OSTEOSARCOMA

In its classic (intramedullary) form, osteosarcoma is a

highly malignant tumour arising within the bone and
spreading rapidly outwards to the periosteum and surrounding
soft tissues. It is said to occur predominantly
in children and adolescents, but epidemiological studies
suggest that between 1972 and 1981 the age of
presentation rose significantly (Stark et al., 1990). It
may affect any bone but most commonly involves the
long-bone metaphyses, especially around the knee and
at the proximal end of the humerus.
Pain is usually the first symptom; it is constant, worse
at night and gradually increases in severity. Sometimes
the patient presents with a lump. Pathological fracture
is rare. On examination there may be little to find
except local tenderness. In later cases there is a palp able
mass and the overlying tissues may appear swollen and
inflamed. The ESR is usually raised and there may be
an increase in serum alkaline phosphatase.
X-rays
The x-ray appearances are variable: hazy osteolytic
areas may alternate with unusually dense osteoblastic
areas. The endosteal margin is poorly defined. Often
the cortex is breached and the tumour extends intothe adjacent tissues; when this happens, streaks of
new
bone appear, radiating outwards from the cortex the
so-called sunburst effect. Where the tumour
emerges from the cortex, reactive new bone forms at
the angles of periosteal elevation (Codmans triangle).
While both the sunburst appearance and Codmans
triangle are typical of osteosarcoma, they may occasionally
be seen in other rapidly growing tumours.
Diagnosis and staging
In most cases the diagnosis can be made with confidence
on the x-ray appearances. However, atypical
lesions can cause confusion. Conditions to be
excluded are post-traumatic swellings, infection, stress
fracture and the more aggressive cystic lesions.
Other imaging studies are essential for staging purposes.
Radioisotope scans may show up skip lesions,
but a negative scan does not exclude them. CT and
MRI reliably show the extent of the tumour. Chest xrays
are done routinely, but pulmonary CT is a much
more sensitive detector of lung metastases. About 10
per cent of patients have pulmonary metastases by the
time they are first seen.
A biopsy should always be carried out before commencing
treatment; it must be carefully planned to
allow for complete removal of the tract when the
tumour is excised.
Pathology
The tumour is usually situated in the metaphysis of a
long bone, where it destroys and replaces normal boneAreas of bone loss and cavitation alternate with
dense
patches of abnormal new bone. The tumour extends
within the medulla and across the physeal plate. There
may be obvious spread into the soft tissues with ossification
at the periosteal margins and streaks of new
bone extending into the extraosseous mass.
The histological appearances show considerable
variation: some areas may have the characteristic spindle
cells with a pink-staining osteoid matrix; others
may contain cartilage cells or fibroblastic tissue with
little or no osteoid. Several samples may have to be
examined; pathologists are reluctant to commit themselves
to the diagnosis unless they see evidence of
osteoid formation.
Treatment
The appalling prognosis that formerly attended this
tumour has markedly improved, partly as a result of
better diagnostic and staging procedures, and possibly
because the average age of the patients has increased,
but mainly because of advances in chemotherapy to
control metastatic spread. However, it is still important
to eradicate the primary lesion completely; the
mortality rate after local recurrence is far worse than
following effective ablation at the first encounter.
The principles of treatment are outlined on page
192. After clinical assessment and advanced imaging,
the patient is admitted to a special centre for biopsy.
The lesion will probably be graded IIA or IIB. Multiagent
neoadjuvant chemotherapy is given for 812
weeks and then, provided the tumour is resectable
and there are no skip lesions, a wide resection is carried out. Depending on the site of the tumour,
preparations
would have been made to replace that segment
of bone with either a large bone graft or a custommade
implant; in some cases an amputation may be
more appropriate.
The pathological specimen is examined to assess the
response to preoperative chemotherapy. If tumour
necrosis is marked (more than 90 per cent),
chemotherapy is continued for another 612 months;
if the response is poor, a different chemotherapeutic
regime is substituted.
Pulmonary metastases, especially if they are small
and peripherally situated, may be completely resected
with a wedge of lung tissue.
Outcome
Long-term survival after wide resection and
chemotherapy has improved from around 50 per cent
in 1980 (Rosen et al., 1982; Carter et al., 1991) to
over 60 per cent in recent years (Smeland et al.,
2004). Tumour-replacement implants usually function
well. There is a fairly high complication rate
(mainly wound breakdown and infection) but, in
patients who survive, 10-year survival with mechanical
failure as the end point is 75 per cent and for failure
for any cause is 58 per cent. The limb salvage rate
at 20 years is 84 per cent (Jeys et al., 2008) Aseptic
loosening is more prevalent in younger patients.