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Definition Epidemiology Pathogenesis Classification Skeletal distribution Clinical presentation Evaluation Management Prognosis
Primary Malignant tumour of bone Origin : Mesenchymal cells Formation of osteoid or bone by tumour cells Site :metaphysis
2nd most common primary malignant bone tumour 20% of all primary bone malignancies Global incidence : 1-5 per million/year All age gps, bimodal distribution Slightly more common in males(1.6 :1)
70%-acquired genetic mutations Germline mutations in RB gene (1000 fold increased risk !) Li-Fraumeni syndrome (p53) p16, p14.
SITE OF ORIGIN
HISTOLOGY
PRIMARY
Commoner 15-25 age No premalignant
SECONDARY
Older age gp Premalignant lesions : Pagets disease, fibrous dysplasia,
lesion
More malignant
multiple enchondromatosis,
Prior irradiation, Multiple osteochondromas
SURFACE
PAROSTEAL
PERIOSTEAL
BASED ON HISTOLOGY: Osteoblastic Chondroblastic Fibroblastic, Telangiectatic/Osteolytic Small cell Giant cell
WORST PROGNOSIS
PAIN SWELLING(metaphysis) Pathological fractures Skin : shiny, prominent veins Warm & tender Margins : not well defined Movements of adj joint restricted Compression symptoms Regional LN REACTIVE METASTASIS : BLOOD : LUNGS
RADIOLOGICAL EXAMINATION 1.Irregular destruction,new bone fmn 2.Periosteal reaction 3.Extension to the marrow or soft tissue 4.Codmans triangle 5.Sunburst Effect
Sunburst Appearance
Follow up
Rise after initial fall :
recurrence/metastasis
Biopsy
To Confirm the diagnosis
LOCAL CONTROL
CONTROL OF MICRO & MACROMETASTASIS
AMPUTATION
PALLIATIVE
DEFINITIVE
ROLE OF CHEMOTHERAPY PRE AND POST OPERATIVE METHOTREXATE LEUCOVORIN CYCLOPHOSPHAMIDE CISPLATIN
NEW CONCEPT
PORTION OF TUMOR
IMPLANTED INTO SARCOMA SURVIVOR REMOVED AFTER 14 DAYS + SENSITISED LYMPHOCYTES
Without treatment : death in 2 yrs 5 yr survival with surgery alone : 20% 5 yr survival with surgery & CT : 70 %
WORST PROGNOSIS : TELANGIECTATIC/OSTEOLYTIC TYPE BETTER PROGNOSIS : PAROSTEAL
- AISHWARYA. G(2K9)