THE VARIABLE IMAGING APPEARANCE OF OSTEOSARCOMA

S. Van de Perre1,2, F.M. Vanhoenacker1,2, A. Snoeckx1,2, P. Van Dyck2, J. Gielen2, P.M. Parizel2
The purpose of this brief review is to give an overview of the different imaging features of the various types of
osteosarcoma, based on their macroscopic location within the musculoskeletal system. Further subdivision can be
made by histological criteria and/or more specific location. Standard radiographic features allowing their differentiation will be highlighted. The value of cross-sectional imaging in the pre-operative staging, assessment of local
extension, monitoring of response to treatment and guiding biopsy will be emphasized as well.
Key-word: Osteosarcoma.

Osteosarcoma is the second most

common primary malignant bone
tumor after multiple myeloma. The
age of presentation ranges between
10-30. Males are affected twice as
often as females.
Osteosarcomas can be divided
into different subtypes, each with
characteristic imaging features (1).
Grossly, there are 3 major groups,
namely intramedullary, surface (or
juxtacortical) and extraskeletal

osteosarcoma. Each group can be

further divided in different subtypes
(Table I).
The imaging features of each
subtype of osteosarcoma differ,
depending on their exact localization in the bone or specific histology (1, 2).
In the next section the imaging
features of some of the subtypes
of osteosarcoma will be discussed.

From: 1. Department of Radiology AZ St.-Maarten Duffel/Mechelen, Duffel, 2. Department of Radiology Universitair Ziekenhuis Antwerpen, Edegem, Belgium.
Address for correspondence: Dr F.M. Vanhoenacker, M.D., Dept. of Radiology, University Hospital Antwerp, Wilrijkstraat 10, B-2650 Edegem.

Discussion

Intramedullary osteosarcomas
High-grade osteosarcoma
75% of all osteosarcomas belong
to this subtype, also known as classic or conventional osteosarcoma (2).
They mostly affect the metaphysis of the long bones, with predilection of the distal femur and proximal
tibia.
Clinically, they present as a
painful swelling and are often incidentally diagnosed after minor trauma (1, 2).
On standard radiography, they
usually present as a mixed sclerotic

PROCEEDINGS OF THE SRBR-KBVR OSTEOARTICULAR SECTION JUNE MEETING

Table I. Categorization of osteomas.

Location
Intramedullary

Subtype
High-grade
Telangiectatic
Low-grade
Small cell

Osteosarcomatosis
Surface

Gnathic
Intracortical
Periosteal
Parosteal
High-grade

Extraskeletal

and osteolytic geographic lesion,

although their presentation can
range from pure lytic to pure sclerotic.
An aggressive type of periosteal
reaction (Codmans triangle, laminated or sunburst/hair-on-end pat-

tern), reflects the malignant behaviour of the tumor (Fig. 1 and Fig. 2).
In most cases, standard radiography is sufficient to make the diagnosis (1, 2).
Cross-sectional imaging (CT and
MRI) is used for pre-operative
assessment and staging.
Where CT is the method of choice
for detection of distant metastasis
(lung, lymphnodes and bone), MRI
is the superior to determine the
exact local extension of the lesion.
Invasion of the epiphysis or adjacent joint has to be evaluated, as
well as the relationship of the tumor
to the neurovascular bundle (Fig. 3).
MRI is also useful to depict skip
lesions in the same bone.
The tumor is of high signal intensity (SI) on T2-WI and intermediate
SI on T1-WI, with enhancement after
contrast administration in the viable
tumor areas. The areas of mineralization show low SI on both pulse
sequences. Regions of high SI on

205

both pulse sequences represent

hemorraghe, whereas a low SI on
T1-WI and a high SI on T2-WI, with
absence of contrast enhancement
represents necrosis (2).
Dynamic contrast-enhanced MR
imaging is used to determine tumor
vascularization and can guide targeting a biopsy site, for the most
viable part of the tumor. It is also
used in monitoring the response to
pre-operative chemotherapy. In this
context, tumor vascularisation is a
better parameter to evaluate tumor
response than tumor volume (3, 4).
Angiography is not used anymore solely for diagnostic purposes.
The only residual indication for
angiography is the local administration of pre-operative chemotherapy
via intra-arterial way, better known
as isolated limb perfusion (ILP) (5).
Bone scintigraphy is the mainstay to detect distant bone metastases (2).

Fig. 1. Schematic drawing of the different locations and extension of osteosarcoma in relation to the cortex.
1: intramedullary osteosarcoma
2: intracortical
3: juxtacortical
4: extraskeletal
5: sequential stages of tumor growth in periosteal osteosarcoma. The tumor originates from the deep layer of the periosteum
(dashed line). Evolving tumor growth will violate the periosteum, causing a Codman triangle.
6: sequential stages of tumor growth in parosteal osteosarcoma. The tumor originates from the superficial layer (solid line) of the
periosteum. The tumor can extent directly into the surrounding soft-tissue, without violating the periosteum. This explains why an
aggressive type of periosteal reaction is absent.

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JBRBTR, 2005, 88 (4)

The secondary lesions are smaller, more sclerotic and lack periosteal
reaction and cortical disruption (2, 8,
9).
Patients with osteosarcomatosis
have a very poor prognosis (1, 2, 8,
9).
Gnathic osteosarcoma
This lesion is subdivided only
because of his typical location in the
maxillary-mandibular region (1, 2).

Surface osteosarcomas
Intracortical osteosarcoma

Fig. 2. Standard radiograph, AP view (A) and lateral view

(B) of the distal femur showing high grade osteosarcoma of the
left knee in a 15-year-old boy. Osteosclerotic lesion is seen at
the metaphysis of the distal femur with a sunburst periosteal
reaction, reflecting the aggressive behavior of the lesion. On
this image, the epiphysis seems uninvolved.

Telangiectatic osteosarcoma
A telangiectatic osteosarcoma is
a primary malignant bone tumor
characterized by large cavities filled
with blood. The peripheral wall, as
well as the internal septations of
these cavities contain sarcomatous
cells, producing osteoid matrix
which may calcify (2, 6).
This lesion clinically presents as a
rapidly-growing painful mass.
Telangiectatic osteosarcoma can
also be a secondary lesion, arising
in association with fibrous dysplasia, Paget disease or following radiation therapy (2).
On standard radiographs, telangiectatic osteosarcoma has a purely
lytic appearance, with possible
aggressive type periosteal reaction
and cortical destruction. Pathologic
fractures are common (1, 2, 7).
Bone scintigraphy demonstrates
peripheral increased radionuclide
uptake with central photopenia, also
known as donut sign (2, 6).
Cross-sectional imaging (CT and
MRI) is used for pre-operative staging and assessment of tumor extension, as well as for differentiation
with aneurysmal bone cyst (2, 6).
CT shows a lesion with a hypodense center (compared with muscle) with mineralization of the
matrix in the intraosseous and/or
soft-tissue components of the
lesion.

Aggressive growth and mineralization of the peripheral matrix, best

appreciated on non-enhanced CT,
are also indicative for telangiectatic
osteosarcoma (2, 6).
Fluid-fluid levels may be seen
both on CT and MRI (1, 2, 6, 7).
On MRI, areas of hemorrhage
(methemoglobin) have a high SI on
both pulse sequences.
After administration of contrast, a
thickened wall with mural nodules
and internal septations may be seen
on CT as on MRI. This characteristic
helps to distinguish telangiectatic
osteosarcoma from aneurysmal
bone cyst, as the latter will only
show faint peripheral enhancement.
Moreover, biopsy has to be preferentially guided toward the enhancing nodules, in order to obtain a representative sample of viable tumor
tissue (2, 6).
The prognosis of telangiectatic
osteosarcoma is similar to that of a
classic osteosarcoma (2, 6).
Osteosarcomatosis
The term osteosarcomatosis is
used whenever there are multiple
intraosseous foci at the time of diagnosis (2, 8, 9). Probably, in osteosarcomatosis, there is one dominant
lesion, with the imaging features of
classic osteosarcoma, with the other
lesions being rapidly progressive
metastases.

Intracortical osteosarcoma (ICOS)

is the least common subtype of
osteosarcoma (2, 10, 11).
They arise in the diaphyseal cortex of the long bones (femur and
tibia).
Clinically, the patients present
with moderate pain and local tenderness (10, 11).
Intracortical osteosarcoma presents on standard radiography as a
geographic lytic lesion, with possible intralesional mineralization.
The lesions are mostly smaller
than 4 cm and are surrounded by
thickened sclerotic cortical bone (2,
10, 11).
CT is used to demonstrate cortical destruction, rare matrix calcifications and the extent of the surrounding cortical sclerosis (11).
The tumor has an intermediate SI
on T1-WI and high SI on T2-WI. After
contrast administration, peripheral
contrast enhancement may be seen
(10, 11).
However, fluid-sensitive MR
sequences, such as fat-saturated T2WI may detect perilesional and
medullary edema, making it a better
imaging modality than CT for local
staging. It was previously believed
that ICOS was confined solely within
the cortical bone, neither the
medullary space nor the soft tissues
were involved (10, 11). In the pre-MRI
era, the predilection for the relative
thick cortex of the middiaphysis of
the long bones rather than the thin
cortex of the metaphysis, was
thought to explain the rare incidence
of medullary infiltration (10).
The
differential
diagnosis
includes adamantinoma, osteofibrous dysplasia, osteoid osteoma
and intracortical abscess (10, 11).
Therefore, biopsy of the lesion is
mandatory to confirm the diagnosis
histologically (11).
Periosteal osteosarcoma
Periosteal osteosarcomas are
intermediate
grade
lesions,

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207

A
B

C
Fig. 3. MR imaging of the same patient, demonstrating the
superiority of MRI in local staging of the tumor. Axial SE T1-WI
(A). The lesion is of intermediate SI relative to muscle with areas
of low SI representing mineralization. The extra-osseous component is well appreciated on this image. Axial (B) and coronal (C)
fat-saturated SE T1-WI after gadolinium contrast administration:
There is heterogeneous contrast enhancement of the lesion.
There is disruption of the cortex at the medial side with softtissue extension. The neurovascular bundle seems uninvolved.
Note also the invasion of the epiphysis. Coronal fat-saturated TSE T2-WI (D): the tumor is of intermediate SI on T2-WI. Areas of low
SI represent mineralization. Epiphyseal extension is seen as a high SI area within the medial aspect of the distal femoral epiphysis.

originating from the deep layer of

the periost. Further progression of
the tumor will violate the periost
and is responsible for the marginal
Codman triangle (Fig. 1).
They arise from the diaphyseal
region of long bones and present as
a radiolucent lesion, corresponding
with the nonmineralized tumor cartilage. Indeed, periosteal osteosarco-

ma is less radiodense compared

with a parosteal osteosarcoma,
because the bulk of the tumor consists of chondroid matrix where in a
parosteal osteosarcoma, the majority of the tumor contains mineralized
osteoid (14). Brushlike spicules of
bone extending from the underlying
thickened cortex, are typically seen
(1, 2, 12-14).

The medullary cavity is only

exceptionally invaded (2, 12).
CT is superior to MRI for detecting cortical involvement and
periosteal reaction (12).
The appearance on MR imaging
is similar to that of cartilage tumors,
with septal and ring and arc
enhancement after intravenous
administration of gadolinium (13).

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The prognosis is worse than parosteal osteosarcoma, but better than

classic osteosarcoma (1, 2, 12-14).
Parosteal osteosarcoma
Parosteal osteosarcomas surge
from the superficial layer of the
periost, making a direct extension to
the surrounding soft-tissue possible.
This explains why periosteal bone
formation is not a classic feature of
a parosteal osteosarcoma, in contradinstinction with a periosteal
osteosarcoma (Fig. 1).
On
standard
radiography,
parosteal osteosarcomas are radiodense, lobulated or oval masses
with a broad stalk to the external
cortex of the underlying bone. A
thin, radiolucent cleavage plane
between the tumor and the underlying bone is a characteristic but not a
constant
finding.
Progressive
growth of the tumor may obliterate
this plane and the bone can be
entirely encased (1, 2).
Plain radiography is diagnostic in
most cases.
CT and MRI allow assessment of
medullary invasion and can depict
regions of dedifferentiation (1, 2, 15).
Low-grade lesions seen as as solid
osteoid lesions of low SI on T1- and
T2-WI can be treated with wide local
excision, but a high rate of local
recurrence has been described (1, 2).
Initial biopsy is not necessary (15).
Low grade parosteal osteosarcoma
has an overall good prognosis (1, 2).
Meticulous follow-up for early
detection of recurrence is necessary.
MRI is advised only when recurrence is suspected on clinical or
radiographical basis (16).
Occasionally, a portion of the
tumor dedifferentiates into a higher
histologic grade, acquiring a more
aggressive behaviour as well as
metastatic potential. In addition to
surgery, neoadjuvant chemotherapy
is advisable (1, 2, 15).
These high-grade lesions can also
be detected on angiography, presenting as a hypervascular blush,
where low-grade parosteal osteosarcoma is typically hypovascular (1).
This lesion has to be differentiated from myositis ossificans. In
myositis ossificans the ossification
occurs at the periphery of the lesion.
In the latter, there does not seem to
be a connection with the underlying
bone (1, 2).

Extraskeletal osteosarcoma
Extraskeletal osteosarcoma is a
slow growing painful mass in the

JBRBTR, 2005, 88 (4)

deep soft tissue of the thigh, upper

extremity and retroperitoneum (1, 2,
17, 18).
On imaging, the tumor present as
a large soft-tissue mass. Only half of
the lesions are calcified, rendering
correct diagnosis on standard radiograph very difficult (17).
The nonmineralized areas have
an attenuation similar to muscle on
CT (2).
On MRI, the osteoid matrix (irrespective of its calcification or ossification) has a low SI on both pulse
sequences, whereas the other parts
of the tumor demonstrate a high SI
on T2-WI and a low SI on T1-WI, with
enhancement after contrast administration (18).
An extraskeletal osteosarcoma
has an overall poor prognosis (2, 17).
Conclusion
High
grade
intramedullary
osteosarcoma has relative specific
radiographic characteristics. The
other subtypes of osteosarcoma are
more challenging to diagnose,
because they may mimic other
even benign pathology.
The knowledge and recognition
of some specific semiological signs
are the clue to the correct diagnosis.
Multiplanar imaging (CT and
especially MRI) have an important
role in staging, assessment of local
tumor extension and follow-up of
tumor response to therapy. CT and
MRI may also be helpful to guide
biopsy and to demonstrate the most
viable area within the tumor.
Angiography only plays a role in
the administration of intra-arterial
pre-operative chemotherapy. In
parosteal osteosarcoma it can also
depict areas of dedifferentiation.
Bone scintigraphy is used for
detection of distant bone metastases.
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