Osteosarcoma

Osteosarcoma is the commonest primary malignant bone tumour. It is charecterised by the malignant tumour cells which directly produce the osteoid (nonmineralised bone matrix). It occurs in two forms ; Primary (occur de novo) - the commonest
form

Secondary (6-10%) - secondary to pre-existing

eg. Paget's disease Encondromatosis

bone pathology

Osteochondromatosis ? chronic osteomyelitis Fibrous dysplasia Past history of bone irradiation

Age :

Usually young patients below 20 ; Very rarely occurs among older patients (except in the case of malignant transformation of Paget's disease of the bone) Male > female

Sex :

Sites : Usually start in the medullary cavity at metaphysis of a long bone Commonest sites include ; - lower end of femur / upper end of tibia (knee area 60%) - upper end of femur (15%) - upper end of humerus (10%) Pathogenesis : Unknown. May be multifactorial...genetic, constitutional & enviromental factors

Macroscopic appearence :

A typical gross appearence of osteosarcoma. The tumour is solid and very vascular. Usually start in the medullary cavity of the metaphysis and than grows aggressively, penetrates the cortex and elevates the periosteum. Finally the tumour penetrates the periosteum and extends into the surrounding soft tissue. Extension into the joint cavity is very rare. Areas of haemorrhage and necrosis are common. Pathological fracture might be seen. Superficial osteosarcoma known as juxtacortical and subperiosteal osteosarcoma are very rare.

Microscopic appearence Sheets of very pleomorphic malignant tumour cells which directly produce osteoid (1.). Atypical mitotic figure is also noted (arrow).
Base on histology, osteosarcoma is subclassify into four types; - osteoblastic osteosarcoma (osteogenic osteosarcoma) - chondroblastic osteosarcoma (when the tumour also produces a lot of condroid matrix) - fibroblastic osteosarcoma (when the tumour is so fibroblastic) - telangiectatic osteosarcoma (when it is so vascular more than the usual type - this particular variant is always more aggressive and carries poorer prognosis)

Tumour spread Direct spread - to the surrounding soft tissue (however direct penetration through skin and giving rise to
skin ulcer is very unusual for osteosarcoma) and along the medullary cavity of a long bone. Direct penetration into the joint cavity is very rare.

Haematogenous spread Lymphatic spread

- to distant sites eg. lungs and other organs is common and occur early.

- to regional lymph node is almost never occur. This probably due to the poor lymphatic supply to the bone, and most important because the tumour is so vascular therefore haematogenous spread is more common.

Important investigations 1. Plain X-ray

A. Bone forming tumour - newly formed bone spicules producing a'sunburst' pattern B. A well-defined Codman's triangle-as a result of tumour expansion which lifted up the periosteum from the cortex C. Non-calcified tumour (purely lytic) CXR and skeletal survey are important to look for metastases (especially when CT scan is not available)

2. CT-scan - this is important to asses the tumour extension 3. Tissue biopsy - fine needle aspiration biopsy / open biopsy Prognosis Generally the prognosis is not so good. Except the superficial variant. 5 years survival rate : Surgery alone = 20% Surgery + radiotherapy + chemotherapy = 60%
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