Malignant fibrous histiocytoma of the abdominal cavity: report of a case


Atmatzidis, K.S.; Pavlidis, T.E.; Galanis, I.N.; Papaziogas, B.T.; Papaziogas, T.B.

Surgery Today 33(10): 794-796

2003


Malignant fibrous histiocytoma (MFH) is a soft-tissue sarcoma originating from fibroblast cells, characterized by a high rate of metastasis or recurrence. This tumor rarely develops in the gastrointestinal tract, with no more than 30 cases described in the literature. We report a case of MFH of the abdominal cavity in a 45-year-old woman who presented with epigastric pain, anorexia, and weight loss. A computed tomography (CT) scan of the abdomen revealed multiple solid tumors in the peritoneal cavity. We performed exploratory laparotomy and found at least 15 solid whitish tumors attached to the wall of the small intestine, as well as to the parietal peritoneum. There were three metastases in the liver. All of the tumors were excised, most of which were about 10 cm in diameter. Histopathological findings indicated a stromal tumor consisting of spindle cells, and immunohistochemical examination of the resected specimens established the definite diagnosis of a pleomorphic MFH. The patient had an uneventful postoperative course and was given adjuvant chemotherapy. She is currently well 2 years after her operation. We review the clinical picture of this tumor in the abdominal cavity, and discuss its diagnosis, pathogenesis, and treatment.

Surg
Today
(2003)
33:794-796
DOI
10.1007/s00595-003-2577-4
SURGERY
TODAY
©
Springer-Verlag
2003
Malignant
Fibrous
Histiocytoma
of
the
Abdominal
Cavity:
Report
of
a
Case
KONSTANTINOS
S.
ATMATZIDIS,
THEODOROS
E.
PAVLIDIS,
IOANNIS
N.
GALANIS,
BASILIOS
T.
PAPAZIOGAS,
and
THOMAS
B.
PAPAZIOGAS
Second
Surgical
Department,
Medical
Faculty,
Aristotle
University
of
Thessaloniki,
G.
Gennimatas
Hospital,
Ethnikis
Aminis
41,
546
35
Thessaloniki,
Greece
Abstract
Malignant
fibrous
histiocytoma
(MFH)
is
a
soft-tissue
sarcoma
originating
from
fibroblast
cells,
characterized
by
a
high
rate
of
metastasis
or
recurrence.
This
tumor
rarely
develops
in
the
gastrointestinal
tract,
with
no
more
than
30
cases
described
in
the
literature.
We
report
a
case
of
MFH
of
the
abdominal
cavity
in
a
45-
year-old
woman
who
presented
with
epigastric
pain,
anorexia,
and
weight
loss.
A
computed
tomography
(CT)
scan
of
the
abdomen
revealed
multiple
solid
tumors
in
the
peritoneal
cavity.
We
performed
explor-
atory
laparotomy
and
found
at
least
15
solid
whitish
tumors
attached
to
the
wall
of
the
small
intestine,
as
well
as
to
the
parietal
peritoneum.
There
were
three
metastases
in
the
liver.
All
of
the
tumors
were
excised,
most
of
which
were
about
10
cm
in
diameter.
Histo-
pathological
findings
indicated
a
stromal
tumor
con-
sisting
of
spindle
cells,
and
immunohistochemical
examination
of
the
resected
specimens
established
the
definite
diagnosis
of
a
pleomorphic
MFH.
The
patient
had
an
uneventful
postoperative
course
and
was
given
adjuvant
chemotherapy.
She
is
currently
well
2
years
after
her
operation.
We
review
the
clinical
picture
of
this
tumor
in
the
abdominal
cavity,
and
discuss
its
diag-
nosis,
pathogenesis,
and
treatment.
Key
words
Malignant
fibrous
histiocytoma
Soft
tissue
tumor
Stromal
tumor
Sarcoma
Storiform-
pleomorphic
lesion
Introduction
Malignant
fibrous
histiocytoma
(MFH)
is
a
pleomor-
phic
sarcoma,
the
malignant
potential
of
which
has
only
Reprint
requests
to:
I.N.
Galanis,
Kerasountos
6,
Kalamaria,
551
31
Thessaloniki,
Greece
Received:
Junuary
17,
2002
/
Accepted:
January
21,
2003
recently
been
recognized.
O'Brien
and
Stout
first
de-
scribed
this
tumor
in
1964.'
The
occurrence
of
this
le-
sion
in
the
gastrointestinal
tract
is
very
rare,
with
only
about
30
cases
documented
in
the
English
literature.
The
histologic
features
of
MFH
include
spindle
and
round
cells
arranged
in
a
storiform
pattern
interspersed
with
pleomorphic,
inflammatory,
and
giant
cells.
It
may
be
distinguished
from
other
soft-tissue
tumors
by
immu-
nocytochemical
staining?
In
adults,
this
malignant
tu-
mor
most
commonly
arises
in
the
deep
soft
tissues
of
the
extremities,
followed
by
the
trunk
and
retroperitoneum
in
decreasing
order
of
frequency.
Primary
gastrointes-
tinal
MFH
is
unusual
and
so
are
intestinal
secondary
sites,
which
have
also
occasionally
been
described.'
Pri-
mary
small
bowel
MFH
is
even
more
unusual.
To
our
knowledge,
only
five
cases
have
been
reported
in
the
Western
literature.
4
We
describe
a
unique
case
of
multi-
centric
MFH
of
the
small
intestine
and
peritoneum,
with
liver
metastases,
in
a
45-year-old
woman.
Case
Report
A
45-year-old
woman
was
admitted
to
our
Surgical
Department
with
a
history
of
persistent
epigastric
ab-
dominal
pain,
anorexia,
and
weight
loss
of
10
kg
in
the
3
months
before
admission.
The
patient
had
undergone
open
cholecystectomy
for
cholelithiasis
8
years
earlier,
and
a
total
hysterectomy
for
fibromyomatosis
6
years
earlier.
Symphysiolysis
had
been
performed
for
adhe-
sive
acute
intestinal
obstruction
5
years
before
this
admission.
She
had
a
long
history
of
vague,
nonspecific
gastrointestinal
tract
complaints.
Routine
laboratory
investigations,
including
biochemical
parameters,
blood
count,
and
erythrocyte
sedimentation
rate,
revealed
normal
results,
and
no
abnormalities
were
seen
on
chest
radiography.
Computed
tomography
(CT)
scanning
of
the
abdomen
demonstrated
multiple
solid
tumors
with
rich
vascularity
(Fig.
1).
The
latter
was
indicated
by
K.S.
Atmatzidis
et
al.:
Malignant
Fibrous
Histiocytoma
of
the
Abdomen
795
A
Fig.
1.
Computed
tomography
of
the
abdomen
showing
multiple
solid
tumors
with
rich
vascularity
X.
-
As.
'V
.
-?•••-%f2
1
".
Fig.
2.
Microscopy
showing
spindle
cells
in
a
storiform
pat-
tern,
with
nuclear
atypia
and
mitoses
(H&E
X100)
contrast
medium
within
the
masses.
After
consulting
an
oncologist,
we
decided
to
proceed
with
an
exploratory
laparotomy.
On
opening
the
abdomen,
we
found
at
least
15
solid
whitish
tumors
in
the
peritoneal
cavity,
without
any
adhesions
to
the
adjacent
tissues.
They
were
attached
to
the
parietal
peritoneum,
as
well
as
to
the
wall
of
the
small
intestine.
There
were
also
three
metastases
in
the
liver.
All
of
the
lesions
were
excised
with
debulking
surgery,
and
most
of
them
were
about
10
cm
in
diam-
eter.
Three
of
the
lesions
were
located
in
the
same
intestinal
loop
of
ileum,
which
was
resected
with
an
end-
to-end
anastomosis.
Another
four
lesions
were
found
in
different
parts
of
the
small
bowel,
all
of
which
were
completely
excised
by
performing
partial
resection
of
the
intestinal
wall.
The
other
lesions,
which
were
in
the
parietal
peritoneum,
were
resected
easily
and
the
defect
repaired.
Partial
hepatectomy
of
the
right
hepatic
lobe
was
done
to
excise
the
three
metastases.
Histopathological
examination
of
the
resected
specimens
indicated
a
mesenchymatous
stromal
tumor
consisting
of
spindle
cells.
Immunohistochemical
ex-
amination
of
multiple
sections
of
paraffin-embedded
tissue
revealed
a
mixture
of
pleomorphic
fibroblast-like
spindle
cells
arranged
in
fascicles
and
a
storiform
pat-
tern,
with
irregular,
prominent,
round-edged
nuclei
(Fig.
2).
Numerous
cells
were
positively
stained
for
vimentin
and
CD34
antigen.
Stains
for
desmin,
actin
of
smooth
muscular
fibers,
S-100
protein,
and
surface
membrane
antigen
were
negative.
Based
on
these
re-
sults,
a
definite
diagnosis
of
MFH
was
made.
The
patient
was
discharged
after
an
uneventful
postoperative
course.
She
was
given
adjuvant
chemo-
therapy
for
6
months,
comprising
six
consequent
ses-
sions,
with
3-week
intervals,
of:
epirubicin
70
mg/m
2
for
1
day,
efophosphamide
2
g/m
2
for
1-3
days,
and
methna
2
g/m
2
for
1-3
days.
The
patient
is
currently
well
2
years
after
her
operation,
without
any
evidence
of
recurrence.
Discussion
MFH
most
commonly
occurs
in
the
seventh
decade
of
life
and
men
are
more
often
affected
than
women.
The
lesion
usually
arises
in
the
extremities
(68%)
or
retro-
peritoneum
(16%),
and
often
involves
skeletal
muscle
(59%).
Most
MFHs
are
locally
invasive,
but
distant
metastasis
may
spread
via
the
blood
(30%)
or
lymphat-
ics
(12%).
The
risk
of
local
recurrence
and
distant
me-
tastasis
correlates
best
with
the
depth
and
size
of
the
primary
tumor.
The
overall
2-year
survival
rate
is
60%
and
another
20%
will
die
or
suffer
local
recurrence.
3-6
This
malignant
tumor
rarely
originates
in
the
alimen-
tary
tract.
Multifocal
MFHs
most
commonly
develop
in
bone,
and
only
occasionally
in
other
sites?
-1
°
In
fact,
we
were
able
to
find
only
five
cases
of
MFH
in
the
small
intestine
in
the
Western
literature,
making
our
case
ex-
ceedingly
rare.
The
two
major
clinical
manifestations
of
gastrointestinal
involvement
are
bleeding
from
ulcer-
ated
tumors
and
intussusception
caused
by
polypoid
tumors."'"
Our
patient
did
not
present
with
either
of
these
emergencies.
Although
MFH
is
considered
to
be
aggressive,
the
manifestations
in
our
patient
were
of
mild
intensity.
Moreover,
it
is
very
unusual
to
find
a
metastasis
from
an
MFH
before
discovery
of
the
pri-
mary
tumor.
5
In
our
patient,
it
was
impossible
to
distin-
guish
the
primary
site
of
the
lesion
within
the
abdomen
and
there
was
no
evidence
of
a
primary
tumor
in
the
extremities,
or
elsewhere
outside
the
peritoneal
cavity.
Our
patient's
surgical
history
supports
the
opinion
of
Froehner
et
al."
that
chronic
postoperative
repair
pro-
cesses
may
be
a
promoting
factor
in
the
tumorigenesis
of
this
neoplasm,
based
on
the
analogy
of
MFH
arising
in
different
sites.
Since
MFH
was
first
described
in
1964,
many
at-
tempts
have
been
made
to
define
its
pathogenesis
and
histogenesis.
Based
on
its
histopathologic
features,
MFH
has
been
categorized
into
storiform-pleomorphic,
796
K.S.
Atmatzidis
et
al.:
Malignant
Fibrous
Histiocytoma
of
the
Abdomen
myxoid,
inflammatory,
giant
cell,
and
angiomatoid
vari-
ants.
Among
these
types,
the
storiform-pleomorphic
is
most
common,
accounting
for
two
thirds
of
cases.
It
is
composed
of
spindle-shaped
cells
arranged
in
short
fas-
cicles
in
a
cartwheel
or
storiform
pattern,
along
with
plump
histiocyte
cells,
mitotic
features,
and
multinucle-
ated
giant
cells.
14
The
histologic
pleomorphism,
degree
of
vimentin
staining,
absence
of
the
smooth
muscle
marker
desmin,
and
the
macroscopic
and
microscopic
findings
of
the
resected
tumors
from
our
patient
favored
the
diagnosis
of
MFH.
Surgical
resection
is
the
treatment
of
choice,
and
de-
pends
on
the
stage
of
disease
and
the
depth
of
invasion
by
the
tumor.
Radiotherapy,
chemotherapy,
and
immu-
notherapy
are
other
therapeutic
modalities.
14
Long-
term
follow-up
with
regular
chest
X-rays
and
CT
scans
of
the
abdomen
to
detect
any
local
lymph
node
involve-
ment
is
mandatory."
We
considered
some
form
of
adjuvant
chemotherapy
necessary
for
our
patient
as
the
disease
was
extensive
with
liver
metastasis,
and
the
resection
was
not
curative.
She
responded
well
to
the
chemotherapeutic
regimen.
Although
there
is
some
conflict
as
to
whether
chemo-
therapy
contributes
significantly
to
the
outcome,
based
on
her
good
long-term
outcome,
we
consider
that
the
operative
procedure
and
chemotherapy
protocol
used
were
very
successful.
In
conclusion,
we
believe
that
re-
section
of
the
tumor
followed
by
adjuvant
chemo-
therapy
was
the
most
appropriate
treatment
for
this
unique
disease
in
a
relatively
young
woman.
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