Secondary Chondrosarcoma

Patrick P. Lin, MD, et al
Table 1
Major Series on Secondary Chondrosarcoma
Study
No. of
Cases
Preexisting
Lesion (No.)
Grade 14
(No. of Cases)
52 Solitary OC (21), HME (4),
Coley and
N/A
Higinbotham 6 enchondroma (23), Ollier
disease (4)
Garrison et al 7 75 Solitary OC (40), HME (35) 1 (64), 2 (10), 3 (1)
Hudson et al 8 15 Solitary OC (12), HME (3) 1 (11), 2 (3), 3 (1)
Sun et al 12 5 Maffucci syndrome (all) 1 (1), 2 (3), N/A (1)
Liuetal 9 12 Ollier disease (all) 1 (10), 2 (2)
Merchan et al 10 4 HME (all) 1 (4)
Wuisman
45 Solitary OC (16), HME (29) 1 (30), 2 (13), 3 (2)
et al 13
Schaison
29 Solitary OC (17), Ollier
1(9),2(18),N/A(2)
et al 11 disease (12)
Ahmed et al 4 107 Solitary OC (61), HME (46) 1 (97), 2 (10)
Altayetal 5 32 Solitary OC (14), HME (10), enchondroma
(6), Ollier disease
(1), Maffucci syndrome (1)
1(28),2(4)
HME = hereditary multiple exostosis, N/A = not available, OC = osteochondroma
Table 2
Risk Factors for Secondary
Chondrosarcoma
Clinical
Pain
Increasing size of a palpable lesion
Male predominance (2:1)
Location in the pelvis or hip
Peak age in mid 30s
Radiographic
Surface irregularity
Blurriness of the border
Osteochondroma >5 cm
Increase in size of osteochondroma
Cartilage cap >2 cm
Inhomogeneous mineralization of large
cartilage cap
Genetic
Hereditary multiple exostosis (EXT1,
EXT2, EXT3 mutations)
Ollier disease
Maffucci syndrome
(5.5%) underwent dedifferentiation. 2
Most cases of dedifferentiated chondrosarcomas
appear to originate
from central primary chondrosarcoma;
it is estimated that the fraction
of primary chondrosarcomas undergoing
dedifferentiation may be as
high as 15.4%. 3 In most cases, the
prognosis of patients with dedifferentiated
chondrosarcoma is dismal,
which is quite unlike the prognosis
of patients with secondary chondrosarcoma.
Secondary chondrosarcoma is a
well-recognized clinical diagnosis.
However, relatively little has been
published on the subject, perhaps as
a result of the rarity of the condition.
In a review of the literature, we
found only 10 studies pertaining directly
to the subject that were not
case reports (Table 1).
Clinical Presentation
The most frequent presenting complaints
with secondary chondrosarcoma
are a palpable mass and pain.
In many cases, the signs and symptoms
are subtle, and the complaints
are not severe. Large masses in the
pelvis and thigh are easily hidden
from patient and clinician alike. New
onset of pain in a preexisting osteochondroma
should alert the physician
to the possibility of enlargement
of the cartilaginous cap. Rarely,
some tumors in the pelvis may cause
urinary and bowel irregularities.
A male predilection for secondary
chondrosarcoma seems to be evident.
Based on combined data from the
published series, 63% of patients
with the disease are male and 37%
are female, resulting in a male:female
ratio of approximately 2:1. 4-13 The
mean age of persons with secondary
chondrosarcoma is 34 years, which
is notably younger than the average
age of persons with primary conventional
chondrosarcoma. 4-13 The most
common site of involvement is the
pelvis, followed by the proximal femur.
The scapula and proximal humerus
are also relatively common
sites. Clinical, radiographic, and genetic
features associated with secondary
chondrosarcomas are summarized
in Table 2.
Our review of the major published
series indicates that the great majority
of cases arise in osteochondromas
(88%), with solitary osteochondroma
being slightly more common
than multiple osteochondromas (ie,
hereditary multiple exostosis). 4-7,11,13
Secondary chondrosarcoma can also
occur in persons with Ollier disease
(ie, multiple enchondromas) and
Maffucci syndrome (ie, multiple enchondromas
associated with softtissue
hemangiomas). Other benign
cartilaginous lesions that have been
reported, although rarely to result in
secondary chondrosarcoma include
solitary enchondroma, synovial
chondromatosis, and chondromyxoid
fibroma. 6,15-20 These lesions have
been noted mostly in case reports,
and there may be some question
whether some cases were malignant
at the outset. 6,15-20
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