Chondrosarcoma

Practice Essentials
Chondrosarcoma is a collective term for a group of tumors
that consist predominantly of cartilage and that range
from low-grade tumors with low metastatic potential to
high-grade, aggressive tumors characterized by early
metastasis.

Nerve dysfunction of the lumbosacral plexus or the

sciatic or femoral nerves, with pelvic lesions near a
neurovascular bundle
Limitation of joint range of motion and disturbance of
joint function, with chondrosarcomas close to a joint
Pathologic fracture
Diagnosis
The workup rests primarily on diagnostic imaging modalities
(eg, plain radiography, as well as CT and MRI).

Essential
update:
Stereotactic
radiosurgery
demonstrates superiority over conventional radiotherapy
for sarcoma that has metastasized to the spine
In a study of 88 patients with high-grade sarcoma that had
spread to the spine, Folkert and colleagues found that
treatment with image-guided stereotactic radiosurgery
(IG-SRS) provided 87.9% local control at 12 months. By
comparison, 12-month local control rates with conventional
radiotherapy have historically ranged from 50-77%. In the
study, 12-month local control was better with singlefraction IG-SRS than with hypofractionated IG-SRS
(90.8% vs 84.1%; P = .007). IG-SRS treatment was well
tolerated.[1, 2]
Chondrosarcoma types and grades

Plain radiography

Different types of chondrosarcoma have been described,

as follows:

Conventional chondrosarcoma, which accounts for nearly

90% of all chondrosarcomas
Dedifferentiated chondrosarcoma
Clear cell chondrosarcoma
Mesenchymal chondrosarcoma
Juxtacortical chondrosarcoma
Secondary chondrosarcoma
Benign cartilage lesions can be difficult to differentiate
from slow-growing, low-grade chondrosarcomas. Secondary
chondrosarcoma can occur in a previously benign
cartilaginous lesion.

Chondrosarcomas can be classified into the following 3

histologic grades, depending on findings of cellularity,
atypia, and pleomorphism:

Grade I (low grade) Cytologically similar to

enchondroma[3] ; cellularity is higher, with occasional
plump nuclei with open chromatin structure
Grade II (intermediate grade) Characterized by a
definite and increased cellularity; distinct nucleoli are
present in most cells, and foci of myxoid change may be
seen
Grade III (high grade) Characterized by high
cellularity, prominent nuclear atypia, and the presence of
mitosis
The higher the grade, the more likely the tumor is to
spread and metastasize. Grade I lesions rarely metastasize,
whereas 10-15% of grade II lesions and more than 50%
grade III lesions metastasize.
Clinical Presentation
Clinical features of chondrosarcomas are as follows:

Deep, dull, achy pain

Pain at night

Chondrosarcomas are usually large (> 5 cm)

The bony contour appears thinned and expanded, and
multiple surface erosions (endosteal scalloping) are seen
Cortical thickening may also be visible
The extent of bony destruction depends on the histologic
grade of the tumor
The periosteum overlying the tumor may be elevated; this
leads to new bone formation that results in hazy cortical
irregularity and fuzziness or parallel periosteal new bone
formation
Variable amounts of stippled or punctuate calcification
are seen
In some cases, calcifications resemble popcorn or
commas; in others, arcs and rings of calcification are seen
around lobules of cartilage
The typical appearance
of a dedifferentiated
chondrosarcoma is an area of punctate opacities
surrounded by a permeating, destructive lytic lesion
Secondary malignant degeneration should be suspected
when sequential follow-up radiographs of benign cartilage
tumors show the following findings:
Growth of the lesion
Decreased calcification and increased lysis
Endosteal erosion
Permeative lesions with destruction of the cortex
Soft-tissue mass
Growth in a previously stable exostosis or enchondroma in
an adult
Expansion of the cartilaginous cap in exostosis

MRI

The investigation of choice for assessing the extent of a

chondrosarcoma
Helps delineate the extent of soft-tissue involvement
Important for preoperative planning and for confirming
or diagnosing recurrence at a surgically treated site

CT scanning

May be useful for detecting subtle calcifications in the

matrix when the diagnosis is in doubt
May improve visualization of bony destruction and depict
the extent of bony delineation

Biopsy

Performing a truly representative biopsy of a

chondrosarcoma is challenging because the lesion is
composed of areas that carry different histologic grades.
Identification of the most aggressive component of the
tumor is critical. Considerations when performing biopsy
are as follows:

Biopsy should be directed at areas that may harbor foci

of high-grade tumor, such as areas of endosteal
scalloping, soft-tissue components, or diffusely enhancing
areas with minimal mineralization
Biopsy can be performed with either an open or a closed
technique[4]
Closed biopsy involves fine-needle aspiration (FNA)
cytology or core biopsy
Discussion with the radiologist and the histopathologist is
essential in obtaining the correct tissue for biopsy
With cartilaginous tumors, histopathologic examination of
the biopsy specimen alone does not permit accurate
classification of the tumor
Biopsy should be done as meticulously as possible, to avoid
seeding of the biopsy tract with bone-tumor cells
When a definitive procedure is performed, the whole
tract should be completely excised
Staging
The Enneking staging system for musculoskeletal sarcomas
is applicable to chondrosarcomas, as follows[5] :

Stage I (low-grade tumor): I-A, intracompartmental; I-B,

extracompartmental
Stage II (high-grade tumor): II-A, intracompartmental;
II-B, extracompartmental
Stage III (distant metastasis)
Management
Surgery is the primary treatment for any chondrosarcoma.
Complete, wide surgical excision of the chondrosarcoma is
the preferred method when it is feasible. Radiotherapy and
chemotherapy play limited roles in primary treatment. An
exception is their use as adjuvant therapy or palliative
treatment for tumors in surgically inaccessible areas or
diffuse metastasis.
Image library

Plain radiograph shows

a low-grade chondrosarcoma in the pelvis(B). An incidental
finding is that the proximal femur contains a benign
enchondroma(A).
Overview
Chondrosarcoma is a tumor of mesenchymal origin that
predominantly is made of cartilage; it is the second most
common primary malignant tumor of the bone.
Chondrosarcoma is a collective term that encompasses a
group of heterogeneous lesions with diverse morphologic
features and clinical behaviors.[6] These lesions range from
low-grade tumors with low metastatic potential to highgrade, aggressive tumors characterized by early
metastasis.
The term chondrosarcoma should be used for a malignant
tumor of the cartilage when the tumoral matrix is entirely

cartilage. If the tumor exhibits bone-forming elements and

primitive mesenchymal elements in addition to cartilaginous
differentiation, it should not be classified as a
chondrosarcoma, because its clinical behavior and
therapeutic responses differ from those of a primary
malignant chondrosarcoma.
Tumors with the aforementioned elements (ie, the
presence of bone-forming elements and primitive
mesenchymal elements in addition to cartilaginous
differentiation) usually behave like chondroblastic
osteosarcomas and are more aggressive than the
conventional chondrosarcomas.
Different types of chondrosarcoma have been described,
as follows:

Conventional chondrosarcoma, which accounts for nearly

90% of all chondrosarcomas
Dedifferentiated chondrosarcoma
Clear cell chondrosarcoma
Mesenchymal chondrosarcoma
Juxtacortical chondrosarcoma
Secondary chondrosarcoma
Despite various investigations, it may be difficult to
differentiate a benign cartilage lesion from a slow-growing,
low-grade chondrosarcoma. Secondary chondrosarcoma can
occur in a previously benign cartilaginous lesion.
Pathophysiology
Chondrosarcomas
secondary lesions
chondrosarcomas
chondrosarcomas
cartilage.

may be divided into primary and

on the basis of their origins.[7] Primary
arise de novo, whereas secondary
arise from preexisting lesions of the

Except for their origin in preexisting cartilaginous

conditions, secondary chondrosarcomas are similar to
conventional chondrosarcomas in all respects. In addition,
the genes responsible for the lesions depend on the
primary
benign
cartilaginous
condition.
Secondary
chondrosarcomas occur in individuals with Ollier disease,
Maffucci syndrome, multiple hereditary exostosis
(diaphyseal aclasis), solitary osteochondroma, solitary
enchondroma, solitary periosteal enchondroma, Paget
disease, or radiation injury.
Genetics
Bovee et al reported that most peripheral chondrosarcomas
had
a
higher
proliferation
rate
on
Ki-67
immunohistochemistry and that they were associated with
loss of heterozygosity at many loci.[8, 9] Only a few
chondrosarcomas had anomalies, which were restricted to
9p21, 10, 13q14, and 17p13. These anomalies were
peridiploid or near-haploid. Structural chromosomal
aberrations and genetic instability were seen during
cytogenetic analysis of well-differentiated, grade I
chondrosarcomas. Nearly all grade III and some grade II
chondrosarcomas were aneuploid.
Amplification
of
the c-myc protooncogene[10] and fos/jun[11] has also been implicated in the
pathogenesis of chondrosarcoma.

With
extraskeletal
myxoid
chondrosarcomas,[12] the
t(9;22)(q22;q12)
translocation
is
common,
though
t(9;17)(q22;q11.2) has also been described. Numerous
genetic alterations have been found for dedifferentiated
chondrosarcomas, but a shared loss of chromosome 13
suggests that the differentiated and dedifferentiated
components originate from a common precursor.
Histologic grading
Chondrosarcomas can be classified into the following 3
histologic grades, depending on findings of cellularity,
atypia, and pleomorphism:

Grade I (low grade) This is cytologically similar to

enchondroma[3] ; cellularity is higher, with occasional
plump nuclei with open chromatin structure
Grade II (intermediate grade) This is characterized by
a definite and increased cellularity; distinct nucleoli are
present in most cells, and foci of myxoid change may be
seen
Grade III (high grade) This is characterized by high
cellularity, prominent nuclear atypia, and the presence of
mitosis
The higher the grade, the more likely the tumor is to
spread and metastasize. Grade I lesions rarely metastasize,
whereas 10-15% of grade II lesions and more than 50%
grade III lesions metastasize.
Low-grade chondrosarcomas resemble benign cartilaginous
tumors, and it is difficult to differentiate the 2 lesions on
the basis of histologic features alone. The essential
differences are the limited growth potential of benign
cartilaginous tumors and the slow growth capacity of lowgrade chondrosarcomas.

Fewer than 2% of all chondrosarcomas are mesenchymal

chondrosarcomas. The maxilla and the mandible are the
most common sites of involvement, followed by the
vertebrae, the ribs, the pelvis, and the humerus. The
appendicular skeleton is rarely involved.
Juxtacortical chondrosarcomas are rare and generally
involve the surface of the diaphysis or metaphysis of long
tubular bones.
Age-, sex-, and race-related demographics
Incidences do not differ among ethnic groups. Sex and age
distributions are listed in the table below.
Table 1. Sex Ratios and Ages of Peak Incidence for
Different Types of Chondrosarcoma (Open Table in a new
window)
Chondrosarcoma
Conventional

Male-to-Female
Ratio

Frequency by tumor type

Conventional central chondrosarcomas account for nearly
80-90% of all chondrosarcomas and 20-27% of all primary
bone sarcomas[13] .They demonstrate a predilection for the
axial skeleton. Rates of involvement are as follows: pelvis
and ribs, 45%; ilium, 20%; femur, 15%; humerus, 10%; and
others, 10%. The spine and the craniofacial bones are
rarely involved.
Dedifferentiated chondrosarcomas are responsible for as
many as 10% of all chondrosarcomas. The femur is the site
most commonly involved, accounting for one-third of all
dedifferentiated chondrosarcomas. The other sites of
involvement are the pelvis (20%), the humerus (16%), the
ribs (7%), and the scapula (7%).
Clear cell chondrosarcomas account for fewer than 5% of
all chondrosarcomas. They have a predilection for the ends
of long tubular bones, involving the epiphysis. Like
chondroblastomas, these lesions extend to involve the
articular cartilage. The proximal aspect of the femur is the
site most often affected (45%), followed by the proximal
portion of the humerus.

Peak

Almost 1:1 (slight 50-70

y
(most
male
common > 50 y,
predominance)
gradual
increase
with age)

Dedifferentiated Similar
to
ratio above

the > 50 y

Clear cell

2.4:1

20-40 y (common
10-90 y)

Mesenchymal

1:1

20-30 y (common in
teenagers
and
young adults)

Juxtacortical

1:1

20-40 y

Dedifferentiated chondrosarcomas are more aggressive

than grade III conventional chondrosarcomas.
Epidemiology

Age
of
Incidence

Clinical Presentation
Deep, dull, achy pain is a common symptom in
chondrosarcomas. Pain at night is another feature.
Although the finding of pain is important for distinguishing
malignant lesions from benign cartilaginous lesions, it can
be somewhat unreliable when the small bones of the hands
and feet are involved.
If the lesion is near a neurovascular bundle, as pelvic
lesions are, the patient may present with nerve dysfunction
of the lumbosacral plexus or the sciatic or femoral nerves.
If a chondrosarcoma is close to a joint, it may limit the
joints range of motion and disturb its function. These signs
are common with juxtacortical chondrosarcomas, though
they can also be present with pathologic fractures. More
than half of all patients with dedifferentiated
chondrosarcomas present with a pathologic fracture.
The mean interval from pain to diagnosis is 19.4 months for
grade I and grade II chondrosarcomas and 15.5 months for
grade III chondrosarcomas, as per the Rizzoli institute
experience.[14]

Clear
cell
chondrosarcomas
and
mesenchymal
chondrosarcomas can produce symptoms for longer than 1
year because of their low-grade nature. Mesenchymal
chondrosarcomas can manifest as a soft-tissue mass.

chondrosarcomas from osteosarcomas, which results in

perpendicular periosteal new bone formation that has a
sunburst appearance.
Variable amounts of stippled or punctuate calcification are
seen. In some cases, calcifications resemble popcorn or
commas; in others, arcs and rings of calcification are seen
around lobules of cartilage. The typical appearance of an
dedifferentiated chondrosarcoma is an area of punctate
opacities surrounded by a permeating, destructive lytic
lesion.

Differential Diagnosis
The differential diagnosis includes the following conditions:

Chondroblastoma
Chondroma
Chondromyxoid fibroma
Chordoma
Fibrosarcoma
Fibrous dysplasia
Fibrous histiocytoma
Metastatic carcinoma
Osteosarcoma
Paget sarcoma[15, 16]
Synovial chondromatosis
Laboratory Tests
Routine blood investigations are performed as part of
preoperative examinations. Tests of liver, lung, and renal
functionwith bone biochemical analysesmay be used for
preoperative assessment and for evaluations of the distant
spread of tumors (metastasis).
Otherwise, workup rests primarily on diagnostic imaging
modalities (eg, plain radiography, as well as computed
tomography [CT] and magnetic resonance imaging [MRI]).
Plain Radiography
The typical appearance of a cartilaginous lesion on plain
radiographs is discrete calcification (see the image below).
The lesion may be radiolucent on radiographs, which may
show stippled or punctate calcifications. Appearances vary
from lesion to lesion, depending on the amount of
mineralization that has occurred.

Plain radiograph shows

a low-grade chondrosarcoma in the pelvis(B). An incidental
finding is that the proximal femur contains a benign
enchondroma(A).
Characteristic findings
Chondrosarcomas are usually large (> 5 cm). The bony
contour appears thinned and expanded, and multiple
surface erosions (endosteal scalloping) are seen.
Chondrosarcomas sometimes produce cortical thickening as
well. The extent of bony destruction depends on the
histologic grade of the tumor.
The periosteum overlying the tumor may be elevated. This
effect leads to new bone formation that results in hazy
cortical irregularity and fuzziness or parallel periosteal new
bone
formation.
This
process
differentiates

Endosteal scalloping and thinning may be observed with

most lesions. Sometimes, as in clear cell chondrosarcoma,
images depict cortical thickening around the lysis with
endosteal
expansion.
High-grade
lesions
produce
permeative lesions as they destroy the cortex.
Findings suggestive of secondary malignant degeneration
Secondary malignant degeneration should be suspected
when sequential follow-up radiographs of benign cartilage
tumors show the following findings:

Growth of the lesion

Decreased calcification and increased lysis
Endosteal erosion
Permeative lesions with destruction of the cortex
Soft-tissue mass
Growth in a previously stable exostosis or enchondroma in
an adult
Expansion of the cartilaginous cap in exostosis
MRI
MRI is the investigation of choice for assessing the extent
of a chondrosarcoma; it also helps delineate the extent of
soft-tissue involvement (see the images below). Its ability
to determine the exact extent of the tumor and the
degree to which various soft-tissue compartments are
involved make it an important tool for preoperative
planning. In addition, MRI is an important study for
confirming or diagnosing recurrence at a surgically treated
site.

T1-weighted MRI shows

a low-signal-intensity lesion in the pelvis, a chondrosarcoma.

T2-weighted
MRI
shows a high-signal-intensity lesion in the pubis, a

chondrosarcoma.
MRI of
a chondrosarcoma shows contrast enhancement of the
lesion. A-Enchondroma; B-Chondrosarcoma
CT, Bone Scan, and Ultrasonography
CT may be useful for detecting subtle calcifications in the
matrix so as to help diagnose cartilaginous tumors when the
findings are in doubt. CT may improve visualization of bony
destruction and depict the extent of bony delineation.
Bone scanning and chest CT are used for systemic staging
of the tumor before surgical treatment. Abdominal CT,
abdominal ultrasonography, or both may be used for this
purpose as well.
Biopsy
Performing a truly representative biopsy of a
chondrosarcoma is challenging because the lesion is
composed of areas that carry different histologic grades.
A tumor should be graded on the basis of its most
aggressive component. Hence, the challenge during biopsy is
to ensure that the most aggressive part is identified.
Biopsy should be directed at areas that may harbor foci of
high-grade tumor, such as areas of endosteal scalloping,
soft-tissue components, or diffusely enhancing areas with
minimal mineralization. The rationale behind targeting areas
with minimal calcification of the matrix is that high-grade
areas usually contain myxomatous matrix that is relatively
resistant to calcification. By comparison, low-grade areas
usually contain chondroid matrix that calcifies.
Biopsy can be performed with either an open or a closed
technique.[4] Closed biopsy involves fine-needle aspiration
(FNA) cytology or core biopsy. Core biopsy through a TruCut biopsy or a core-needle biopsy yields results equivalent
to those of open biopsy. Discussion with the radiologist and
the histopathologist is essential in obtaining the correct
tissue for biopsy. However, with cartilaginous tumors,
histopathologic examination of the biopsy specimen alone
does not permit accurate classification of the tumor.
Chondrosarcomas are mostly gelatinous. Therefore, the
risk of seeding of the biopsy tract with bone-tumor cells is
high. Furthermore, because the cartilaginous tumor matrix
is avascular, malignant cartilage cells can survive when they
are spilled into a wound. For these reasons, biopsy of a
chondrosarcoma should be done as meticulously as possible.
When a definitive procedure is performed, the whole tract
should be completely excised.
In summary, biopsy of a chondrosarcoma is not a benign
procedure and must therefore be planned efficiently. The
normal principles of biopsy for any tumor should be
adhered to, as follows:

The biopsy should be done by the surgeon who will be

providing definitive treatment; if that is not feasible, it
should least be done after a detailed discussion with the
surgeon
The path selected for the biopsy should be in the same
line as that to be used for the definitive operation, so
that the biopsy tract will be excised in the course of
surgical treatment
The biopsy tract should follow a straight line and avoid
contamination
of
joints,
uninvolved
structures,
neurovascular bundles, and bones; preoperative planning is
essential, and the biopsy tract should not be placed in the
flap that will be used for coverage after excision of the
tumor
Transverse incisions should be avoided
Appropriate handling of tissue after the biopsy specimen
is obtained is essential; this should be decided before the
operation in consultation with the histopathologist
Adequate hemostasis should be achieved before closure
A suture may be used to mark the skin entry site to
facilitate excision of the biopsy tract during definitive
surgery
Histologic Findings
Gross findings
The cartilaginous nature of chondrosarcomas is apparent on
cross-section. The lesion has a grossly lobulated
architecture with translucent, gray-black hyaline tissue.
Intense mineralization accentuates lobules at the
periphery. The myxoid variety may be gelatinous. Two
featurestumoral oozing from the cut surface and frank
liquefactionhelp in differentiating malignant tumors from
benign tumors of cartilage. Chondrosarcomas appear as
sessile, lobulated masses in the pelvis and have large
extraosseous components.
Microscopic findings
A tumor must be uniformly cartilaginous to be classified as
a chondrosarcoma. Chondrosarcomas contain chondroid
matrix with increased cellularity, and they have binucleate
cells. As noted (see Pathophysiology), these tumors are
classified into low, intermediate, and high grades (grades I,
II, and III, respectively).
Low-grade
chondrosarcomas
typically
look
like
enchondromas.
Intermediate-grade
tumors
contain
cartilage cells that have enlarged round nuclei with
prominent nucleoli. Their cytoplasm shows prominent rough
cytoplasmic reticula, mitochondria, and large amounts of
glycogen. High-grade tumors are characterized by high
cellularity, prominent nuclear atypia, and mitosis. Highgrade tumors constitute 5-10% of all chondrosarcomas.
Immunohistochemical tests show the presence of S-100
protein and vimentin in low- and intermediate-grade
chondrosarcomas. However, results for S-100 protein and
vimentin are focally negative in high-grade lesions,
especially
among
dedifferentiated
areas.
Immunohistochemical staining is not routinely indicated for
the diagnosis of cartilaginous tumors. These lesions are
more readily diagnosed with conventional hematoxylin-eosin
stains than with immunohistochemical stains.

Measurements of DNA ploidy reveal that low-grade tumors

are diploid, whereas intermediate- and high-grade tumors
are aneuploid. Aneuploidy is correlated with the
aggressiveness of the chondrosarcoma.

up clinical evaluations and radiologic investigations are

required.
Outcome and Prognosis
The prognosis is correlated with the grade and stage of the
lesion at the time of diagnosis.[18] The location of the lesion
is also important because tumors in areas where complete
wide resection is possible are associated with better
prognoses.

Chromosomal anomalies reveal an accumulation of p53

protein; in high-grade lesions, this finding indicates a poor
prognosis.
Staging
The Enneking staging system for musculoskeletal sarcomas
is applicable to chondrosarcomas, as follows[5] :

Recurrence and distant metastasis may develop. The

metastasis rate for primary chondrosarcoma is higher than
that for secondary chondrosarcoma, and the rate of
distant metastasis is higher in patients with local
recurrence than in those without local recurrence.

Stage I (low-grade tumor) - I-A, intracompartmental; IB, extracompartmental

Stage II (high-grade tumor) - II-A, intracompartmental;
II-B, extracompartmental
Stage III (distant metastasis)
Treatment & Management

Mortality and morbidity data for the various types of

chondrosarcomas are summarized below.
Conventional chondrosarcoma

Radiotherapy and chemotherapy

Radiotherapy and chemotherapy play limited roles in
primary treatment. An exception is their use as adjuvant
therapy or palliative treatment in surgically inaccessible
areas.
Diffuse metastasis is usually an indication for systemic
radiotherapy or chemotherapy. The results are generally
poor, as they are in dedifferentiated chondrosarcoma, for
which the 1-year survival rate is 10%.[13]
Chemotherapy and radiotherapy may be used in
dedifferentiated chondrosarcoma because distant systemic
metastasis may be present at the time of diagnosis.
However, current evidence indicates that surgery with
clear margins remain the primary treatment for
dedifferentiated chondrosarcomas a well. Grimer et al
recommended further use of chemotherapy in such cases
only as a trial or treatment protocol.[17]
Surgical therapy
Surgery is the primary treatment for any chondrosarcoma.
Complete, wide surgical excision of the chondrosarcoma is
the preferred method when it is feasible. Success depends
on the stage of the disease, with low-grade
intracompartmental lesions offering the best prognosis
after complete surgical resection with surgically clear
margins. Lesions with isolated pulmonary metastasis can
still be surgically resected if metastasectomy of the
isolated pulmonary lesion is feasible.
Chondrosarcomas in the appendicular skeleton are amenable
to wide excision. Hence, these lesions tend to fare better
with surgery than those occurring in the axial skeleton.
Complete removal of most lesions in the axial skeleton is
difficult. Complete en-bloc excision can cure clear cell
chondrosarcomas. Surgery remains the primary treatment
for mesenchymal chondrosarcomas as well.
Follow-up
Regular follow-up is required to rule out local recurrences
of chondrosarcoma and distant metastases. Regular follow-

Evans et al[19] showed that the survival rate depends on the

histologic grade of the tumor, as follows:

Grade I tumors - 90% survival at 5 years

Grade II tumors - 81% survival at 5 years
Grade III tumors - 29% survival at 5 years
Overall, the 5-year survival rate for conventional
chondrosarcomas is 48-60%.[13]Intralesional surgery is not
advised even in grade I lesions, especially in the pelvis,
because the local recurrence rate is 100% in such cases.
Grade I tumors do not metastasize, whereas 66% of grade
III tumors do. The most common sites for metastases are
the lungs. Recurrences typically appear 5-10 years or longer
after surgery.
Dedifferentiated chondrosarcoma
Dedifferentiated chondrosarcoma is highly lethal. It is
associated with a 10% survival rate after 1 year. Even with
early surgical treatment, disseminated hematogenous
metastasis occurs in most patients.[13]
Clear cell chondrosarcoma
Although clear cell chondrosarcomas are low-grade tumors,
they can lead to distant metastasis. Late recurrences (>10
y) have been described. Overall, the recurrence rate is
16%[13] .
Mesenchymal chondrosarcoma
The 5-year survival rate for mesenchymal chondrosarcoma
is less than 50%, with an overall 10-year survival rate of
28%.[13]
Juxtacortical chondrosarcoma
Juxtacortical chondrosarcomas are low- to intermediategrade lesions; the prognosis for these tumors is better
than that for other tumors.[13]