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Thoracic, abdominal and musculoskeletal involvement in Erdheim-Chester disease: CT, MR and PET imaging findings.
[erdheim-chester disease]
Erdheim-
Chester
disease
(
ECD
)
is
a
rare
,
non-
Langerhans
cell
histiocytosis
with
characteristic
radiological
and
histological
features
.
This
entity
is
defined
by
a
mononuclear
infiltrate
consisting
of
lipid-laden
,
foamy
histiocytes
that
stain
positively
for
CD
68
and
negatively
for
CD
1
a
.
Osseous
involvement
is
constant
and
characteristic
.
Extra
-osseous
lesions
may
affect
the
retroperitoneum
,
lungs
,
skin
,
heart
,
brain
and
orbits
.
Both
radiography
and
technetium-
99
m
bone
scintigraphy
may
reveal
osteosclerosis
of
the
long
bones
,
which
is
a
typical
finding
in
ECD
.
For
visceral
involvement
,
computed
tomography
(
CT
)
is
most
useful
,
while
magnetic
resonance
(
MR
)
imaging
is
more
sensitive
for
cardiovascular
lesions
;
2
-
[
fluorine-
18
]
fluoro-
2
-
deoxy-d-glucose
(
FDG
)
positron
emission
tomography
(
PET
)
/
CT
scanning
is
useful
in
assessing
the
extension
of
ECD
lesions
.
The
prognosis
is
extremely
variable
and
is
often
worse
when
there
is
cardiovascular
system
involvement
.
Diagnosis
is
based
on
the
combination
of
radiographic
,
CT
,
MR
imaging
and
nuclear
medicine
features
and
a
nearly
pathognomonic
immunohistochemical
profile
.
The
aims
of
this
work
are
to
perform
a
systematic
review
of
Erdheim-
Chester
disease
as
seen
on
imaging
of
the
chest
,
abdomen
and
musculoskeletal
system
and
to
discuss
the
diagnostic
workup
and
differential
diagnoses
according
to
the
imaging
presentation
.
Teaching
points
•
Bone
involvement
is
usually
present
in
patients
,
and
the
imaging
findings
are
pathognomonic
of
ECD
.
•
The
circumferential
periaortic
infiltration
may
extend
to
its
branches
,
sometimes
becoming
symptomatic
.
•
Cardiac
involvement-the
pericardium
,
right
atrium
and
auriculoventricular
sulcus-worsens
its
prognosis
.
•
Perirenal
infiltration
extending
to
the
proximal
ureter
is
highly
suggestive
of
this
disease
.
Diseases
Validation
Diseases presenting
"which is a typical finding in ecd"
symptom
erdheim-chester disease
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