Secondary Chondrosarcoma

Secondary Chondrosarcoma
Local recurrence is a significant
problem for patients with secondary
chondrosarcoma, and this may affect
10% to 20% of patients. 4,5,13 Local
control of disease is particularly important
for patients with pelvic and
centrally located disease. In one series,
more deaths occurred from local
recurrence than from distant metastasis.
4 The ramifications of local recurrence
on the patient’s chances for
survival should not be underestimated.
Local recurrence of conventional,
primary low-grade chondrosarcoma
has been associated with
decreased survival, increased metastasis,
and increased grade of tumor.
44,45 Thus, an oncologically
sound operation for the primary tumor
with wide surgical margins is of
critical importance to maximize the
chances for long-term patient survival.
Summary
Secondary chondrosarcoma is an uncommon
tumor that arises from a
benign cartilaginous lesion. The tumor
frequently develops in the context
of a syndrome that produces
multiple cartilaginous tumors, including
HME, Ollier disease, and
Maffucci syndrome. It appears most
commonly in the pelvis and proximal
femur. Several signs may alert the clinician
to the possibility of malignant
transformation, such as new onset of
pain, sudden growth of a lesion, and
radiographic changes.
The size of the cartilage cap of an
osteochondroma is important. MRI
with gadolinium contrast may help
define the thickness of the cap. Although
no demarcation in size of the
cap can be considered diagnostic of
malignant transformation, a cap
thickness of >1 cm is worrisome,
particularly when there is documented
growth of the cap.
The overall prognosis for patients
is good. Most tumors are low grade,
and distant metastasis is uncommon.
Secondary chondrosarcomas must be
distinguished from dedifferentiated
chondrosarcomas, which are very
aggressive, high-grade tumors with a
poor prognosis. The management of
secondary chondrosarcoma relies
chiefly on wide surgical excision. Inadequate
surgical resection can lead
to uncontrollable local recurrence,
which is perhaps as likely a cause of
demise as distant metastasis.
References
Evidence-based Medicine: Levels of
evidence are described in the table of
contents. In this article, references
23, 30, 31, 33, 34, 37, and 38 are
level III studies. References 2-18, 27-
29, 32, 35, 39-42, 44, and 45 are
level IV studies. References 1, 19-21,
24-26, 36, and 43 are level V expert
opinion.
Citation numbers printed in bold
type indicate references published
within the past 5 years.
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