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ParostealOsteosarcoma
Overview
Parosteal osteosarcoma arises from periosseous tissues
adjacent to the cortex. It is usually considered as an
osteosarcoma variant, which must be differentiated from
the intracortical and periosteal variants of osteosarcoma.
Parosteal osteosarcoma is slow-growing and late to
metastasize; accordingly, it carries a better prognosis than
other variants of osteosarcoma. It is generally a low-grade
highly differentiated tumor but has the potential to turn into
a high-grade poorly differentiated tumor, even though the
incidence of such conversion is very low. Often, the tumor
is palpable because of the large amount of homogeneous
lobulated new bone outside the bone shell extending into
the soft tissue. It is juxtacortical in position and densely
ossified.
Parosteal osteosarcomas make up 4% of all
osteosarcomas. There is no sex predilection, though
some studies suggest female preponderance. More than
half of the tumors arise from the posterior aspect of the
distal femur in the metaphysis. The other common sites
are the proximal tibia and the humerus. Rare examples of
involvement of acral skeleton and craniofacial bones are
also described in the literature.
Chemotherapy and radiotherapy generally are not very
effective in treating parosteal osteosarcomas. Wide
resection with limb salvage is the surgical treatment of
choice. Gene therapy is the future for treating such
cancers in the orthopedic setting; however, it is still in its
infancy, and considerable research and development will
be required before it can be widely implemented.

PathophysiologyandEtiology
Parosteal osteosarcoma occurs in the periosseous
tissues. It has not been described to arise from sites other
than the major tubular bones. The most commonly
affected sites, in descending order, are the distal femur

(typically the posterior surface), the proximal humerus, the

proximal femur, the proximal tibia, and the proximal ulna.
By contrast, the proximal tibia is the most common site for
periosteal osteosarcoma.
Parosteal osteosarcoma has a slow rate of development;
however, it eventually destroys the cortex and involves the
medulla at later stages. It is a well-differentiated and latemetastasizing tumor and thus has a better prognosis.
The exact etiology of osteosarcoma is still unknown.
However, numerous chemical agents, including
methylcholanthrene, beryllium oxide, and zinc beryllium
silicate, have been implicated in animal models. Other
causes include radiation, viruses and heredity.

ClinicalPresentation
Parosteal osteosarcoma usually occurs in older age with a
peak in the third and fourth decades of life, with no sex
predilection. It commonly presents as a slow-growing
painless lump in the posterior aspect of the distal femur;
however, it can be painful as well. The pain may be dull
and aching in nature. Rarely, parosteal osteosarcoma can
present with restriction of joint movement when it occurs
close to the joint. The duration of symptoms may range
from 1 month to 2 years before the diagnosis.

PlainRadiography

Orthogonal views of the affected long bone with the joints

above and below should be obtained, preferably in
conjunction with full-length radiographs of the bones.
Because parosteal osteosarcoma is a bone-forming
tumor, the lesions will be radiopaque on radiography. The
degree of opacity depends on the maturity and quantity of
the osseous component (see the image below).
Inline figure

Plain radiograph showing the increased opacity in the lesion

(parosteal osteosarcoma) in the posterior aspect of distal femur
which is the classical site.

/Inline figure
Commonly, peripheral areas appear cloudy and indistinct,
indicating that they are less mature. However, there is no
correlation between radiopacity and histologic grade: even
a lesion that is heavily radiopaque on radiographs and
has sharply demarcated margins can be a high-grade
poorly differentiated tumor. A radiotranslucent line may be
seen between the cortex and the tumor, indicating the
presence of fibrous and cartilaginous tissues. The tumor
can present late; thus, cortical erosion and medullary
involvement may be seen at presentation.

ComputedTomography

Computed tomography (CT) may be employed for local

staging (see the images below), but magnetic resonance
imaging (MRI) is preferred.
Inline figure

CT Sagittal Reconstruction of the same lesion as in plain

radiographic image shown above.

/Inline figure Inline figure

CT axial section of the same lesion as in image above.

/Inline figure

MagneticResonanceImaging
MRI is very useful for evaluating intramedullary
involvement, especially in late-presenting cases. It is also
useful for determining joint involvement, assessing tumor
encroachment on major neurovascular tissues, and
examining the surrounding reactive zone for the purposes
of preoperative planning (see the images below). To
exclude rare skip lesions in dedifferentiated parosteal
osteosarcoma, MRI should cover the whole bone.
Inline figure

T1 weighted MRI scan of the same lesion as in plain radiographic

image shown above.

/Inline figure Inline figure

T2 weighted MRI axial section of the same lesion shown in image

above.

/Inline figure

OtherTests
Routine blood tests are performed to assess the patients
general medical condition and assessment of fitness for
anesthesia.
Angiography was more commonly used before the advent
of CT and MRI scanning, when it was valuable for defining
the relation of the tumor to the major vascular structures,
particularly in the popliteal and axillary regions.
Bone scanning shows an area of dense uptake within the
lesion. It is useful for identifying metastasis.

Biopsy

Core biopsy may be done with Islam needles to confirm

the diagnosis. If a sufficient sample cannot be obtained,
open biopsy may be performed. The following basic
principles of bone biopsy should be adhered to:
The biopsy must be done by the surgeon who will be
providing the definitive surgical treatmentor, at least,
discussed with him or her
The incision should be placed in such a way that it lies in
the same line as the planned incision for the definitive
procedure, so that it can be completely excised
The incision should not cross neurovascular structures
The specimen should be taken from the tumor margin
because the center of the tumor may have only necrotic
tissue; the periphery of the lesion is usually active, with a
reactive zone
If a drain is maintained, it should be placed very near the
wound; the drain tract must be excised in toto during
definitive surgery

HistologicFindings
The surgical specimen shows a hard matrix-producing
tumor arising from the underlying cortex of the bone.
Microscopically, parosteal osteosarcoma consists of wellformed bone trabeculae in a hypocellular spindle-cell
stroma.
The bone trabeculae are arranged in a parallel fashion.

Spindle cells usually show minimal atypia, though in

approximately 20% of cases the stroma is more cellular,
with moderate cytologic atypia. Mitotic figures are rare.
Approximately 50% of cases show cartilaginous
differentiation in the form of either cartilaginous nodules or
cartilaginous cap. Marrow involvement and cytologic
atypia do not predict a worse outcome.
Dedifferentiation is seen in approximately 15% of
parosteal osteosarcomas, either at the time of the original
tumor or, more often, at the time of recurrence.
Dedifferentiated parosteal osteosarcomas carry a worse
prognosis, comparable to that noted with the conventional
osteosarcomas.

Treatment&Management
Chemotherapy and radiotherapy are not very effective in
parosteal osteosarcomas. If the tumor specimen is of a
high histologic grade, then postoperative chemotherapy is
usually advised.
Because parosteal osteosarcoma can dedifferentiate and
metastasize and can cause local infiltration and
destruction, its presence is an indication for treatment.
Wide resection with limb salvage is the surgical treatment
of choice for parosteal osteosarcoma, in view of the
typically low grade of the tumor and the low rate of local
recurrence. Amputation is rarely indicated. The only
relative contraindication for surgical treatment in parosteal
osteosarcoma is the presence of multiple metastases in a
patient whose general condition is poor.
Resection of tumor
In planning the wide excision, it is necessary to obtain a
clear picture of the extent of the tumor. Various imaging
techniques are employed. In cases involving limb salvage
and custom-made prosthesis replacement, the
measurements are made by means of full-length
radiographs, and a hinged prosthesis is fabricated
according to the individual patients requirements.
Routine blood tests and various investigations are

performed to rule out secondary metastasis. The patients

general condition is assessed with respect to his or her
fitness for anesthesia. The patient must be completely
informed regarding the procedure; if needed, a tumor
counselor should be involved.
Because the tumor is extracortical and is usually present
in the posterior aspect of the distal femur, the important
neurovascular structures within the fibrofatty tissue must
be carefully dissected and preserved. Wide excision
should be performed in accordance with preoperative
planning on the basis of the measurements in the images.
In rare cases, when there is doubt regarding the extent of
the tumor at the time of surgery, tetracycline labeling may
be used to determine the adequacy of the surgical
margins.
Routine mobilization and physiotherapy are provided as
appropriate for a custom-made prosthetic replacement.
The main postoperative workup involves histopathologic
grading of the lesion and assessment of the adequacy of
the surgical margins. If the tumor turns out to be a highgrade lesion, postoperative chemotherapy must be
considered. Inadequate surgical margins are associated
with a high incidence of local recurrence; consequently,
postoperative chemotherapy or radiotherapy may be
considered in such instances.
Complications of surgery
Potential complications of tumor resection include the
following:
Impaired wound healing - Superficial wound infection,
wound dehiscence, or failure of wound healing may occur
Infection - Deep infection may be difficult to eradicate,
especially in patients with a prosthesis, and repeated
wound washing and appropriate antibiotics may be
required; persistent infection may lead to implant removal
and, in severe cases, amputation
Hematoma - Postoperative bleeding can lead to
hematoma formation in the space resulting from wide

excision
Muscle atrophy
Neurovascular injury - Because the popliteal
neurovascular tissues are closer to the tumor, they may
be injured during surgery; intraoperative application of
traction can result in neurapraxia
Stiffness - Because the tumor is close to the joint,
postoperative stiffness should be anticipated; regular
physiotherapy is needed to overcome this complication
Implant failure - Implant loosening, polyethylene wear,
fracture of the implant, and periprosthetic fracture can
result in implant failure
Local recurrence (common in tumors with inadequate
surgical margins)
Metastasis In rare cases, late secondary metastasis
may develop after surgery, especially with dedifferentiated
tumors

Outcome/Prognosis
Because parosteal osteosarcoma is typically a low-grade
tumor, it carries the best prognosis of all the
osteosarcomas. For low-grade lesions, complete
resection ensures cure. If high-grade areas are present in
the lesion, the prognosis then approaches that of
conventional high-grade osteosarcomas, and the patient
requires chemotherapy in addition to surgical wide
excision.
Routine follow-up is needed to rule out local recurrence or
the appearance of metastasis, even though these are rare
in parosteal osteosarcoma.