Ewing sarcoma

Ewing sarcoma is the second most common primary bone tumour of childhood after
osteosarcoma, and may present with very variable radiographic features.
Epidemiology
Typically occurs in children and adolescents between 10 and 20 years of age (95 between !
and 25 years of age", and has a slight male predilection (#$% 1.5$1"
1&2
.
Clinical presentation
'resentation is non&specific with local pain being by far the most common symptom.
(ccasionally a soft tissue mass may be palpable. 'athological fractures also occur.
Pathology
)wing sarcoma is a small round blue cell tumour with regular si*ed primitive appearing cells.
+t is closely related to the soft tissue tumours p',)T, -s.in tumourand neuroepithelioma,
which collectively are referred to as )wing sarcoma family of tumours ()/%T"
1
. They share
not only microscopic appearances but also demonstrate a non&random t(11022"(12!0112"
chromosome rearrangement.
Location
lower limb $ !5
o femur most common
pelvis $ 20
upper limb $ 12
spine and ribs $ 12
o sacrococcygeal region most common
!

s.ull 3 face $ 2
-lternatively
2
$
long bones $ 50 & 40
o femur $ 25
o tibia $ 11
o humerus $ 10
flat bones $ !0
o pelvis $ 1!
o scapula
o ribs $ 4
-s far as location within long bones, the tumor is almost always metaphyseal or diaphyseal
2&
2
$
mid&diaphysis $ 22
metadiaphysis $ !!
metaphysis $ 15
epiphysis $ 1 & 2
Radiographic features
)wing sarcomas tend to be large poorly marginated tumours, with over 50 demonstrating
e6tension into ad7acent soft tissues. +t should be noted that p',)T often e6tend into bone,
ma.ing the distinction difficult.
Plain film and CT
The appearance of these tumours is very variable, but usually have clearly aggressive
appearance. 8ommon findings include
2
$
permeative $ 94
laminated (onion s.in" periosteal reaction $ 59
sclerosis $ !0
They occasionally demonstrate other appearances, including 8odman triangles, spiculated
(sunburst" or thic. periosteal reaction and even bone e6pansion or cystic components.
/oft tissue calcification is uncommon, seen in less than 10 of cases
2
.
MRI
T1 $ low to intermediate signal
T1 C+ (d! $ heterogeneous but prominent enhancement
T" $ heterogeneously high signal, may see hair on end low signal striations
#uclear medicine
)wing sarcomas demonstrate increased upta.e on both :allium
49
&citrate and Technetium
99
m
methylene diphosphonate scans
4
.
Treatment and prognosis
/ystemic chemotherapy is the mainstay of treatment with surgery and 3 or radiotherapy
playing a role depending of the location and si*e of the tumour.
;hat was once a uniformly fatal tumour now has respectable survival rates, although these
vary with location. /pinal tumours for e6ample have up to 54 long term survival compared
to 25 of sacrococcygeal tumours
!
. The overall 5 year survival is in the order of 50 & 95 of
patients with local disease only at the time of presentation
5
.
'rognosis is significantly impacted by the presence of distant metastases at the time of
diagnosis, which are far more common for the pelvis (25 & 20" compared to e6tremities
(<10"
5
. #etastases most fre1uently go to bone or lungs.
Etymology
,amed after $ames %tephen Ewing $ -merican pathologist (1544 & 19!2"
5

&ifferential diagnosis
other )wing sarcoma family of tumours
o p',)T $ large soft tissue component with e6tension into bone
o -s.in tumour $ chest wall
osteosarcoma
osteomyelitis
metastatic disease
haematological malignancy
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