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Pseudotumor
of Hemophilia
with
R.
By
LTHOUGH
tions
also
may
the
PTC
SJLBER
Deficiency
W.
AND
hemarthroses
in a Patient
of
B.
CHRISTENSEN
hemophilia
are
osteolytic
lesions
are generally
accompanied
and usually,
therefore,
are called
pseudotumors
and
roentgenologic
manifestations
may
be
malignant
in
osteolytic
the
tumors.
literature.
following
This
case
report
A total
rare
of a patient
L.W.:This
27-year-old
the
for
patient
had
Slight
hip.
a period
mass
appeared
in
the
thigh.
right
the
PTC
the
other
3,
of
5,
knee
symptoms
the
patient
had
trauma
to
profuse
for
10
hemarthroses.
at
the
treatment
the
Because
of
referred
About
sustained
of
However,
three
of
course
this,
but
months
the
of
10.
severe
Five
had
years
and
and
the
the
over
the
the
right
a painless
this
gradually
anterior
aspect
which
lasted
bleeding
into
A
of
blood
In
the
and
and
carefully
maternal
joint.
of
transfusions
hematuria
occupation
the
2 weeks.
the
hemarthrosis
bleeding.
of
on
of
period.
gingival
a cousin
General
admission,
accident,
multiple
bouts
to
spontaneously
months
by
later,
Lake
region
extractions.
a sedentary
uncle
the
nine
this
dental
repeated
sought
grand
in
subsided
after
followed
epistaxis
noted
prior
epistaxis
was
after
Salt
to
following
during
the
year
this
developed
knee
noted
had
he
A
trauma
a spontaneous
left
age
of
minor
noted
suffered
patient
exercise.
been
to
one
trauma,
Numbness
was
the
the
the
the
bleeding
years,
physical
was
mass.
had
recorded
deficiency.
draftsman
site
In
been
illustrated
REPORT
a watermelon.
developed
following
strenuous
flank.
of
minor
required
the
No
of
age
right
were
that
the
bleeding
Such
tissue
swelling
The
clinical
from
those
of
have
is
abdominal
and
at
weeks.
right
to
later,
the
a staircase
four
the
an
noted
about
size
age
year
At
on
was
in
increased
At
fallen
of
male
regarding
swelling
over
One
white
consultation
cases
complication
with
manifesta-
abnormal
articulations.
massive
soft
of hemophilia.
indistinguishable
18 such
serious
that
the
by
of only
but
CASE
Hospital
well-known
of this disease,
it is not commonly
realized
cause
bone
destruction
in sites remote
from
side
course
of
numerous
avoided
were
known
hemophiliacs.
Physical
vital
examination
signs
of
the
not
of
A
was
as
No
the
joints
and
as
obese
mass
discrete
and
posteriorly
into
was
The
revealed
male,
bulging
tenderness
hemorrhage.
was not otherwise
an
large,
firm
well
warm.
accompanying
tion
It
ligament
was
revealed
normal.
abdomen.
inguinal
skin
were
mass
limitation
who
was
was
neither
readily
extended
the
region
from
the
the
right
of
elicited
on
was
readily
palpable
of
abduction
pressure.
of
pale
on
costa!
rectal
right
icteric.
the
The
side
was
no
to
the
overlying
evidence
examination.
hip.
The
right
margin
flank.
There
on
the
nor
apparent
of
Examina-
Physical
examination
remarkable.
From the Departments
Salt Lake City, Utah.
of
Niedicine
and
Radiology,
University
of
Utah,
College
of
Medi-
cine,
One
of
the
authors
(R.S.)
is
a
Trainee,
National
Cancer
Institute,
Public
Health
Service.
The authors
acknowledge
Maxwell
Wintrobe,
Ceorge
with
E.
sincere
Cartwright
appreciation
and
the
Russell
S.
advice
Jones
paper.
Submitted
July
7,
1958;
accepted
for
publication
584
July
25,
1958.
in
and
assistance
the
preparation
of
of
Drs.
this
From www.bloodjournal.org by guest on June 17, 2017. For personal use only.
PSEUDOTUMOR
Fic.
The
volume
7850
per
of
platelets
was
216,000
per
The
not
cu.mm.
The
to
Additional
defect
of
sky
units;
mg.
per
X-ray
the
The
serum
hundred
ml.
of
intact.
the
Only
major
demonstrable.
soft
new,
mass
The
tissue
gested
either
to
mass.
that
by
to
PA
film
the
extensive
a neoplasm,
from
of
the
chest
soft
such
as
ilium
and
tissue
osteogenic
of
ilium
calcification
apparent
a skeletal
and
sarcoma,
survey
the
in
of
pelvis.
bone
a
all
the
coagulation
5.5
the
not
were
no
longer
except
pos-
induced
in
body
remarkable.
had
of
well-defined
and
main
hematoma.
3.5
remained
been
medial
of
of
It
The
some
its
fragments
destruction
Bodan-
destruction
present
within
large
the
directions
were
irregular
normal
was
fairly
ilium
was
of
phosphorus
body
Some
were
or
the
15
hours.
defect
structure
lobulated,
fragments
of
serum
bony
of
a large,
the
The
irregular
this
portion
of
the
were
mass
of
with
flecks
midline
the
extensive,
lateral
body
and
two
addition
correct
was
was
after
phosphatase
ml.
showed
discrete
Irregular
the
of
the
the
not
and
)
Lee-\Vhite)
the
was
Cronkite
abnonnal.
did
count
erythrocytes
good
after
alkaline
portions
and
was
serum
hundred
associated
displaced
formation.
remnants
crest
)
(
time
normal
The
1)
blood
the
and
was
Douglas
inferior
of
Schneiderman
became
per
1956.
white
Clotting
patient’s
(fig.
was
clearly
extended
be
the
extended
had
bone
which
presumed
which
this
and
defect
and
test
The
retraction
deficiency.
hips
ml.
3,
morphology
negative.
clot
mg
medial
of
bony
mass
periosteal
portion,
and
portion
This
tissue
teriorly.
that
10.8
the
was
the
PTC
calcium
abdomen
ilium.
The
showed
100
( Brecher,
( Biggs
since
October
The
Qualitative
known
pelvis,
ml.!
count
test
serum
with
42
test
).
of
normal.
platelet
tourniquet
studies
the
was
was
minutes
patient’s
a patient
right
cells
generation
the
serum.
red
The
12
thromboplastin
localized
film
differential
unusual.
( control,
minutes
1.-Anteroposterior
packed
cu.mm.
585
HEMOPHILIA
OF
bone
the
was
produced
irregularity
soft
sug-
From www.bloodjournal.org by guest on June 17, 2017. For personal use only.
586
SILBER
of
tile
of
the
defect,
OSSCOUS
mass
periosteal
and
the
consideration.
The
he be hospitalized,
An
ordinary
A small,
spine.
forth.
The
men
residual
at
seen.
only
to
The
On
its
and
4,
The
continued
tempt
to
large
plasma
plasma
to
by
size.
was
also
I)eterioration
and
he
cultured
became
disoriented.
improve
the
following
death
despite
the
the
with
that
this
cavity
in
the
day
the
tumor.
speci-
elements
the
The
hematoma
an
effusion
which
patient
had
outcome,
its
original
of
pseudotumor
ml.
of
anemia
was
continued
to
decreased
Despite
continuous
1957.
The
same
simultaneously.
patient
became
These
symptoms
grand
confused
did
mal
not
seizures
patient’s
abdominal
of
hospitalization.
size.
This
and
mass
had
At
decrease
repeatedly
at-
repeated,
slowly
months
had
an
750
His
appeared
several
the
of
May.
February
The
four
average
course.
in
aureus
In
of
wound
downhill
the
size.
however,
septicemia
General
staph.
regime
to
mass,
appeared.
Lake
in
operative
flank
last
Salt
a
December
edema
1/3
the
disease,
complication.
the
about
of
unchanged
and
fatal
during
was
the
the
size
formed
a hemolytic
received
from
pleural
23,
Despite
in
swelling
fact
May
of
had
emptied
the
occurred
and
refilled
blood.
Radiographs
ment
in
obtained
appearance.
constitution
in
day.
decreased
of
after
On
patient
a desultory
dyspnea
gushed
of the
No
to
with
of
staphylococcic
rapid
digitalization.
progressively
time
more
iliac
blood
evidence
evacuation
essentially
and
sided
clot.”
hospital.
superior
of old
no
that
another
examination
with
transfusions.
a left
in
was
transferred
hospital
pursued
from
there
infected
was
abdominal
a
Cardiomegaly,
with
expired
patient
developed
serious
week.
The
the
The
periphery
a
anterior
ml.
blood
progression
blood
material.
the
therapy,
in
1956
Pathologic
again
tumor
initiated.
stay
but
was
prevent
whole
bloody
Nevertheless,
organism
his
numerous
purulent,
antibiotic
was
during
clots,
disappeared
was
The
the
malignancy
right
1000
closed.
unhealed,
and
transfusions
old
following
patient
blood.
bleeding
day
controlled
in
remained
ooze
control
per
drain
the
site
17,
the
“degenerating
the
at
made
October
below
wound
largely
over
calcification
tumor
approximately
only
swelling
1956,
biopsy
on
a few
the
showed
size
tissue
CHRISTENSEN
to his physician
with
the recommendation
and that a needle
biopsy
obtained.
opened,
and
irregular
soft
immediately
contained
original
December
Hospital.
was
inserted
surgery
formation,
the
attempted
made
lata
abdominal
regain
was
was
cavity
was
of
returned
with plasma
biopsy
fascia
drain
obtained
were
was
incision
the
Penrose
A
surgical
bone
size
patient
transfused
vertical
\Vhen
new
unusual
AND
of
size.
The
contour.
the
gross
bulky,
ilium
crest
A
on
The
had
and
lateral
lesion
was
of
soft
taken
place.
border
of
death
( fig.
tissue
mass
The
the
obviously
2)
no
of
erosion
area
ilium
still
demonstrated
as
had
present
considerable
longer
had
approached
but
Definite
decreased
considerably
a
a striking
improve-
apparent.
fairly
re-
normal
response
to
bloody
necrotic
external
therapy
had
occurred.
Autopsy
Findings
The
right
iliac
measuring
ilium.
The
pus
was
psoas
seen.
revealed
no
fossa
evidence
polymorphonuclear
lobe
damage
with
tis
noted
and
all
were
necrotic
tions
stained
and
which
brain.
invaded
but
were
the
proliferation
on
microscopic
The
with
Unfortunately,
this
pleura
of
the
stages
an
was
pulmonary
no
debilitated,
cultures
in
sarcolemmal
hemorrhagic
brain
tissue
heart.
and
within
were
from
the
obtained
the
were
large
to
the
is,
exact
showed
in
of
of
was
present
small.
was
present
Zones
was
The
There
areas
a
myocardiin
vessel
present
unknown.
of
the
arteritis
walls
leukocytes.
nature
the
neuronal
diffuse
The
zones
therefore,
gross
evident.
was
polymorphonuclear
inflammatory
right
No
hematonia
nuclei.
showed
There
the
sections
infiltration.
and
patient
of
Hemosiderosis
fibrotic.
and
wall
area
also
cell
the
artery,
antibiotic-treated
the
Numerous
increase
macrophages
mycelia
from
material
in
consistency.
formation
of
defect
flabby
bone
large
thickened
large
new
A
of
of
taken
of
with
of
a
macrophages.
polymorphonuclear
with
showed
and
sections
Sections
mass
was
color
laden
infiltration.
infiltrated
PAS
of
atrophic
and
defined
There
in
examination
left
branches
and
brown
various
brain.
glial
spleen.
involving
of
a poorly
diameter.
hemosiderin
leukocytic
parietal
were
dark
and
neoplasm,
fibers
in
examination
changes
muscle
by
cm.
was
Microscopic
of
adjoining
liver
occupied
10
muscle
fibrotic
right
was
approximately
Secin
the
lung
fungus
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PSEUDOTUMOR
OF
587
HEMOPHILIA
Fic.
2.-Anteroposterior
film
of pelvis,
May
24,
1957.
DISCUSSION
The
first
1918.1:1
because
of
8 months
were
seen
into
report
He
the
blood
patient
hemopitilic
a
a large
tumor
swelling
was
These
have
of the
surrounding
Little
been
soft
is
of hemophilia.
The
tissue.
the process
producing
bleeding
originates
regarding
exact
thigh.
large
the
anatomic
anemic
evidence
of
destructive
bleeding
to
bone
destruction
under
the periosteum
and
this
been
vell
physiology
of the
are
occurred
and
750
in
hemorrhage
uncertain.
and
produces
of
The
in 10
bones
(table
1).
swelling
pronounced
of
ml.
refilled.
expired
neoplasm.
changes
“pseudotumor
have
pathologic
site
and
aid
of the femur
with
bleeding
tendencies1’4’5’6’8’2
hematomas
names,
had
probed
4 days
in
medical
trauma
in
Starker
by
sought
thinning
the bone
was
sac.
became
with
The
Slight
swelling
tumor
fever,
with
Presented
who
and
periosteal
of sarcoma
of
revealed
no
extra-articular
of bone”1#{176}appear
known
right
of the
in 18 patients
associated
tumor
his
The
collapse
developed
examination
original
report,
reported
“resorption
in
was
hemophiliac
destruction
the diagnosis
entertained.
aspirated,
with
subsequently
been
Pseudt1mor
14-year-old
earlier.
Cortical
on x-ray
and
days.
Pathologic
Since
this
have
of
described
hemophilia”4
or
accepted.
of
this
and
complication
the
It is possible
erosion
by
nature
of
that
the
pressure
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588
SILBER
from
the
outside;
increasing
hematomas
In
also
patient
been
noted
in dogs
taming
ceivable
originate
hematoma
in
by
the
through
the
other
Others
extension
from
was
under
saline
At
present
hemophilia
there
possesses
As has
deficiency.
has
into
their
continuously
account
for
is
the
evidence
that
osteolytic
potency
the
in the development
Development
in only
eight
in that
months
of signs
swelling
and
of
therefore,
exact
of
amount
preceded
cases.
The
may
elapse
or even
years
)
.
The
customary
duration.
formation
osteitis
include
fibrosa
static
normal
ulna,
tibia
and
secondary,
osteogenic
solitary
and
osteogenic
are clearly
sarcoma,
important
may
also
bleeding
tendencies
a possibility
in
even
amputation.
Needle
incision.
even
are
However,
this
presents
continually
Therapy,
in
in
PTC
plasma
deficient.
assumption
may
be
Roentgen
that
presence
considerable
present.
our opinion,
it hastens
a
may
aspiration
the
the underlying
coagulation
plasma
transfusions
over
or stored
develops
hemophilia
risk.
should
slowly
cell
is a safer
The
dangers
aimed
at
primary
clinical
festures
diagnosis
of
a patient
with
of
Awareness
exploration
technic
in
tumor,
meta-
both
patient
biopsy
a hematoma
may
new
Differentiation
It is apparent
mass.
the
The
which
and
giant
cell
sarcoma,
malignancies,
growing
save
of
be
affected.
considerations.
he most
difficult.
vell
appearance
lesions
elevation
the characteristics
listed
above
that
all radiologic
and
osteogenic
sarcoma
may
be duplicated.
A presumptive
pseudotumor
should
probably
be made
in any instance
where
such
injury
been
painless
involved
is
been
an
known
factors
of
the
from
of
bleeding
is questionable
deformans,
The
sarcoma.
the most
in particular,
of
and
reticulum
with
primary
history
osteolytic
periosteal
con-
due to a PTC
in this disorder.
the
injury
osteitis
of
main-
It is contumor
of
duration
are
have
plasmacytoma,
has
patients
complaint
has
most
frequently
thumb
This
blood.12
was
a definite
between
osteomyelitis,
cystica,
malignancy
and
from
of
of trauma
presenting
The
bone
the
in the case
of
or an intramedullary
blood
and
by
by
that
and
12 hours.1’
“resorption
met
space
importance
effect
necrosis
cavities
for
the
defect
illium
has been
the only flat bone
involved.
The
differential
diagnosis
of such
localized
show
varying
degrees
of irregular
margination,
bone
pressure.
massive
than
coagulation
reported
symptoms.
The
several
months
1
the
of the
of a pseudotumor.
this lesion
was
of the
( table
that
nature
this
hemarthroses.7
femoral
be
no
have
produced
this situation
would
a confined
sub-periosteal
greater
suspect,
than
femur
old
CHRISTENSEN
suggested
been
indicated,
our patient’s
bleeding
tendency
This is the first reported
instance
of pseudotumor
would
rather
of
also
have
considerable
Larsen
sterile
into
might
shaft.
cases.7”2
infusing
All requirements
minimal
oozing
cavity.
the
on
a pressure
of 180 cm. of water
that
a similar
mechanism
may
bone.”
tinual
One
hemorrhage
pressure
may
our
bone
intramedullary
internal
AND
than
surgical
a hemophiliac
patient
of hemorrhage
and
the
or
correction
or
infection
control
of
defect.
Active
bleeding
must be stopped.
Repeated
a long period
of time may be required.
Fresh
plasma
given
depending
therapy
resorption
has
on
been
of the
whether
advocated
tumor.1#{176}
the
patient
in
the
is AHG
past
on
or
the
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PSEUDOTUMOR
OF
589
HEMOPHILIA
1.-Pseudotumor
TABLE
Number
of
Age
of
patients
of Bone
reported
18
patients
9-55
Sex
Affected
Bone
Femur
10 cases
Tibia
3 cases
Ilium
3 cases
Thumb
Fatal
2 cases
outcome
Surgical
to pseudotumor)
(related
intervention
(biopsy,
or
Duration
The
mortality
18
because
the
for
of
sarcoma)
10 cases
4 months
patients
reported
in
In four
poor
of
pseudotumor
rate
patients
complications.
to
of
7 cases
exploration
amputation
possibility
the
years
Male
of the
outcome.
with
the
cases,
complication
died
surgical
Progression
over
a period
of years
mediate
outcome
is not
this
literature
of
and
result
fatal.
is
the
may
untreated
serious
years
high.
Seven
pseudotumor
intervention
the
in
of
-24
have
its
contributed
pseudotumor
incapacitation
of
or
may
even
occur
if the
im-
SUMMARY
1. A case
of pseudotumor
2. The
natural
hemophilia
of the
history
are
ilium
and
1. Es
reportate
de
2. Es
serie
of
deficient
this
patient
serious
is reported.
complication
of
reviewed.
SuuIAmuo
cientia
in a PTC
treatment
un
caso
de
INTEIILINGUA
IN
pseudotumor
del
ilium
in
un
patiente
con
defi-
PTC.
presentate
un
complication
revista
de
del
historia
natural
e del
tractamento
de
iste
hemophilia.
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1959 14: 584-590
Pseudotumor of Hemophilia in a Patient with PTC Deficiency
R. SILBER and W. R. CHRISTENSEN
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