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CHONDROSARCOMA

Introduction:
Chondrosarcoma is one of the commonest malignant, slow-growing,
cartilaginous tumor originating in bone
usually present for many months before being discovered.

Chondrosarcomas take various forms, usually designated according


to:
(a) Their location in the bone (central or peripheral);
(b) Whether they develop without precedent (primary
chondrosarcoma) or by malignant
change in a pre-existing benign lesion (secondary
chondrosarcoma); and
(c) The predominant cell type in the tumour.

Incidence:
 The highest incidence is in the fourth and fifth decades and
 Men are affected more often than women.
C/F
Patients may complain of a dull ache or a gradually enlarging lump.
Medullary lesions may present as a pathological fracture.

Site:
almost 50 % appear in the metaphysis of the long bones, mostly in the lower
limbs.
The next most common sites are the pelvis and the ribs.
Rarely in the bones of the hands and feet

By far the majority of chondrosarcomas fall into two well-defined categories:


1. central tumours occupying the medullary cavity of the bone, and
2. peripheral tumours’ growing out from the cortex.
Less common varieties are
3. juxtacortical chondrosarcoma,
4. clear-cell chondrosarcoma and
5. mesenchymal chondrosarcoma.

Central chondrosarcoma The tumour develops in the medullary cavity of


either tubular or flat bones, most commonly at the proximal end of the femur
or in the innominate bone of the pelvis.
X-rays show an expanded, somewhat radiolucent area in the bone, with flecks
of increased density due to calcification within the tumour.
Aggressive lesions may take on a globular appearance with scalloping or
destruction of the cortex.
When a benign medullary chondroma (enchondroma) undergoes malignant
transformation, it is difficult to be sure that the lesion was not a slowly
evolving sarcoma from the outset.
Peripheral chondrosarcoma This tumour usually arises in the cartilage cap of
an exostosis (osteochondroma) that has been present since childhood.
Exostoses of the pelvis and scapula seem to be more susceptible than others
to malignant change, but perhaps this is simply because the site allows a
tumour to grow without being detected and removed at an early stage. Xrays
show the bony exostosis, often surmounted by clouds of patchy calcification
in the otherwise unseen lobulated cartilage cap. A tumour that is very large
and calcification that is very fluffy and poorly outlined are suspicious
features, but the clearest sign of malignant change is a demonstrable
progressive enlargement of an osteochondroma after the end of normal bone
growth. MRI is the best means of showing the size and internal features of the
cartilage cap.

Juxtacortical (periosteal) chondrosarcoma Here the lesion appears as an


excrescence on the surface of one of the tubular bones – usually the femur. It
arises from the outermost layers of the cortex, deep to the periosteum. X-ray
changes comprise features of both a chondrosarcoma and a periosteal
osteosarcoma: an outgrowth from the bone surface, often containing flecks of
calcification, as well as ‘sunray’ streaks and new-bone formation at the
margins of the stripped periosteum. The dominant cell type is chondroblastic
but there may also be sparse osteoid formation, leading one to doubt
whether this is a cartilage tumour or a non-aggressive osteosarcoma.

Clear-cell chondrosarcoma There is some doubt as to whether this rare


tumour is really a chondrosarcoma. In some respects the tumour resembles
an aggressive chondroblastoma (e.g. its typical location in the head of the
femur rather than the metaphysis). However, despite the fact that it is very
slow-growing, it does eventually metastasize. Mesenchymal chondrosarcoma
This is an equally controversial entity. It tends to occur in younger individuals
and in about 50 per cent of cases the tumour lies in the soft tissues outside
an adjacent bone. The x-ray appearances are similar to those of the common
types of chondrosarcoma but the clinical behaviour of the tumour is usually
more aggressive. Histology shows a mixture of mesenchymal cells and
chondroid tissue.

Staging
If a chondrosarcoma is suspected, full staging procedures should be
employed. CT scans and MRI must be carried out before performing a biopsy.

Pathology
A biopsy is essential to confirm the diagnosis. However, low-grade
chondrosarcoma may show histological features no different from those of an
aggressive benign cartilaginous lesion. High-grade tumours are more cellular,
and there may be obvious abnormal features of the cells, such as plumpness,
hyperchromasia and mitoses.

Treatment
Since most chondrosarcomas are slow-growing and
metastasize late, they present the ideal case for wide
excision and prosthetic replacement, provided it is
certain that the lesion can be completely removed
without exposing the tumour and without causing an
unacceptable loss of function; in that case amputation
may be preferable. In some cases isolated pulmonary
metastases can be resected. The tumour does not
respond to either radiotherapy or chemotherapy.
Prognosis is determined largely by the cellular
grade and the resection margin. There is a tendency
for these tumours to recur late and the patient should
therefore be followed up for 10 years or longer.

A chondrosarcoma may occur in the area of a treated “enchondroma.” In this


circumstance, the original pathology specimen should be reviewed.

The pattern of calcification has been described as “punctate,” “popcorn,” or


“comma-shaped.” Compared with enchondroma, however, chondrosarcoma
has a more aggressive appearance with bone destruction, cortical erosions,
periosteal reaction, and rarely a soft-tissue mass.

If the cartilaginous cap is larger than 2 cm in a skeletally mature patient, a


secondary chondrosarcoma must be considered.