1. Bone Tumors MUHAMMAD FARRUKH BASHIR FCPS(ortho)

2. General considerations Primary bone tumors are much less than secondary tumors. All age groups affected, but some tumors occur in certain age Almost every bone can be affected, but some tumors prefer certain location Most of the tumors give osteolytic lesion in X- ray, but few are osteoblastic

3. Age of Tumors 20>…..osteoid osteoma, osteoblastoma, osteogenic Sarcoma, Ewings. 20- 40……Giant cell tumors, Secondary Osteogenic Chondrosarcoma, Lymphoma, Mets. 60……Mets, Myeloma, Chondrosarcoma, MFH, Fibrosarcoma.

5. Site or location of Tumors

6. Parosteal osteosarcoma Chondroblastoma Adamantinoma Site of the lesion.

7. Evaluation History Physical examination Investigations; labs, imaging Biopsy

8. Analytic approach to evaluation of the bone neoplasm

9. Evaluation; history Age Symptomatology – Pain – Swelling – History of trauma – Neurological sympts Pathological fracture

10. Evaluation; physical examination Lump/swelling Effusion Deformities Regional nodes

11. Evaluation; imaging Plain radiograph CT scan MRI Radionuclide scanning PET

12. Radiography Information yielded by radiography includes : – Site of the Lesion – Borders of the lesion/zone of transition – Type of bone destruction – Periosteal reaction – Matrix of the lesion – Nature and extent of soft tissue involvement

13. Radiographic Features of the Various Tumors Benign: well circumscribed, no reaction and sclerotic border. Malignant: ++++reaction, large, permeative, destructive and mouth eaten. Conditions/Mets: more than one bone, symmetry.

14. Staging of bone tumors  Benign tumors (Enneking staging of benign tumors)  Stage 1 - latent  Stage 2 - active  Stage 3 - aggressive  Malignant tumors  TNM staging  AJCC staging system  Musculoskeletal tumor society staging system(enneking) Surgical staging  Note  Benign tumors - classified using Arabic numerals(1,2,3)  Malignant tumors - classified using roman numerals(I,II,III)

15. Enneking staging of benign tumors Stage 1; Latent – Well defined margin – Grows slowly and then stops – Heals spontaneously e.g. osteoid osteoma – Neglible recurrence after intracapsular resection Stage 2; Active – Progressive growth limited by natural barriers – Well defined margin but may expand thinning cortex e.g. ABC – Negligible recurrence after marginal excision – Rx marginal resection Stage 3; aggressive – Growth not limited by natural barriers e.g. GCT – Mets present in 5% of these pts – Have high recurrence after intracapsular or marginal resection – Extended resection preferred

16. Enneking surgical Staging of malignant tumors

18. Bone biopsy  Options  Needle biopsy 90% accuracy at determining malignancy Accuracy at determining specific tumor much lower Absence of malignant cells less re-assuring than incisional biopsy  Core biopsy Provides accurate diagnosis in 90% of cases  incisional biopsy  Primary resection instead of biopsy can be done in;  Small(<3cm) subc mass- marginally resected if likely malignant  Characteristic radiographic appearance of benign lesion  Painful lesion in an expendable bone e.g prox fibula, distal ulna

19. Malignant tumor Benign tumor

20. Classification of primary bone tumors A. Bone-Forming Tumors BENIGN Osteoma: Osteoid osteoma: Osteoblastoma: Malignant: Primary osteosarcoma Secondary osteosarcoma

21. Classification of primary bone tumors B. Cartilage-Forming Tumors BENIGN : Osteochondroma Chondroma MALIGNANT : Chondrosarcoma

22. C. Miscellaneous Tumors Giant-cell tumor (usually benign) Ewing tumor (malignant) D. Tumor-like lesions Fibrous Cortical Defect (benign) Fibrous Dysplasia (benign)

23. Bone-Forming Tumors: Osteoma: Age: 40-50 ys. Site: on or inside the skull, paranasal sinuses and facial bones Exophytic growth: Round-to-oval sessile Project from subperiosteal or endosteal surfaces Usually single Usually slow- growing benign tumors Presentation: sinus obstruction, disfigurement and pressure on brain.

24. Osteoid Osteoma &Osteoblastoma Both are benign bone tumors with similar histologic features Grossly both tumors round to oval, hemorrhagic and gritty Differ in: Size Sites of origin Symptoms Behavior

25. Osteoid OsteomaOsteoblastoma Age10-20 years Sex2:1 males SiteFemoral neckSpine PainMod.-severe Worse at night, Aspirin response 90% dull aching pain Worse at night, Aspirin relief,<50% Nidusless than 2.0 cm2.0 -10.0 cm RecurrenceNo10%

26. Osteosarcoma Most common primary malignant tumor of bone Age: 10-20 years: 75% in patients below 20 years of age (primary type) 25 % old age (secondary to Paget disease) Site: Metaphyses of long bones of limbs (60% occur around the knee ) M : F ratio = 1.6 : 1

27. Major sites of Osteosarcoma

28. Risk factors Paget disease of bone Ionizing radiation Fibrous dysplasia Chronic osteomyelitis Bone infarcts Mutation of TP53 gene (retionblastoma gene).

29. Osteosarcoma Distal femoral osteosarcoma with prominent bone formation extending into the soft tissues. The periosteum, which has been lifted, has laid down a proximal triangular shell of reactive bone known as a Codman triangle (arrow).sunburst appearance

30. Clinical features Presenting symptoms: Pain Swelling Pathological fracture Marked increase in the serum alkaline phosphatase. Early hematogenous spread to the lungs, liver and brain.

32. Chondrosarcoma

34. Ewing Sarcoma may be confused with osteomyelitis Commonly affects diaphysis with onion skin appearance

35. Principles of management Multidisciplinary team approach Benign asymptomatic tumors – If certain observe – If in doubt biopsy Benign symptomatic or enlarging tumors – Biopsy – Excision/ curretage Suspected malignant tumors – If primary admit for work-up – Staging – Choices; amputation, limb sparing surgery, adjuvant therapy (chemo/radiotherapy)

36. Tumor work up(mets) History Exams: PR EXAM. BREAST EXAM Investigations: CBC+ESR.CRP.LFT,S.RFT,S.PSA. XRAYS CHEST,USG ABDOMEN.CT CHEST. BONE SCAN.

37. Benign tumors - not aggressive Bone-forming tumors – Osteoid osteoma – Bone island Cartilage lesions – Chondroma – Osteochondroma Fibrous lesions – Nonossifying fibroma – Cortical desmoid – Benign fibrous histiocytoma – Fibrous dysplasia – Osteofibrous dysplasia – Desmoplastic fibroma Cystic lesions – Unicameral bone cyst – Aneurysmal bone cyst – Intraosseous ganglion cyst – Epidermoid cyst Fatty tumors – Lipoma Vascular tumors – Hemangioma Other nonneoplastic lesions – Paget disease – Brown tumor-hyperparathyroidism – Bone infarct – Osteomyelitis

38. Aggressive benign tumors Giant cell tumor Chondroblastoma Chondromyxoid fibroma Osteoblastoma Langerhans cell histiocytosis