An Approach to Malignant Bone Tumors

1. An Approach to Malignant Bone Tumors Dr. Shweta Shendey

2. Introduction Bone tumors are classified into: Primary bone tumors Secondary bone tumors ( Metastasis) Most are classified according to the normal cell of origin and apparent pattern of differentiation. Forms 0.2% of human tumor burden. Primary malignant bone tumors make 1% of all malignant tumors. Commonest bone tumour is secondaries from other sites. Commonest primary bone tumour is multiple myeloma, second osteosarcoma.

3. Occurence

4. Bone-forming tumors (malignant) • Osteosarcoma • Central (medullary) • Peripheral (surface) • Paraosteal • Periosteal • High grade surface Cartilage-forming tumors (malignant) • Chondrosarcoma • Differentiated chondrosarcoma • Juxta-cortical chondrosarcoma • Mesenchymal chondrosarcoma • Clear cell chondrosarcoma Marrow tumors (malignant) • Ewing’s sarcoma • Neuroectodermal tumour • Malignant lymphoma of bone (Primary/secondary) • Myeloma Vascular tumors (malignant) • Angiosarcoma • Malignant haemangio pericytoma Other tumors (malignant) • Chordoma • Adamantinoma • Fibrosarcoma

5. Evaluation Age & Sex Symptomatology Pain Swelling History of trauma Neurological symptoms Restriction of movement Other constitutional sympotms (cough, chest pain, backache, loss of appetite etc) Pathological fracture Associated conditions Prior surgeries/investigations

6. Imaging Plain radiography CT scan MRI Radionuclide scanning PET

7. Plain Radiography Information yielded by radiography includes : Size 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 Multiplicity

8. Plain radiography (contd.) Size It helps us in pre treatment staging of the tumor. The larger the lesion the more likely to be aggressive or malignant.

9. Plain radiography (contd.) Site • Type of bone • Long bone / Flat bone • Intramedullary / Eccentric / Cortical lesion • The epicenter of the tumor helps to determine the origin. • Epiphysis / Metaphysis / Diaphysis

10. DIAPHYSEAL Adamantinoma Leukemia, Lymphoma, Reticulum cell sarcoma Ewing sarcoma Metastasis Osteoblastoma/ osteoidosteoma Nonossifyingfibroma . . . Location in Longitudinal Plane • METAPHYSEAL • Osteogenic sarcoma, chondrosarcoma • Osteochondroma • Brodie abscess • Nonossifyingfibroma (close to growth plate) • Solitary bone cyst, ABC, GCT • EPIPHYSEAL • Giant cell tumor • Aneurysmal bone cyst • Geode (subchondral cyst) • Infection • Eosinophilicgranuloma • Chondroblastoma • Clear cell chondrosarcoma

12. MARGIN • Margin between tumor and native bone is visible on the plain radiograph. • Slowly progressive process is “walled-off” by native bone, producing distinct margins. • Rapidly progressive process destroys bone, producing indistinct margins.

13. Radiographic Margins • Margin types 1A, 1B, 1C, 2, and 3 • least aggressive 1A, to most aggressive 3 • Aggressiveness increases likelihood of malignancy. • BUT, not all aggressive processes are malignant. • AND, not all malignant diseases are aggressive.

14. Three Patterns of Bone Destruction • Geographic Pattern • Moth-Eaten Pattern • Permeative Pattern • Result from the degree of aggressiveness of the lesion

15. Type 1a geographic lesion. (a) Diagram shows well-defined lucency with sclerotic rim. (Adapted and reprinted, with permission, from reference 1.) (b) Lateral radiograph shows intraosseous lipoma of the calcaneus, with a sclerotic rim (arrows). geographic lesion. (a) Diagram shows well-defined lucency with sclerotic rim. (Adapted and reprinted, with permission, from reference 1.) (b) Lateral radiograph shows intraosseous lipoma of the calcaneus, with a sclerotic rim (arrows). Type 1 a Geographic Lesion. Well-defined lucency with sclerotic rim. Intra osseous lipoma with a sclerotic rim .

16. Type 1 b Geographic Lesion well-defined lucent lesion without sclerotic rim. Well-defined geographic lytic focus without sclerotic rim , Endosteal scalloping seen. myeloma

17. Type 1 c Geographic Lesion ill-defined lytic lesion with indistinct margin Large ill-defined lytic lesion , Codman’s triangle Periosteal interruption, Tumor-induced new bone . . osteosarcoma

18. Margins: 1A, 1B, 1C IA: GEOGRAPHIC DESTRUCTION WELL – DEFINED WITH SCLEROSIS IN MARGIN IB: GEOGRAPHIC DESTRUCTION WELL – DEFINED BUT NO SCLEROSIS IN MARGIN IC : GEOGRAPHIC DESTRUCTION WITH ILL DEFINED MARGIN increasing aggressiveness

19. Type 2 Moth-eatenAppearance Areas of destruction with ragged borders Implies more rapid growth Probably a malignancy myeloma, metastases infection EG osteosarcoma chondrosarcoma lymphoma osteosarcoma

20. Type 3. Permeative Pattern ill-defined lesion with multiple “worm-holes” Spreads through marrow space Wide transition zone Implies aggressive malignancy Ewing EG infection myeloma, metastasis lymphoma osteosarcoma Leukemia Ewing sarcoma.

21. Less malignant Patterns of Bone Destruction Permeative Geographic • Moth-eaten More Malignant

22. Zone of Transition The zone of transition is the most reliable indicator in determining whether an osteolytic lesion is benign or malignant. The zone of transition only applies to osteolytic lesions since sclerotic lesions usually have a narrow transition zone. A small zone of transition results in a sharp, well-defined border and is a sign of slow growth. A sclerotic border especially indicates poor biological activity. An ill-defined border with a broad zone of transition is a sign of aggressive growth.

23. Narrow zone of transition Wide zone of transition

24. Types of Periosteal reaction • Benign • None • Solid/Continuous • More aggressive or malignant • Interrupted • Lamellated or onion-skinning • Sunburst • Codman’s triangle

25. Periosteal Reactions Sunburst Codman's Triangle Lamellated Solid Less malignant More malignant

26. Types of matrix: osteoblastic The matrix of a typical osteoblastic lesion is characterized by the presence of the following features : B. presence of the wisps of tumor-bone formation, like in this case of osteosarcoma of the sacrum A. fluffy, cotton-like densities within the medullary cavity, e.g in this case of osteosarcoma of the distal femur C. by the presence of a solid sclerotic mass, such as in parosteal osteosarcoma

27. Types of matrix: chondroid matrix A: Schematic representation of various appearances of chondroid matrix calcifications. B: Enchondroma displays a typical chondroid matrix C: Chondrosarcoma with characteristic chondroid matrix

28. Radiographic features that may help differentiate benign from malignant lesions

29. CT • Features are similar to that of plain radiograph, however CT scanning may be helpful locally when the radiographic appearances are confusing, particularly in areas of complex anatomy. • Very useful in early diagnosis. • Cross-sectional images provide a clearer indication of bone destruction, as well as the extent of any soft tissue mass, than the radiographs. • CT scanning may depict small amounts of mineralized osseous matrix not seen on radiographs. • The modality may be particularly helpful in visualizing flat bones, in which periosteal changes may be more difficult to appreciate. • Early detection of pulmonary secondaries • Exact measurement for limb salvage procedures (Prosthesis/allograft) • Used for prognostic follow-up of the patient.

30. MRI • Investigation of choice to assess intra-medullary extension and soft tissue involvement. • Defines the relationship to the nearby neurovascular bundles. • Ambiguous and inconspicuous cases. • Helps in staging of the tumor and to plan its surgical management. Radio-nuclide bone scan • For pre biopsy staging • Dissemination of tumour • Silent secondaries and skip lesions Arteriogram • Planning limb sparing surgery • Therapeutic embolization • To assess vascularity of tumour

31. MALIGNANT BONE TUMOURS

32. OSTEOSARCOMA MALIGNANT BONE FORMING TUMOUR PRIMARY SECONDARY • INTAOSSEOUS-TELANGIECTATIC,SMALL CELL,LOW GRADE • SURFACE- PAROSTEAL ( common surface OS ) - PERIOSTEAL • MULTICENTRIC

33. Osteosarcoma • Osteosarcoma is a primary bone-producing malignant mesenchymal tumor. • It is the most common primary malignant tumor of bone, excluding plasma cell myeloma. • Osteosarcoma represents 20% of all primary malignant bone tumors. • Osteosarcoma is encountered most commonly in the age group from 10 to 25 years (75% of cases); few cases occur before age 5 or after age 30. • The metaphyseal lesion abutting the physis is the classic location in 75% of cases.

34. PRESENTATION -PAIN -PALPABLE MASS -PATHOLOGICAL # SITE METAPHYSIS OF LONG BONES -DISTAL FEMUR -PROXIMAL TIBIA -OTHERS HUMERUS,RIBS,ILEUM,JAW BONES LUNG - Cannonball mets, subpleural nodule > cavitate > rupture >Spontaneous pneumothorax Skeletal mets – Skip lesions

35. Osteosarcoma (contd.) Radiography Conventional Radiography Permeative or ivory lesion from metaphysis medullary and cortical bone destruction wide zone of transition, permeative or moth-eaten appearance aggressive periosteal reaction sunburst type Codman triangle soft-tissue mass often growing to large dimensions tumour matrix ossification/calcification ill-defined "fluffy" or "cloud-like" cf. to the rings and arcs of chondroid lesion.

36. X-ray : The endosteal margin is poorly defined. sunburst’ effect and Codman’s triangle .

38. “Sunburst” periosteal formation • Codman’s Triangle

40. Osteosarcoma (contd.) CT • Cross-sectional images provide a clearer indication of bone destruction, as well as the extent of any soft tissue mass, than do radiographs. • CT scanning may depict small amounts of mineralized osseous matrix not seen on radiographs. The modality may be particularly helpful in visualizing flat bones, in which periosteal changes may be more difficult to appreciate

41. Central Osteosarcoma Expansile lytic destruction or radiodense slerotic homogeneous sclerosis 50% Osteolytic 25% mixed 25% Aggressive features such as cortical or medullary bone destruction

42. Multicentric Osteosarcoma • Rare form of osteosarcoma in which multiple independant lesions occur simultaneously • Do not represent metastatic foci • Occur in metaphysis of long bone • Early presentation 5-10 yrs • Course rapid, fatal • Early pulmonary mets • Early stages resemble benign bone islands, • as the disease progress fill entire medullary canal • D/d –heavy metal poisoning

44. Parosteal Osteosarcoma • Origin-surface of the bone. • swelling, mass formation and dull aching pain • Grows -surrounding soft tissues, may also infiltrate bone marrow. • M/C on the posterior side of the distal femur. • Slow growing, 30-50 yrs • Ossification in a parosteal osteosaroma - in the center than at the periphery. • D/D myositis ossificans present close to the cortical bone, but maturation develops from the periphery to the center Cleavage plane

45. Radiographic features • large lobulatedexophytic, 'cauliflower-like' • Mass with central dense ossification • string sign thin radiolucent line separating the • tumour from cortex, Cleavage plane. • +/- soft tissue mass(3-25 cm) • cortical thickening without aggressive periosteal reaction is often seen. • tumour extension into medullarycanal,frequently • Treatment • Smaller lesion – en bloc resection • Larger lesion-amputation

46. string sign

47. A well-defined and homogeneous sclerotic mass with a broad base to the underlying diaphysis of the fibula.

48. Radiograph. • Homogeneous ossified mass adjacent to the cortical bone of the distal femur

49. Periosteal osteosarcoma Broad-based surface soft-tissue mass Extrinsic erosion of thickened underlying diaphyseal cortex M/c Femur, Tibia Perpendicular periosteal reaction extending into the Soft-tissue component:   Periosteal reaction common, •  Sunburst •  Codman triangle

50. Periosteal OS Lytic in appearance, cortical erosion periosteal reaction.