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Article in JBR-BTR: organe de la Société royale belge de radiologie (SRBR) = orgaan van de Koninklijke Belgische Vereniging voor Radiologie (KBVR) · September 2007
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Ewing’s sarcoma (ES), peripheral from four institutions, and to com- Tumor matrix was scored on a
primitive neuroectodermal tumors pare our findings with the literature. subjective scale as the sclerosis
(PNET), and Askin tumors (AT) are compared to of osteolysis within
referred to as Ewing’s tumors Materials and methods the osseous component of the
(ETs) (1). ES is the second most tumor.
common malignant bone tumor in Sixty-four patients with histologi- The margins of the tumor on radi-
children and young adults, with an cally and/or genetically proven diag- ographs/CT were evaluated and
unknown histogenesis (2). nosis of ES were included. In our graded by the following criteria:
ES is a round-cell sarcoma, show- series, we assembled 52 plain radi- sharp demarcation, partially sharp
ing varying degrees of neuroecto- ographs, 36 CT scans and 62 MRI demarcation (less than 50%
dermal differentiation. These tumors scans. The MR imaging protocol unsharp) and unsharp demarcation
are cytogenetically well described, consisted of at least T1-weighted, T2- (more than 50% unsharp).
and in 85% of the cases, a balanced weighted images (with or without Periosteal reaction was scored as
t(11;22)(q24;12) is found (3). fat suppression) and T1-weighted lamellar or onion peel, interrupted
Most patients complain of pain, images after intravenous adminis- lamellar, Codman’s triangle, sun-
occasionally accompanied by tration of gadolinium chelates in dif- burst or hair-on-end or mixed.
swelling at the affected site. ferent slice directions. Codman’s triangle is due to forma-
Infrequent presenting symptoms We analyzed all patient files for tion of reactive bone between the
include fever, weight loss, cough, clinical parameters, i.e. age, gender elevated intact periosteum and the
anemia and leukocytosis. and location. Secondly, we evaluat- underlying cortex at the zone of
ed the available radiographs/CT transition to the extraosseous
Aim scans for lesion distribution within extension of the tumor. Formation
the skeleton (axial skeleton, flat of long, thin filiform spicules, radiat-
The aim of this article is to define bones or epi-, meta- or diaphyseal ing perpendicularly from the cortex,
an imaging prototype of Ewing’s location within the long bones), is typical for a spicular, sunburst
sarcoma, based on the analysis of a matrix, margins, periosteal reaction periosteal reaction.
large cohort of patients, originating and articular extension. Articular extension on radio-
graphs/CT was defined as cortical
breakthrough of the joint margins
and/or tumor extension on both
sides of the adjacent joint. Cortical
From: 1. Dept. of Radiology University Hospital Antwerp, 2. Dept. of Radiology Leiden reaction was scored by following
University Medical Center, 3. Dept. of Radiology University Hospital Leuven, 4. Dept. criteria, namely cortical destruction,
of Radiology University Hospital Gent, 5. Dept. of Orthopaedic Surgery University cortical permeation and cortical
Hospital Leuven, 6. Vision Lab, University of Antwerp.
Address for correspondence: Dr B. Peersman, MD, Department of Radiology,
thickening or thinning. Also the
University Hospital Antwerp, Wilrijkstraat 10, B-2650 Edegem, Belgium. presence of a pathologic fracture at
E-mail: benjaminpeersman@hotmail.com. initial presentation was evaluated.
EWING’S SARCOMA — PEERSMAN et al 369
B
Fig. 2. — Lesion matrix.
A. Plain radiograph of the pelvis shows an example of a pure-
ly lytic ES of the left iliac bone.
B. Axial CT image of a mixed sclerotic-lytic type in the right
femoral neck. Note areas of sclerosis (white arrow) combined
A with areas of osteolysis (bone destruction) (black arrow).
370 JBR–BTR, 2007, 90 (5)
Discussion
Table I. — Percentage of sclerotic component in lesion matrix were described as uncommon find-
ings. Soft tissue calcification,
saucerization, honeycombing, sharp
margins and vertebra plana were
described as rare findings. Poor
margination, extensive soft tissue
component, sclerotic matrix and
permeation are also common find-
ings in our study. On the other hand,
laminated periosteal reaction is an
uncommon finding in our study
(14%). Although Reinus et al.
described spiculated periosteal
reaction as an uncommon finding ,
it is more than common (> 50%) in
our series. The milder forms of
periosteal reaction, namely laminat-
ed periosteal reaction and onion
skin were described also by Resnick
to be common manifestations
(57%), and the spiculated periosteal
reaction as a less frequent manifes-
tation (28%) (8).
Cortical permeation and destruc-
An exclusively sclerotic lesion is infrequent (6%). A mixed sclerotic-lytic type tion are common findings in our
with a sclerotic component of 75% occurs in 40% of cases. A 50% sclerotic- study (respectively 31 and 42%),
50% lytic type is seen in 20% of the cases. Only 11% present as a lesion with whereas cortical thickening is rather
a sclerotic component of 25%. 23% of ES’s in our study present as a purely uncommon (20%). A pathologic
lytic lesion (sclerotic component of 0%). fracture (7.8%), as well as soft tissue
calcification and sharp margins are
rare findings in our study.
Pathologic fracture was described
as an uncommon finding (14%) by
Reinus et al.
CT scan is a good imaging
Table II. — Type of periosteal reaction frequency modality to evaluate joint extension,
periosteal reaction and matrix of the
lesion. A large soft-tissue mass is
well illustrated by CT-scan, especial-
ly after intravenous administration
of contrast material.
MRI is accurate in the assessment
of the intramedullary tumor extent
in patients with bone sarcoma
which is documented by the excel-
lent correlation between longitudi-
nal T1-weighted images and identi-
cal macrosections of the surgical
specimens. Transverse TSE T2-
weighted images best display the
interface between tumor and adja-
cent soft tissues and the anatomical
relationship with the neurovascular
structures, allowing differentiation
between intracompartmental and
extracompartmental disease.
Contrast between tumor and normal
Frequency of occurrence of the different types of periosteal reaction. The tissue, especially fat-containing tis-
onion peel reaction occurs in 14%, the interrupted lamellar form in 18%, sue, is greatly enhanced by combin-
Codman’s triangle in 27% and the spicular periosteal reaction in 52% of ing TSE with fat-selective presatura-
cases. tion. T1-weighted images after
administration of contrast material
can be successfully combined with
fat-selective presaturation to
enhance contrast resolution (8-23).
sue involvement, permeative com- Spiculated periosteal reaction, corti- Non-homogeneous signal intensity
ponent, laminated periosteal reac- cal thickening and violation, purely is seen on all pulse sequences. No
tion and sclerotic matrix were lytic matrix, pathologic fracture, cys- specific degree and pattern of
described as common findings. tic component and bone expansion enhancement on MRI is seen. Skip
372 JBR–BTR, 2007, 90 (5)
A C
D E F
Fig. 4. — Variable periosteal reaction in different patients.
Plain radiograph of the femur. Lamellar periosteal reaction.
CT scan of the same patient. Lamellar periosteal reaction.
Plain radiograph of the distal ulnar diaphysis. Note the interrupted (arrow) lamellar periosteal reaction, depicting regions of more
aggressive tumor extension into the soft tissue.
D-E. Plain radiograph of the femur and sagittal reformatted CT scan of the forearm in 2 different patients. Codman’s triangle or
spur (arrows).
F-G. Radiographs and CT scan of the femur. Aggressive spicular, ‘sunburst’ periosteal reaction.
EWING’S SARCOMA — PEERSMAN et al 373
A B
C D
Fig. 5. — Evaluation of local tumor extension (bone and soft tissue component).Evaluation of joint extension by different imag-
ing modalities.
A. Plain radiograph of the pelvis. Sclerotic lesion of the left iliac bone. Evaluation of soft tissue component and articular exten-
sion is not possible.
B. Axial CT scan. Sclerosis is mainly located at the left iliac bone (black arrow) but minor sclerosis on the sacral side of the sacro-
iliac joint suggests articular extension (white arrow).
C. Axial T2-weighted image with fat saturation. We can now clearly demonstrate the articular extension of the tumor and invasion
of the sacrum (white arrow). Note also huge soft tissue component (black arrows).
D. Axial T1-weighted image after intravenous administration of gadolinium chelates. This sequence also demonstrates the articular
extension of the pelvic tumor and tumoral invasion of the sacrum (white arrow). Soft tissue extension is indicated by black arrows.
A B
Fig. 6. — Signal intensity on T1-weighted images.
A. Coronal T1-weighted image of the sacrum. ES of the sacrum (arrows), with a signal intensity equal to
that of the surrounding muscles.
B. Coronal T1-weighted image of the pelvis. Ewing’s sarcoma of the left iliac bone with a large soft-tissue
component (arrows). The overall signal intensity is higher than that of surrounding muscles, but lower than fat.
374 JBR–BTR, 2007, 90 (5)
administration of gadolinium
chelates. This is accentuated in diffi-
cult anatomic regions (e.g. pelvis).
We couldn’t compare the value of
MRI and CT scan because the num-
ber of CT-scans available was limit-
Fig. 8. — Signal intensity on fat suppressed T2-weighted ed, many scans contained only
images. images in axial plane and not all
Axial T2-weighted image with fat suppression. ES of the left scans were performed after intra-
iliac bone, with a large soft-tissue component. The overall sig- venous contrast administration.
nal intensity is higher than that of the surrounding muscles, but Other limitations in our study are
lower than water (arrows). due to the retrospective design.
There were indeed slight differences
in the MR protocol between the dif-
ferent institutions and not all
metastases are present in 14% of defining intraosseous tumor length images were available in a full digi-
cases. Jiya et al. described the and was as accurate as CT in tal format (DICOM) rendering more
occurrence of skip lesion in ES to be demonstrating cortical bone and accurate volume measurements
rare (24). Most tumors in our series joint involvement (27). But MRI was impossible.
have a large soft tissue component, definitely superior to CT in demon-
65% presented with a soft tissue strating involvement of muscle Conclusion
component of more than 50%. compartments. They concluded that
MRI is very sensitive, but less MRI is the modality of choice for Ewing’s sarcoma is a highly
specific for the determination of epi- local staging of primary bone sarco- malignant bone tumor that predom-
physeal involvement. It is highly ma. Panicek et al. concluded in 1997 inantly occurs in young patients. The
sensitive for excluding joint involve- that CT and MR imaging are equally most characteristic finding is the
ment although false-positive results accurate in the local staging of presence of a large soft tissue mass,
may occur secondary to synovial malignant bone and soft-tissue neo- which is best demonstrated on MRI.
inflammatory reactions (25, 26). plasms in specific anatomic sites Seventy percent of these tumors
With the recent developments of studied (28). In our study, MRI is the arise in the pelvis or lower extremi-
multiplanar imaging, CT scan is a imaging modality of choice because ty. In contradistinction to the litera-
good alternative in local staging of of its high contrast resolution and ture, a spiculated periosteal reaction
the tumor. Bloem et al. and also the ability to define the margins of is seen in more than half of our
many other authors in the 1980’s the soft-tissue component, especial- patients. Ewing’s sarcoma presents
described that MRI was significantly ly on T2-weighted images and T1- most frequently as a mixed sclerot-
superior to CT and scintigraphy in weighted imaging after intravenous ic-lytic type of tumor, and most
EWING’S SARCOMA — PEERSMAN et al 375
B
A
D
Fig. 10. — Local tumor extension.
A-B. CT scan of the lower leg. Aggressive tumoral lesion of the fibula with cortical
permeation and destruction. Spicular periosteal reaction. Presence of a large soft tis-
sue component (arrows).
C-D. Sagittal T1-weighted image after intravenous administration of gadolinium
chelates (C) and an axial T2-weighted image (D). ES of the fibula with the presence of
a large soft tissue mass (arrows). The soft tissue extension of the tumor is better appre-
C ciated on MR images in comparison to the CT-images.
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