1. Malignant bone tumour By
Dr. Raad J. AL.shaibany

2. OSTEOSARCOMA Types: classic (intramedullary) form
PAROSTEAL OSTEOSARCOMA
PERIOSTEAL OSTEOSARCOMA
PAGET’S SARCOMA
Talengectatic type
Secondary osteosarcomas
Highly malignant tumour

3. Clinical features: predominantly in children and adolescents,.
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.
Pathological fracture is rare.
local tenderness.
In later cases there is a palpable mass and the overlying tissues may appear swollen and inflamed
The ESR is usually raised
Increase in serum alkaline phosphatase.

4. 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 into the 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 (Codman’s triangle).While both the sunburst appearance and Codman’s triangle are typical of osteosarcoma, they may occasionally be seen in other rapidly growing tumours.

5. Diagnosis and staging:
In most cases the diagnosis can be made with confidence on the x-ray appearances. Conditions to be excluded are
post-traumatic swellings,
Infection,
Stress fracture
Aggressive ‘cystic’ lesions .
Other imaging studies are essential for staging purposes like
1.Radioisotope scans may show up skip lesions,but a negative scan does not exclude them.
2. CT andMRI reliably show the extent of the tumour.
3. Chest xrays , but pulmonary CT is a much more sensitive detector of lung metastases. About 10 per cent of patients have pulmonary metastases.

6. Treatment:
it is still important to eradicate the primary lesion completely.
Chemotherapy befor and after surgery.
Amputation or wide resection.
Pulmonary metastases,

7. Variant of osteosarcoma
1.paraosteal-osteosarcoma 2.periosteal-osteosarcoma 3.paget osteosarcoma

8. Osteosarcoma

9. Paraosteal osteosarcoma

10. Osteosarcoma Staging

11. Osteosarcoma treatment

12. Chondrosarcoma
Types: (a) central or peripheral (b) primary chondrosarcoma or secondary chondrosarcoma (c) the predominant cell type in the tumour.

13. Clinical features
The highest incidence is in the fourth and fifth decades and men are affected more
Dull ache
Gradually enlarging lump.
Pathological fracture.
50 per cent appear in the metaphysis of one of the long tubular bones mostly in the lower limbs(proximal end of the femur). The next most common sites are the pelvis and the ribs.

14. X-ray
1. Expanded, somewhat radiolucent area in the bone, with flecks of increased density due to calcification within the tumour with or without destruction of the cortex.(central tumour) 2.Bony exostosis, often surmounted by clouds of patchy calcification in the otherwise unseen lobulated cartilage cap.(peripheral tumour on top of exostosis).

15. Treatment
Since most chondrosarcomas are slow-growing and metastasize late, they present the ideal case for wide excision and prosthetic replacement.
The tumour does not respond to either radiotherapy or chemotherapy

16. Chondrosarcoma

17. Fibrosarcoma
Fibrosarcoma is rare in bone; it is more likely to arise in previously abnormal tissue (a bone infarct, fibrous dysplasia or after irradiation).
Clinical features: The patient usually an adult complains of pain or swelling; there may be a pathological fracture.

18. X-ray :
An undistinctive area of bone destruction. CT or MRI will reveal the soft-tissue extension.
Treatment:
Low-grade, well-confined tumours (stageIA) can be treated by wide excision with prosthetic replacement.
High-grade lesions (IIA or IIB) require radical resection or amputation; if this cannot be achieved, local excision must be combined with radiation therapy.

19. Fibrosarcoma

20. Ewing sarcoma
Ewing’s sarcoma is believed to arise from endothelial cells in the bone marrow.
Clinical features:
Common ages of 10 and 20 years,
The tibia, fibula or Clavicle but it may affect pelvis.
The patient presents with pain – throbbing. Generalized illness, swelling.pyrexia, with a warm, tender swelling and a raised ESR, (may suggest a diagnosis of osteomyelitis).

21. X-ray An area of bone destruction predominantly in the mid-diaphysis
Onion-peel’ effect
Sunray’ appearance and Codman’s triangles
CT and MRI reveal the large extraosseous component.
Radioisotope scans may show multiple areas of activity in the skeleton

22. Differential Diagnosis
1.Bone infection – biopsy 2.Osteosarcoma 3.Reticulum-cell sarcoma 4.Metastatic neuroblastoma.

23. Treatment
The best results are achieved by acombination of all three methods: a course of preoperative neoadjuvant chemotherapy; then wide excision if the tumour is in a favourable site, or radiotherapy followed by local excision if it is less accessible; and then a further course of chemotherapy for 1 year.
Postoperative radiotherapy may be added if the resected specimen is found not to have a sufficiently wide margin of normal tissue.

24. Ewing sarcoma

25. NON-HODGKIN’S LYMPHOMA
Tumour of the reticuloendothelial system. It is usually seen in sites with abundant red marrow: the flat bones, the spine and the long-bone metaphyses.
Clinical features:
The patient,usually an adult of 30–40 years, presents with pain or a pathological fracture. 

26. X-ray:
A mottled area of bone destruction in areas that normally contain red marrow. Radioisotope scan may reveal multiple lesions.
Treatment :
The preferred treatment is by chemotherapy and radical resection;
Radiotherapy is reserved for less accessible lesions

27. NON-HODGKIN’S LYMPHOMA

28. MULTIPLE MYELOMA
Multiple myeloma is a malignant B-cell lymphoproliferative disorder of the marrow, with plasma cells predominating.
It may be presente as a solitary tumour (plasmacytoma) in one of the bones or as multiple lesions (multiple myeloma)

29. Clinical features
Typically aged 45–65, presents with weakness, backache, bone pain or a pathological fracture.
Features of anaemia.
Features of hypercalcaemia such as thirst, polyuria and abdominal pain
Features of renal dysfunction, chronic nephritis and recurrent infection.
Bone resorption leads pathological fractures.
Features of spinal cord or root compression caused by vertebral collapse

30. X-rays
Generalized osteoporos(myeloma is one of the commonest causes of osteoporosis and vertebral compression fracture in men over the age of 45 years.
Multiple punched-out defects with‘soft’margins (lack of new bone) in the skull, pelvis and proximal femur,
A crushed vertebra,
A solitary lytic tumour in a large-bone metaphysis.

31. Investigations Mild anaemia is common. High ESR.
Blood chemistry may show a raised creatinine level and hypercalcaemia.
Bence Jones protein in the urine in 50% of patients.
Bone marrow aspirate positive in 75% of patients and bone biopsy give 100% positive results.

32. Differential Diagnosis
1.Other causes of osteoporosis
2.Metastatic bone disease.(lytic lesions)
3.Paraproteinaemia is a feature of other (benign) gammopathies; it is wise to seek the help of a haematologist before reaching a clinical diagnosis.

33. Treatment General treatment.
Specific treatment: is with alkylating cytotoxic agents(e.g. melphalan).

34. General treatment pain control by analgesia or steroid
Treatment of pathological fractures.
Correction of fluid balance and (in some cases) hypercalcaemia.
Limb fractures treatment.
Spinal fractures carry the risk of cord compression and need immediate stabilization either by effective bracing or by internal fixation. Unrelieved cord pressure may need decompression.either by surgery or radiotherapy.

35. Multiple myeloma

36. METASTATIC BONE DISEASE
In patients over 50 years bone metastases are seen more frequently than all primary malignant bone tumours together.
The commonest source is carcinoma of the breast; next in frequency are carcinomas of the prostate, kidney, lung, thyroid, bladder and gastrointestinal tract. In about 10 per cent of cases no primary tumour is found.
The commonest sites for bone metastases are the vertebrae, pelvis, the proximal half of the femur and the humerus.

37. Spread is usually via the blood stream; occasionally, visceral tumours spread directly to adjacent bones (e.g. the pelvis or ribs).
Resorption is due either to the direct action of tumour cells or to tumour-derived factors that stimulate osteoclastic activity.
Osteoblastic lesions are uncommon; they usually occur in prostatic carcinoma.

38. Clinical features The patient is usually aged 50–70 years.
Pain is the commonest: sudden appearance of backache or thigh pain in an elderly person
Pathological fracture, sudden collapse of a vertebral body or a fracture of the mid-shaft .
Symptoms of hypercalcaemia( anorexia, nausea, thirst, polyuria, abdominal pain, general weakness and depression).
Under 6 years of age, metastatic lesions are most commonly from adrenal neuroblastoma. The child presents with bone pain and fever.

39. Imaging
1.X-rays
Most skeletal deposits are osteolytic areas in the medulla or produce a(moth-eaten appearance in the cortex).
Pathological fracture.
Osteoblastic deposits suggest a prostatic carcinoma.
Generalised osteoporosis.
2. Bone scan : are the most sensitive method of detecting ‘silent’ metastatic deposits in bone.

40. Investigation The ESR may be increased
Haemoglobin concentrationis usually low.
The serum alkaline phosphatase concentration is often increased.
The acid phosphatase also is elevated in prostatic carcinoma.
Patients with breast cancer can be screened by measuring blood levels of tumour-associated antigen markers.

41. Prognosis
In assessing of survivorship at 1 year we depend on (Bauer’s criteria)
A solitary metastasis
No pathological fracture
No visceral metastases
Renal or breast primary
No lung cancer

42. Prognosis
Of patients with 4 or 5 of Bauer’s criteria 50 per cent were alive
Of patients with 2 or 3 criteria 25 per cent were alive
Of patients with only 1 or none of the criteria, the majority survived for less than 6 months and none were alive at 1 year.

43. Treatment
1. Control of pain and metastatic activity : Most patients require analgesics, but the more powerful narcotics should be reserved for the terminally ill.
..Radiotherapy may be used both to control pain and to reduce metastatic growth.
2.Secondary deposits from breast or prostate can often be controlled by hormone therapy(stilboestrolfor prostatic secondaries and androgenic drugs or oestrogens for breast carcinoma).

44. 3.Disseminated secondaries from breast carcinoma are sometimes treated By oophorectomy combined with adrenalectomy or hypophyseal ablation.
4.Hypercalcaemia should be treated by ensuring adequate hydration, reducing the calcium intake and, if necessary, administering bisphosphonates.
5.Treatment of limb fractures : shaft fractures should almost always be treated by internal fixation + methylmethacrylate cement. Fractures near joints may need fixation with plates or sometimes joint replacement .

45. 6.Prophylactic fixation
Mirels scoring system

46. 7.Treatment of metastatic spinal disease: Metastatic spinal disease is 40 times more common than all primary tumours of the spine together mostly in the thoracic spine
and mainly in the vertebral body.
The aims of intervention are to decrease pain, preserve the ability to walk, maintain urinary and faecal continence and prolong survival.
Pathological fractures usually require some form of support.either by brace or by surgical fixation .

47. 8.If there are overt symptoms and signs of cord compression, treatment is urgent to decompress the cord by surgical removal of tumour .
9.radiotherapy alone is reserved for patients with softtissue compression and as palliation for inoperable cases.

48. METASTATIC BONE DISEASE

49. Thank you