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11 Cards in this Set

  • Front
  • Back

Anatomy + Physiology

Outer bone surface covered by a fibrous vascular layer known as periosteum to which tendons, ligaments and fascia are attached


Vessels and connective tissue penetrate the cortex of the bone and arteries enter the medullary cavity throuhg nutrient foramina

Histology


Most common


Osteosarcoma (35%)


Chondrosarcoma (25%)


Ewing's Sarcoma (15%)



Osteosarcoma


Arises in osteoblasts


Presentation later in life associated with Paget's disease

Spread and Lymphatics

Lymph


Upper and lower extremity bones drain through nutrient foramina and traverse the periosteum, emptying into the nearest deep attaching trunk


Lymphatic spread of bone tumours is uncommon. However, there is an increased risk where tumours present in the axial skeleton



Osteosarcoma


Local spread: expands bone destroying the cortex and medulla, extending to surroundign soft tissue


Metastatic: primarily to lung (80% have mets in 1-2 yrs)



Chondrosarcoma


Loca: spread to adjacent joint and marrow cavity


Mets: occurs late in high grade tumours to lungs


Low grade tend not to met, but rather recur locally

Epidemiology and Aetiology

Primary Bone Tumours


Primary bones tumours are rare


May be malignant or benign


M>F


Casuative factors widely unknown


Prolonged growth or over stimualted metabolism, e.g. paget's disease, hyperparathyroidism and oesteomyelitis


Radiation induced malignancy


Chromosomal translocations



Osteosarcoma


10-20 yrs


M>F


Most commonly near epophyseal plates around the knee or proximal humerus


Chondrosarcoma


30-50 y.o.

Tumour Origin

Most commonly affects the extremities


Typically present in the diaphysis of extremities with distal femur + proximal tibia most common


Pelvis, spine, scapula, ribs and sternum



Chondrosarcoma


Malignant tumoru of cartilage


50% in pelvis, femur, humerus and scapula

Staging

1A/2A (T1N0M0): confined within cortex – low grade


1B/2B (T2N0M0): invades beyond cortex


3: not defined – high grade


4A: regional lymph node involvement


4B: distant metastasis



Low grade: exhibit well or moderately differentiated cells. The cell mitotic rate is slow and there is no necrosis


High grade: poorly or un differentiated cells, a high mitotic rate and necrotic tissue

Clinical Presentations

Pain


Swelling


Limp


Pathological fracture


Fever


Fatigue


Neurological symptoms if nerve compression

Detection and Diagnosis

X-ray


CT


MRI


Biopsy (MDT approach essential)


FBC


Radioisoptope bone scan


Liver function tests

Disease Management

Osteosarcoma


Required a MDT


Typically:


Neo-adjuvant multi-agent chemotherapy


Followed by conservative surgical resection of tumour


Followed by post-operative chemotherapy


Chemo:


Adriamycin


Methotrexate


Cisplatin


+/- ifosphamide


Surgery


Preservation of limb as much as possible


Involved removal of diseased bone and replacement with bone graft or artificial prosthesis


Contrainications:


Extensive soft tissue involvement


Neurological involvement


Involvement of an ankle or humeral joint



Chondrosarcoma


Radical srugery is treatment of choice


Chemo has no effect

RT


Only used for improving local control for incomplete resection of extremity tumours and vertebral and pelvic osteosarcoma


Palliation


Possible prophylactic pulmonary irradiation



Chondrosarcoma


Option for recurrent, unresectable and incompletely resected tumours



Considerations


Ensure adequate lymphatic drainage corridor over entire treatment length for limbs/extremities


Field edges at well defined boen margin in uninvolved bones


Skin dose in surgical site important consideration for wound healing



Prescription


Pre-op: 50 Gy in 25


Tumours < 10 cm: CTV = GTV + 5 cm longitudinal margin


>10 cm: GTV + 7-10 cm longitudinal margin


28-35 Gy with chemo


Post-op: 66-70 Gy in 33-35#


Ph 1: CTV + 5 cm longitudinal margin (<10 cm) or 7-10 cm (>10 cm). 45-50 Gy


Ph2: GTV + 2 cm. 66-70 Gy


Ph3: 1cm. 72-76 Gy

Prognosis

Dependant upon chemo-related necrosis


Survival improved in last 30 years due to more effective chemo agents


Extremity prognosis better


60% 3 yr survival



Chondrosarcoma


65-80% 5 yr with resection alone


Reduced to 35% for < 21 yrs