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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
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
/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
/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
/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
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.
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
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.