protein
was
found
in
less
than
10
%
of
cells
in
all
tumor
types
except
myxoid
liposarcoma
.
The
low
-grade
fibromyxoid
sarcomas
were
strongly
positive
for
MUC-
4
.
All
STAs
showed
NCOA
2
split
signals
on
FISH
.
All
tumors
,
regardless
of
histologic
type
,
had
13
q
14
deletion
.
The
NCOA
2
FISH
technique
is
a
practical
method
for
confirming
STA
diagnosis
.
The
combination
of
NCOA
2
FISH
and
Stat
6
IHC
proved
effective
for
the
differential
diagnosis
of
STA
,
even
when
using
small
biopsy
specimens
.
Diseases
Validation
Diseases presenting
"3 each of low-grade myxofibrosarcomas"
symptom
liposarcoma
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