The tumour is radio resistant and so radiotherapy is not used
About
- A bone tumor commonly seen in adolescents.
Aetiology
- Associated with Li-Fraumeni syndrome (germline TP53 mutation).
- Can be linked to exposure to ionizing radiation.
- Characterized by the formation of immature bone (osteoid) by the tumor.
- Typically occurs at the ends of long bones, particularly near growth plates.
Clinical Features
- Most commonly affects adolescents aged 15-18, with a slight male predominance.
- Localized pain and swelling are common but can often be misattributed to other causes, such as growing pains or trauma.
- The distal femur, proximal tibia, and humerus are the most frequently affected sites.
Investigations
- Plain X-ray: May show a characteristic "sunburst" appearance due to spiculated bone growth.
- Imaging (X-ray/CT/MRI): Shows elevation of the periosteum, which forms Codman’s triangle, a classic sign of aggressive bone tumors.
- Raised LDH: Lactate dehydrogenase (LDH) levels are often elevated and can be useful as a prognostic marker.
- Biopsy: A large core or open biopsy is needed to confirm the diagnosis and assess the tumor histologically.
- Staging: Bone scans and CT of the thorax are essential to evaluate for metastases, particularly in the lungs.
Treatment and Prognosis
- Treatment involves complete surgical resection, which may include limb amputation, although limb-salvage surgery is becoming more feasible.
- Surgery alone is often insufficient due to micrometastases; therefore, chemotherapy is typically administered both pre- and post-operatively.
- The prognosis is worse if the tumor involves the axial skeleton (e.g., spine, skull), with an overall survival rate of around 50% with treatment.