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122
J. Day and B. Adams
droid matrix. Histologic features vary considerably
by location and as previously mentioned, it is difcult to distinguish between enchondroma and
low-grade chondrosarcoma by histology alone.
Enchondromas rarely require treatment. When
treatment is necessary, a simple curettage is often
curative. If atypical cartilaginous tumor is a possibility, a local adjuvant such as cryosurgery should
be added to reduce the chance of local recurrence.
Osteochondroma (Exostosis)
Osteochondromas are the most common benign
bone tumor. They typically grow along with the
individual until skeletal maturity is reached. They
are characteristically sessile or pedunculated, arising from the cortex of long tubular bones adjacent
to the physis. Osteochondromas are usually solitary but can be multiple in multiple hereditary
exostosis (MHE). MHE is caused by an autosomal
dominant germline mutation in tumor suppressor
genes EXT1, EXT2, or EXT3. Mutation in the
EXT1 gene has a more severe phenotype with
more osteochondromas, greater deformity, and a
higher chance of malignant transformation
(Fig. 6.15). Solitary osteochondromas frequently
have somatic mutations in the EXT1 gene. Solitary
osteochondromas have a 1% chance of malignant
transformation, while MHE patients have a 6–10%
chance of malignant transformation with the risk
varying by the underlying genetic mutation as
noted above. Osteochondromas of the pelvis may
be at higher risk for malignant transformation than
other lesions.
Plain radiographs are usually diagnostic, with
no further tests required. Radiographs show an
exostosis with continuity of the cortex and medullary bone between normal bone and the lesion.
Sessile osteochondromas, of note, may prove to
be a diagnostic challenge as they must be differentiated from a parosteal osteosarcoma. CT can
be helpful in assessing the cortex in either sessile
lesions or larger lesions that may wrap around the
bone. MRI is useful to assess for a soft tissue
component when there is a question of malignant
transformation. MRI can also be used to assess
the thickness of the cartilage cap. The thickness
of the cartilage cap may be an unreliable indicator for malignancy, but a cap of over 2cm thick in
an adult is worrisome for malignancy.
In general, surgical removal is recommended
only for symptomatic osteochondromas. For a
patient with a newly symptomatic osteochondroma, the clinician should rst distinguish
between mechanical type symptoms and pain
arising from the tumor itself. Tumor type pain or
growth after skeletal maturity should prompt an
evaluation for a secondary chondrosarcoma. If
symptomatic osteochondromas do not respond to
conservative treatment, surgical treatment
involves a marginal excision involving the
entirety of the cartilage cap. Disruption of the
cartilage cap can result in an unnecessarily high
risk of local recurrence.
Fig. 6.15 Multiple hereditary exostosis. There is aring
of the proximal femoral metaphysis in addition to multiple osteochondromas (arrows)
Osteoid Osteoma
Osteoid osteomas are benign lesions, with a characteristic pain pattern. The most common anatomic sites are the femur and tibia; however, they
can occur in virtually any bone. Pain can precede
the appearance of radiographic abnormalities and
lesions can be quite small, so there is often a lag
time in proper diagnosis.
The hallmark clinical nding of an osteoid osteoma is severe, well-localized aching type pain. Pain
is relieved with nonsteroidal anti- inammatories
ab
6 Tumors oftheMusculoskeletal System
123
(NSAIDs). The extent of pain relief is often dramatic with pain relief within 30min, and a history
of pain relief with NSAIDs is essential for diagnosis. Patients commonly come to the ofce having
taken NSAIDs on a regular basis for a year or more.
Periarticular lesions are associated with pain and
swelling that often mimic monoarticular arthritis.
Lesions involving the spine can present as painful
scoliosis.
Osteoid osteomas can be found in any portion of
the bone. Therefore, the position of the lesion relative to the cortex, periosteum, and spongiosa determine the radiographic appearance. The most
common site is intracortical, with plain radiographs
demonstrating a characteristic radiolucent nidus
surrounded by dense, reactive bone. When the
tumor is intramedullary, the sclerotic response is
less dramatic. Osteoid osteomas are often only a
few millimeters in diameter making them difcult
to see on plain radiographs. CT is often the best way
to localize these lesions. The most obvious feature
on MRI is often the surrounding marrow edema.
Traditionally, surgical treatment required simple curettage of the lytic nidus. Given the small
size of the nidus and the surrounding sclerotic
bone, this can prove difcult. Using a burr down
technique to slowly remove sclerotic bone until
visualizing the bright red nidus, which can then
be curetted, is a helpful method to do this. The
majority of lesions are currently treated using
CT-guided radiofrequency ablation. MRI guided
high intensity focused ultrasound (HIFU) has
also been used. Both ablation options are effective, but their use is limited in areas such as the
spine or tibia if the lesion is too close to a nerve
root or the skin respectively.
Aneurysmal Bone Cysts
Aneurysmal bone cysts (ABC) are benign tumors
that are most common prior to skeletal maturity.
They also have no potential for malignancy. ABCs
are usually found in the metaphyseal region of long
bones or the posterior elements of the vertebrae.
They can be primary or secondary lesions. Primary
lesions are more common in skeletally immature
patients and are associated with a genetic translocation that upregulates ubiquitin-specic protease 6
(USP6). Secondary lesions may arise from almost
any bone tumor, with giant cell tumor of bone the
most common. ABCs seen in adulthood are more
likely to be secondary lesions.
Radiographically, ABCs are eccentric, purely
lytic, and expansile. They can thin the cortex signicantly to the point it is only visible on
CT. Fluid-uid levels, indicating blood-lled
cysts, are seen on MRI.A soft tissue component
can also be seen on MRI.Histology shows benign
spindle cells surrounding blood-lled spaces
(Fig. 6.16). Telangiectatic osteosarcoma should
always be on the differential and biopsy is indi-
Fig. 6.16 Aneurysmal
bone cyst in a 14year
male. Plain radiographs
(a) and MRI showing
uid-uid levels (b)
124
J. Day and B. Adams
cated for lesions with more aggressive features,
such as a soft tissue component.
The most common treatment is a standard
intralesional procedure with open curettage
with the use of a local adjuvant. When the
lesion abuts an open physis, consideration
should be given to accepting a higher local
recurrence rate in order to avoid injury to the
physis. Another treatment option gaining
acceptance is the use of doxycycline.
Percutaneous doxycycline mixed with albumin
was rst used in the spine but is now being
used elsewhere. This has the obvious advantage of avoiding the morbidity of open surgery.
A number of technical variations have been
used, but results are promising [18].
Unicameral Bone Cysts
Unicameral bone cysts (UBCs), or simple bone
cysts, are non-neoplastic lesions that occur during skeletal growth (Fig.6.17). They are usually
found in the metaphysis and/or diaphysis of long
bones. The majority of lesions occur in the proximal humerus with the proximal femur being the
next most common site.
The lesions themselves are usually painless
although patients commonly have pain at presentation. Pain is indicative of either a fracture or the
micromotion from an impending fracture.
UBCs appear as a geographic expansile lytic
lesion on plain radiographs. In contrast to an
ABC, they typically expand the bone symmetrically and do not expand beyond the width of the
physis. If a fracture has occurred, a fragment of
the cortex can be seen within the cyst cavity; this
is known as the “fallen leaf” sign. Due to this
characteristic, non-aggressive appearance, they
can usually be diagnosed with plain lms alone,
and are rarely confused with other benign or
malignant tumors. Therefore, further staging
studies are usually not indicated.
UBCs have been treated with a wide variety of
percutaneous strategies. The outcomes for these
strategies are similar and the need for multiple
treatments is common. Good outcomes have
been obtained with injection of methylprednisolone and bone graft after aspiration of the lesion.
Pathologic fractures are allowed to heal. Ten to
fteen percent of lesions resolve after fracture
and fracture healing facilitates percutaneous
treatment in those lesions that do not resolve.
Internal xation is only necessary for lesions in
the proximal femur.
Fig. 6.17 A unicameral bone cyst of the proximal
humerus in the skeletally immature patient
Giant Cell Tumor ofBone
Giant cell tumor of bone is the prototypical
benign but aggressive tumor of bone. It is a
locally aggressive tumor starting in the metaphysis of long bones extending up to the subchondral
surface. They are most commonly found about
the knee but can be found in any bone with the
distal radius and sacrum being other notable
locations. Lesions are more common in females
than males. They most commonly occur between
the third and fth decades of life. Giant cell
tumors also have the notable distinction of being
benign and having a 2–5% chance of metastatic
spread to the lungs. Pain both at rest and at night
is the predominant symptom at presentation.
Giant cell tumor of bone has a readily recognizable appearance on plain radiograph when located
6 Tumors oftheMusculoskeletal System
125
in the long bones (Refer to Figs. 6.1 and 6.6). They
have a razor-sharp margin but typically lack a sclerotic rim. The lesion is usually eccentrically
located and extends to the subchondral bone.
Local advanced imaging is usually not necessary
for lesions in the appendicular skeleton, but MRI
and/or CT can be helpful for lesions in the pelvis
or spine. CT scan of the chest should be included
to screen for the presence of pulmonary metastasis. Preoperative biopsy is warranted for lesions in
the pelvis and spine and in more aggressive
appearing lesions to rule out malignant tumors.
Two basic cell types constitute typical giant
cell tumor of bone. The stroma is characterized
by polygonal to somewhat spindled cells containing central round nuclei. Scattered diffusely
throughout the stroma are benign, multinucleated
giant cells.
Treatment is most commonly an intralesional
procedure with the procedure providing a model
for the treatment of other benign tumors. A cortical window is made with a high-speed burr.
Curets are then used to remove all visible lesional
material; the initial portion can be sent for frozen
section to conrm the diagnosis. The high-speed
burr is then used to remove the reactive zone
around the lesion. A local adjuvant is then used to
kill any remaining tumor cells. Cryosurgery is
typically used at our institution. Reconstruction
involves lling the cavity with polymethyl methacrylate after placing bone graft along the subchondral surface. A locking plate or intraosseous
rush rods are usually placed prior to cementation
to increase torsional stability.
Not all giant cell tumors are best treated with
an intralesional procedure. If sufcient cortical
destruction has occurred or an expendable bone
is involved, wide resection is the treatment of
choice. Radiation has been used, particularly in
sacral lesions not amenable to surgery, but its use
should be avoided for the most part due to a signicant risk of malignant transformation. Tumor
osteolysis occurs through the receptor activator
of nuclear factor kappa beta (RANK) pathway.
Denosumab acts on this pathway and has a role in
the treatment of giant cell tumor. Denosumab is
used primarily to treat unresectable tumors or
patients with metastatic disease. It can also be
used in the preoperative setting to allow for an
intralesional procedure of a tumor that would
otherwise require wide resection. This should be
done with caution as high-recurrence rates have
been reported when adequate curettage of the
surrounding reactive bone is not performed.
Eosinophilic Granuloma
Eosinophilic granuloma is the term used for an
isolated lesion of Langerhans cell histiocytosis
(LCH). LCH can affect many different organ systems and the extent of disease varies widely.
Young children are most commonly affected.
Presenting symptoms for patients with skeletal
involvement includes pain, but may also include
local swelling and malaise, fever, or even
leukocytosis.
The skull is the most location with the pelvis
and proximal long bones also commonly affected.
In the long bones, lesions arise in the diaphysis or
metadiaphysis. Lesions may appear wellcircumscribed with a sclerotic rim or may have a
more permeative appearance with signicant
endosteal scalloping or periosteal reaction.
Eosinophilic granuloma is known as “the great
mimicker” due to its variable appearance that can
be similar to a wide variety of lesions. Because of
this, biopsy is necessary.
Isolated eosinophilic granulomas often resolve
without treatment or after needle biopsy alone.
Lesions can be successfully treated with intralesional steroid injection or with curettage and
bone grafting for lesions with more signicant
cortical involvement. Patients with multisystem
disease are treated with chemotherapy.
Metastatic Bone Disease, Myeloma,
Lymphoma, andPathologic
Fractures
Natural History
With an aging population and advances in the
treatment of many cancers, the overall number of
people living with cancer continues to increase.
126
J. Day and B. Adams
While the rate differs by primary tumor, bone is
the third most common site of metastatic disease.
Estimates of the prevalence of patients with metastatic disease range from 300,000 to 600,000
people in the USA.Bones in the axial skeleton
and proximal extremities are most often affected.
Within the spine, certain regions are more commonly affected than others. Metastatic spread
more frequently impacts the lumbar, thoracic,
than cervical spine. Batson’s venous plexus provides the anatomic basis for differences in bony
locations primarily impacted by metastatic disease spread [19]. Batson’s venous plexus
describes the paravertebral venous system that
connects the basivertebral veins and the pelvic
and thoracic veins.
A patient presenting with a bone lesion is
more likely to have metastatic disease than a primary bone tumor if they are over the age of 40
[19]. The most common primary tumors to
metastasize to bone are breast, prostate, lung,
renal, thyroid, and gastrointestinal cancers.
Although multiple myeloma arises from the bone
marrow, from the treatment perspective of an
orthopedic surgeon, it can be approached in the
same fashion as metastatic disease to the bone.
Orthopedic surgeons are frequently involved in
the care of these patients, so they must be able to
identify and treat patients with metastatic bony
disease.
Clinical Characteristics andPhysical
Examination
Pain is the most coming symptom for patients
presenting with metastatic disease to the bone.
Pain arising from the tumor itself is typical of
most bone tumors and is commonly described as
a dull, aching pain present at rest and pain that
wakes the patient up at night. Mechanical pain
that is worse with weight bearing is indicative of
strain from an impending fracture. Pathologic
fracture is another common initial presentation.
Tumors in the spine may cause neurologic symptoms as well. Differentiating between pain arising from the tumor, mechanical pain, and standard
musculoskeletal pain is an important factor in
deciding treatment. Pain from a bone lesion or
pathologic fracture is often the rst sign of cancer. In patients with a known primary cancer,
lesions are often seen on staging workup. While
these lesions are less likely to need orthopedic
treatment as they are often asymptomatic, attention should be paid to high-risk areas such as the
spine and proximal femur.
Radiographic Findings
Most metastatic disease to the bone appears as a
lytic lesion with cortical destruction. Appearance
ranges from a relatively well-dened punched
out lesion to a more permeative appearance. Most
lesions start in the medullary space, although
lung metastases can arise from the cortex. Breast
cancer metastases classically have a mixed lytic
and blastic appearance, with a lytic lesion with
some areas of bone formation in and around the
lesion. Most prostate cancer lesions are blastic
with dense sclerotic bone formation.
CT is helpful to assess the degree of cortical
involvement of the lesion. It is the most accurate
way to assess for fracture risk. CT also offers a
view of the intramedullary extent of a lesion. In a
patient with an unusual fracture mechanism, a
CT of the involved bone offers a quick way to
screen for cortical thinning or a marrow replacing
lesion that would be indicative of a pathologic
fracture.
MRI is certainly the most sensitive way to
screen for metastatic disease to the bone. It offers
the best view of the intramedullary extent of a
lesion. Signicant soft tissue extension is relatively rare in metastatic disease, but MRI is the
best way to characterize this. MRI is not helpful
in assessing fracture risk.
A technetium bone scan assesses for osteoblastic activity. It is most helpful to screen for
other skeletal disease. Purely lytic lesions will
not have signicant uptake on a bone scan, so a
skeletal survey is typically used to screen for
skeletal disease in myeloma. A bone scan is frequently recommended to assess if an incidentally
found lesion is “active” and requires further
workup or not. While bone scan may be helpful,
6 Tumors oftheMusculoskeletal System
127
its sensitivity and specicity differentiating
benign from malignant lesions is low.
Staging andBiopsy
When a patient presents either with a new bone
lesion or a pathologic fracture with suspected metastatic disease, biopsy and basic staging should be
done prior to any surgical treatment. Imaging and
lab work should typically be done prior to biopsy.
Standard imaging after local radiographs includes
a CT of the chest, abdomen, and pelvis and a
whole body technetium bone scan. Laboratory
work includes a complete blood count, complete
metabolic panel with calcium level, and serum
protein electrophoresis. Additional laboratory values may be obtained but tend to be low yield from
a diagnostic standpoint.
As noted above, a tissue diagnosis is necessary before any treatment is started. This step
should not be skipped or abbreviated in an effort
expedite care. In a patient with no prior history of
cancer or a patient with previously localized cancer who present with an isolated bone lesion,
biopsy should be done as a separate procedure.
This allows for a nal pathologic diagnosis rather
than relying on intraoperative frozen section. The
reliability of frozen section relies on a number of
factors, including the underlying histology, but
the overall accuracy compared to nal diagnosis
is estimated to be 90–95% [20]. Core-needle
biopsy is most commonly done and is often performed under image guidance. In a patient with a
known primary cancer and multiple bone lesions
or wide spread metastatic disease, biopsy at the
time of surgery is sufcient as the effect on treatment decisions is less. In general, patients with
previously biopsy proven metastatic disease do
not require additional biopsy unless there is a
suspicion for a second primary tumor.
Treatment Strategy
Patients with metastatic bone disease should
always be treated in the setting of a multidisciplinary team. In addition to administering che-
motherapy, medical oncologists can help assess
overall prognosis to guide treatment decisions.
Most patients with bone lesions should also
receive either a bisphosphonate or denosumab to
lessen the chance of additional skeletal disease.
External beam radiation can be used to palliate
pain from bone lesions and slow the local progression of disease. This can be done in isolation
or after surgical xation. Radiologists can assist
with biopsy or preoperative embolization of bone
lesions.
The orthopedic goals of treatment for patients
with metastatic disease differ from those targeted
toward patients with primary bone tumors.
Resecting the bone lesion does not affect outcome, so orthopedic treatment is aimed pain
reduction, fracture prevention, ambulation, and
preventing additional neurological sequelae.
There are exceptions to this rule with the most
obvious being patients with renal cell carcinoma
and isolated metastatic lesions. There is a survival benet to resection of the metastatic lesion
in this case.
Fracture prevention is an important part of the
orthopedic care of patients with metastatic disease (Fig. 6.18). Patients who undergo prophylactic xation of a lesion have shorter hospital
stays, faster return to activity, fewer complications, and better overall survival when compared
to those who require surgery to x a pathologic
fracture. Pain is the most important indicator of
fracture risk. A patient with functional pain (pain
with weight bearing) and a lytic lesion is at high
risk of fracture (Fig.6.19). Beyond this, CT is the
most accurate was to assess the cortical involvement of a lesion. Although not as accurate as CT,
a readily available way to assess fracture risk is
with plain radiographs and Mirel’s criteria [21].
The criteria used are the site of the lesion, pain
characteristics, the type of lesion, and the size as
judged on the plain radiograph. A score of greater
than 8 suggests prophylactic xation (Table6.4).
While surgical treatment should be tailored to
the individual patient, a number of general treatment strategies are frequently employed.
Treatment should allow immediate weightbearing whenever possible. Extensive surgical procedures should be avoided when possible.
128
ab
Fig. 6.18 Patient with
multiple myeloma and
multiple impending
fractures including the
distal femur and
proximal tibia.
Preoperative radiograph
(a) and postoperative
radiograph (b) after
curettage, cementation,
and locking plate
application
J. Day and B. Adams
ab c
Fig. 6.19 Proximal femur lesion from multiple myeloma (a). Patient had functional pain and unfortunately fractured
prior to prophylactic xation (b). He was treated with an intramedullary nail (c)
ab
6 Tumors oftheMusculoskeletal System
Fig. 6.20 Patient with
oligometastatic renal
cell carcinoma involving
proximal humerus (a)
Patient was treated with
resection and
endoprosthetic
reconstruction after
preoperative
embolization (b)
129
Table 6.4 Mirel’s criteria for prophylactic xation
Score 1 2 3
Site Upper limb Lower limb Peritrochanteric
Pain Mild Moderate Functional
Lesion Blastic Mixed Lytic
Size <1/3 1/3 to 2/3 >2/3
A score > 8 suggests prophylactic xation
best treated with arthroplasty often augmented
with cement, screws, or metal augments.
Soft Tissue Sarcomas
Soft tissue sarcomas (STS) are malignant tumors
1. Long bone lesions not immediately adjacent
to a joint are treated with intramedullary
xation. This can be supplemented with
cementation as needed.
2. Metaphyseal lesions not amenable to intramedullary xation can be treated with curettage, cementation, and locking plate xation.
3. Periarticular lesions are best treated with
endoprosthetic reconstruction when possible
(Fig.6.20).
4. Lesions in the pelvis most often do not require
surgical xation. Periacetabular lesions that
remain symptomatic after radiation can be
treated percutaneously after ablation. More
extensive lesions involving the acetabulum are
arising from or within the soft tissue. There are
over 50 types of soft tissue sarcomas. This heterogeneous group of tumors arise specically from
the supporting extraskeletal mesenchymal tissues
of the body, that is, muscle, fascia, connective tissues, brous tissues, and fat. Although they are
rare, accounting for less than 1% of malignancies,
they are about three times as common as bone sarcomas. The tendency for growth, recurrence rate,
rate and pattern of metastatic spread, and responsiveness to radiation and chemotherapy all differ
by tumor type. Although treatment is becoming
more specic to tumor type over time, the same
basic approach to treatment strategy can be used
for all soft tissue sarcomas.
130
ac
J. Day and B. Adams
Clinical Presentation
While some soft tissue sarcomas are more common in children and young adults, soft tissue
sarcomas as a whole become more common as
age increases. Soft tissue sarcomas can occur
though out the body, with about half occurring
in the extremities. The lower extremity is more
commonly involved than the upper extremity.
Most soft tissue sarcomas present as a painless
enlarging mass. They can be rm or soft. If they
are located supercial to the fascia, they may be
mobile. While only about 1% of supercial
masses are sarcomas, half of all sarcomas are
supercial. Systemic signs, such as fever, malaise, and weight loss, and laboratory changes are
rare. The nonspecic presentation is the cause of
frequent missed diagnosis and unplanned excisions. A degree of clinical suspicion should be
maintained when approaching any soft tissue
mass, and a large, deep mass should be presumed
to be a sarcoma until proven otherwise.
Radiographic Findings
Plain radiographs often show a soft tissue
shadow corresponding to the mass. They can be
useful to look for calcications within the mass.
Plain radiographs can also assess for any secondary involvement of the bone. MRI is the most
useful imaging modality to assess soft tissue sarcomas. The multiplanar images give a detailed
view of the extent of the mass and its relationship to the surrounding muscular compartments
and neurovascular structures (Fig.6.21). MRI is
relatively unhelpful from a diagnostic standpoint, however. The imaging characteristics of a
soft tissue sarcoma are nonspecic on MRI.In
general, the mass is heterogeneous in nature and
relatively dark, or isointense to muscle, on the
T1 weighted images and bright on the T2
weighted images. Postcontrast imaging reveals
variable enhancement.
Biopsy andStaging
Once imaging is completed, biopsy is necessary.
Biopsy is typically done as a needle biopsy. This
can be done in the clinic for supercial masses
that can be easily palpated. For masses that are
deep, close to major nerves or vessels, or appear
to be largely necrotic on MRI, biopsy can be
done under ultrasound or CT guidance. Small,
supercial lesions can often be removed as an
excisional biopsy. Biopsy should be guided by
the treating surgeon.
Fig. 6.21 High-grade
undifferentiated
pleomorphic sarcoma of
the anterior thigh. The
tumor is heterogenous
and relatively dark on
the T1 weighted
sequence (a), bright on
the T2 weighted
sequence (b), and has
nodular enhancement
after contrast
administration (c). This
is the classic appearance
of a soft tissue sarcoma
and is not specic to
subtype
b
6 Tumors oftheMusculoskeletal System
131
Soft tissue sarcomas have a similar pattern of
spread as bone sarcomas. Because of this staging
involves an MRI of the tumor and surrounding
compartments, CT of the chest, and a bone scan.
Treatment
Treatment for soft tissue sarcoma involves wide
resection of the mass. For many low-grade
lesions, no other treatment is necessary
(Fig.6.22). High-grade lesions and those with a
propensity for local recurrence require the addition of either radiation or chemotherapy. The
most widely accepted treatment for large, highgrade soft tissue sarcomas is a combination of
wide resection and external beam radiation [22].
Radiation can be given in the preoperative or
postoperative setting. Standard dosing is
50–65Gy delivered over 25–30 fractions. While
the sequence of radiation and surgery does not
affect overall survival, there are advantages and
disadvantages to each. Preoperative radiation
allows for a lower overall dose and slightly
shorter treatment interval. It can make the pseudocapsule easier to dissect from the surrounding
tissue and potentially make the tumor smaller.
The major disadvantage to preoperative radiation
is an increase in the wound complication rate to
about 30% [23]. Postoperative operative radia-
tion has the advantage of a lower wound complication rate. Disadvantages stem from the higher
overall dose and larger treatment area that is necessary. These include increased brosis, risk of
fracture, and potentially a higher rate of radiation
induced sarcoma. Pre- or postoperative radiation
reduces the chance of local recurrence to less
than 10% [23].
Chemotherapy in the treatment of soft tissue
sarcoma remains controversial. It is routinely
used in patients with metastatic disease. In isolated disease, its use is less widespread. Data on
overall survival has shown variable effectiveness.
This is probably due at least in part to the wide
heterogeneity of diseases and their variable
responsiveness to chemotherapy [24]. A large
meta-analysis of randomized controlled trials
showed a 11% improvement in overall survival in
patients treated with a regimen including both
doxorubicin and ifosfamide for large, deep, highgrade soft tissue sarcomas of the extremity [24].
Chemotherapy for isolated tumors is typically
given in the preoperative setting and most often is
used in conjunction with either pre- or postoperative radiation therapy.
Patients receiving unplanned surgery for soft
tissue sarcoma remain persistently common.
These patients have a 50% chance of having
residual disease after the initial surgery. Treatment
for these patients is effectively the same as for an
ab
Fig. 6.22 Low-grade bromyxoid sarcoma (a). The sciatic nerve is immediately adjacent to the mass (arrows). The
nerve was freed from the mass leaving the surrounding adventitial tissue as the margin (b)