Osteosarcoma/ Ewing's

Question 1

Definition of osteosarcoma

Answer 1

Osteosarcoma incidence > Ewing’s sarcoma incidence (but Ewing’s is seen more often in younger children)

Most common primary bone malignancy of childhood / most common bone sarcoma

Occurs at the end of long bones (60-75% in knee)

M > F; 10-30yo (75% <20yo)

Question 2

Clinical features of osteosarcoma

Answer 2

Limb is the most common site

Unexplained persistent localised bone pain

mass/swelling

restricted movement

Rapid metastasis to lung

Question 3

Investigations of osteosarcoma

Answer 3

XR (bone destruction and formation) - esp if unexplained persistent localised bone pain
* shows destruction and variable periosteal new bone formation
* Soft tissue calcification = sunburst appearance
* Elevated periosteum = “Codman’s triangle”

Biopsy
* Malignant mesenchymal cells +/- new bone and cartilage formation

CT/PET/MRI

Chest CT scan- used to assess for lung metastases

Bone marrow sampling- excludes bone marrow involvement

Question 4

Management of osteosarcoma

Answer 4

Make sure pt has adequate pain relief if in pain - NSAIDs, opioids may be needed

Specialised sarcoma team (London) management

Surgery (limb-sparing surgery ± amputation) + chemotherapy

Post-treatment -> OT, PT, dietician, orthotics/prosthetics, support (sarcoma UK)

Counsel the child and parents/ carers about diagnosis and treatment, reconstructive surgery or prosthetic fitting

Question 5

Complications/ Prognosis of osteosarcoma

Answer 5

60% 5-year survival

REMEMBER: functional pain is a diagnosis of exclusion and children don’t get frozen shoulder so any patient with severe symp following a minor injury, bone tumours should be considered

Question 6

Ewing’s sarcoma

Answer 6

Primitive Neuroendocrine Tumour (PNET) / malignant, small round blue-cell tumour
* <25y, median 15y
* Long bones of arms, legs, chest, skull and trunk
* Associated t (11:22)(EWSR1/FLI1) (q24; q12)

 
Signs & symptoms:
* Mass or swelling and bone pain
* Malaise, fever, paralysis (may precipitate osteomyelitis)

Investigations:
* XR (bone destruction with overlying onion-skin layers of periosteal bone formation)
* Biopsy (small round blue cells)
* CT/PET/MRI

Management:
* Specialised sarcoma team (London) management
* Surgery (limb-sparing surgery ± amputation) + chemotherapy (VIDE) + radiotherapy
* Post-treatment  OT, PT, dietician, orthotics/prosthetics, support

Prognosis -> survival 5-year at 75% (20-40% for metastasis)