Bone tumors and their diagnosis and management

Bone tumor detailed description.

1. Bone tumor is an abnormal growth of cells within the bone that may be malignant or benign. Etiology = Unknown Inherited mutation Radiation trauma Primary bone tumor = rare accounting 0.2% Secondary bone neoplasms = more common 2

2. Classification of bone tumor Bone tissue consists of Cartilaginous tissue Osteoid Fibrous Bone marrow element Neoplasm of the bone Primary bone tumor Benign malignant Secondary bone tumor Primary bone neoplasms are classified based on Cell type Products of proliferating cells 3

3. Cell type Benign Malignant Osseous Osteoid osteoma Osteoblastoma osteoma Osteosarcoma Cartilagenous Chondroma Chondroblastoma CMF Osteochondroma chondrosarcoma Fibrous NOF Fibrous dysplasia Fibrosarcoma MFH myelogenous Eosinophilic granuloma Ewing’s sarcoma Multiple myeloma Vascular hemangioma Angiosarcoma Unknown Simple bone cyst GCT Malignant GCT Chordoma adamantinoma 4

4. Diagnosis Clinical examination Imaging Lab. Investigation Biopsy 5

5. 1.Clinical examination History Age Useful clue for diagnosis Pain Is the most frequent symptom Deep constant pain, poorly localized,worse at night Mass: painful/painless,rate of enlargment Neurologic Sxs Pathological # Previous Hx of malignancy or family history Systemic sxs- weight loss,respiratory,gastro or genitourinary disturbance 6

6. Age in yrs Benign Malignant 0-5 EG UBC Leukemia 6-18 UBC, CMF ABC, NOF CB,OB ES OS 19-40 GCT EG ES OS 40+ Mets MM, CS,Lymphoma 7

7. P/E Mass: Consistency,Tenderness,Mobility,Site ,shape,local tempreture and change with position should be noted. Regional lymph node examination Near the joint Effusion , ROM , intraarticular masses Spine : Stiffness & painful Scoliosis Atrophy of surrounding musculature/fasciculations Examine the breast, thyroid, lung and prostate Neurological deficits 8

8. 2.Imaging Plain X-ray 1.Anatomic site 2.Borders of the lesion 3. Number of lesions 4.Bone destruction 5.Matrix formation 6.Periosteal reaction 9

9. 10

10. Simple bone cyst 11

11. GCT Parosteal OS 12

12. 2.Borders of the lesion Growth rate & response of the bone Benign lesion Well defined Narrow transition Malignant lesion Poorly delineated or absent margins 13

13. Non-ossifying fibroma Tal. osteosarcoma GCT 14

14. 3. Number of lesions Is it monostoitic or polyostoitic? Examples of polyostoitic Polyostoitic FD Mets MM OS(rare) 15

15. Fibrous dysplasia Oliers dse M. myeloma F. dysplasia 16

16. 4. Bone destruction Reflects the increasing growth rate Three patterns Geographic: destructive lesion with sharply defined border(less aggresive,slow growing usually benign process EG,NOF..) Moth-eaten: destruction with ragged borders,more rapid growth &likely chance of malignancy(MM,ES,Mets..) Permeative: ill defined lesion with multiple "worm holes"wide transition(lymphoma,leukemia,ES,MM) 17

17. CMF Geographic:NOF Moth eaten Permeative: leukemia 18

18. 5.Matrix formation Matrix is internal tissue of tumor. Cartilage matrix: stippled rings and arcs Calcification Enchondroma,chondrosarcoma... Ossific matrix:solid,cloud like... New bone formation Osteosarcoma,osteoblastoma... Osteoblastic:OS 19

19. 6.Periosteal reaction Indicative of malignancy Not pathognmonic Sunburst:OS Cartilaginous:CS 20

20. Codmann’s:ES Onion-skin:ES 21

21. CT & MRI Subtle cortical disruption,calcification and ossification Intraosseous & extraosseous extension R/ship to the surrounding structure Inaccessible area like pelvis & vertebra Detecting pulm. Mets. Tumor spread Within the bone Into the nearby joint Into the soft tissue Blood vessels & the r/ship of the tumor to the perivascular space 22

22. GCT 23

23. Bone scan Polyostoitic inv’t mets intraosseous extension Arteriography Tumor blood supply & r/ship to major vessels Tumor proximity to major vessels Intraarterial chemotherapy 24

24. 3.Lab. investigation CBC, ESR Calcium,inorganic phosphate Alk. Phosphatase Serum acidic phosphatase Prostatic specific antigen Serum protein electrophoresis Bence Jones protein 25

25. Biopsy Crucial in the Dx Types Open biopsy Conventional & reliable method Closed biopsy Multiple sample can be obtained from same puncture Cannot be done in osteosclerotic tumor 26

26. Staging of bone tumor Is the process of finding out how far the cancer may have spread Enneking (1980)- surgical staging, then adopted by American joint committee for cancer in 1997 Based on three factors Histological grade(G) G-0 benign & well differentiated G-1 lesion with low grade malignancy G-2 lesion with high grade malignancy Anatomical location(T) T-0 intracapsular T-1 intracompartmental T-2 extracompartmental Presence of distant metastasis(M) M-0 no metastasis M-1 distant metastasis 27

27. Stage Grade Site Metastasis IA Low (G1) Intracompartmental(T1) None IB Low (G1) Extracompartmental(T2) None IIA High(G2) Intracompartmental(T1) None IIB High (G2) Extracompartmental(T2) None IIIA G1or G2 Intracompartmental(T1) Yes IIIB G1 or G2 Extracompartmental(T2 yes 28

28. Principles of management Multidisplinary Modalities of therapy Surgery Chemotherapy Radiotherapy 29

29. Asymptomatic benign lesions Conservative Excision/ curettage Symptomatic benign lesions Biopsy Excision/ curettage Suspected malignant lesions Admit the pt Confirm the Dx and staging Treatment Surgical Adjuvant therapy 30

30. 1.Surgical treatment Tumor excision- Intralesional, Marginal , Wide excision, Radical Intralesional excision Includes excision & curettage Passes through pseudocapsule Incomplete Macroscopic tumor remain & contaminated operative field applicable for benign lesion with low risk of recurrence 31

31. Marginal excision Entire tumor is removed in a single piece Goes beyond the tumor-plane of dissection through reactive zone Leaves mac. Lesion Suitable for benign lesion High risk of recurrence in malignant lesion up to 50% 32

32. Wide excision En block resection which includes The entire tumor The reactive zone A cuff of normal tissue Dissection – through normal tissue It can be done Stage IA lesion- risk of recurrence 10% Stage IIA lesion along with chemotherapy 33

33. Radical excision The entire compartment is removed en bloc with out exposing the lesion Plane of dissection is beyond the limiting fascial and bony borders Can be done for Stage IIB 34

34. Limb salvage surgery Developed in early 1970s Successful surgery depends on Accurate staging Accurate Dx – biopsy Good surgical technique Pre & postoperative chemotherapy 35

35. Phases of operation 1. Resection of the tumor Avoid recurrence Decide how much tissue to be removed 36

36. 2.Skeletal reconstruction The skeletal defects are reconstructed by diff. Modalities Bone graft- vascularized or non vascularized Endoprosthesis Allograft-prosthetic composite Grafting & arthrodesis 37 prosthesis

37. 38 Grafting & arthrodesis

38. 3.ST & muscle transfer To cover & close resected site To restore motor power 39

39. Contraindication to limb salvage procedure Major NV inv’t Pathologic # Inappropriate biopsy site Infection Skeletal immaturity Extensive muscle inv’t 40

40. Amputation Significant number of still require amputation Indication High grade tumors Limb salvage procedure is not good option 41

41. Chemotherapy Multidrug chemotherapy Neoadjuvant uses: facilitate limb sparing surgery by necrosis of tumor,Rx of micrometastasis, powerful prognostic factor Adjuvant Effective for sensitive tumor Osteosarcoma Ewing’s sarcoma Multiple myeloma uses of adjuvant Reduce the tumor size Prevent metastatic seeding Improves chances of survival 42

42. Radiotherapy Used to destroy radiosensitive tumors or as adjuvant before operation Ewing’s sarcoma Pmet Palliative Tumor in inaccessible area – axial 43

43. 44 Benign bone tumor

44. Osteochondroma Definition OC is a cartilage capped bony projection arising on external surface of bone containing a marrow cavity. Continuous with the underlying bone. Developmental abn. May occur as solitary or multiple. 45

45. Epidemiology Commonest benign tumor-45% Accounts 10-15% of primary bone tr Age: majority of cases occur in the first 3 decades Sex : M< F 46

46. Sites Most common: metaphyseal Distal femur Upper humerus Prox. Tibia & fibula 47

47. Clinical feature Assymptomatic- incidental Symptomatic Swelling, hard & painless Pain Nerve impingement Bursa Infarction Fracture of the stalk Deformity Limb length discrepancy Limitation of motion- juxtaarticular Neurologic manifestation- spinal lesion 48

48. Imaging Plain X-ray Pedunculated or sessile Metaphyseal Pedunculated Grows away from the epiphysis Stalk continuous with cortex 49

49. Sessile OC Flat, plateau like protuberance well demarcated outline 50

50. CT & MRI Shows continuity of marrow space Thickness of cartilaginous cap Evaluate surrounding soft tissue 51

51. Treatment Asymptomatic OC None Surgical removal symptomatic OC Indication Pain Nerve irritation Continued growth Pseudoaneurysm Malignant change Extracapsular marginal excision Include the cap,perichondrium, and deep bony base Recurrence = 5% 52

52. Malignant change suspected Adequate imaging studies Biopsy Treat the pt as conventional CS Wide excision Prognosis Risk of malignant change~ 0.2% in solitary OC, 1-3% in multiple OC Sarcomatous change = low grade CS Evidences Cartilage cap <1cm in adult, <2-3cm in children Cap diameter <8cm Soft tissue mass in CT & MRI Fluffy outline 53

53. Osteoid osteoma Definition a benign bone forming lesion called nidus, surrounded by a dense reactive zone of host bone Epidemiology Accounts 10% of benign bone tumor 2-3% of all primary bone tumor Age Children & adolescent Peak 5-25yrs (85%) Sex : M<F 54

54. Sites Located Cortex Intramedullary cavity Periosteum Any bone can be affected Tibia & femur Spine- post. element 55

55. Clinical feature Pain (80%) Commonest Worse at night Relieved by ASA Limping Swelling Scoliosis –spine inv’t Leg length discrepancy 56

56. Imaging Plain X-ray Dx can be made with radiography alone Diphysis of long bone Lytic nidus surrounded by a sclerotic margin Size of nidus – varies upto 2.5cm 57

57. Treatment Medical NSAIDs Relieves Sx Surgical En bloc excision of the nidus IntraOP localization of the nidus Percutaneous radiofrequency coagulation Best for spine CT guidance Prognosis excellent Recurrence rare 58

58. GCT A benign, locally aggressive neoplasm which is composed of neoplastic ovoid mononuclear cells interspersed with osteoclast giant cell Appears in mature bone Etiology -Unknown Epidemiology 4-5% of all primary bone tumor 20% of benign bone tumor Age Peak age 20-45 yrs Rare in immature bone Sex : F<M 59

59. Sites Ends of long bones Distal femur-24 % Prox. Tibia-24 % Distal radius-10 % Prox. Humerus-7 % Clinical feature Pain Swelling LOM Pathological # 5-10% 60

60. Imaging Plain X-ray Subarticular, eccentric,expanding zone of radiolucency Well delineated with irregular endosteal margin Trabeculated with soap bubble appearance No Periosteal rxn CXR Pul. Metastasis -1-2 % 25% die from pul. mets 61

61. Management Establish firm Dx-Aggressive nature,High potential for local recurrence Rx modalities Curettage alone Recurrence rate -50% En bloc excision Expendable bone Curettage with adjuvant therapy Eliminate microscopic dse Cryotherapy(Liquid nitrogen) Phenol 62

62. Malignant bone tumor Metastatic bone tumor It is a malignant tumor involving the bone which has originated from distant site Commonest cause of bone destruction in adults Third common site after lung and liver Epidemiology Most common malignant bone tumor Age: 40-60yrs ( 2/3rd) About 93% of mets comes from Breast,Lung,Prostate,Kidney,Thyroid Primary site may not be found in 15% 63

63. Sites Persistent red marrow vertebra, sternum, ribs, pelvis, skull, shoulder girdle Axial skeleton - 44.8% Appendicular - 28.8% Multiple bone - 26.9% Rare distal to the wrist and ankle 64

64. Clinical feature Bone pain Swelling Fracture Neurological Sx Paresthesia & weakness Bladder & bowel dysfunction Investigation ESR CBC Alk. Phosphatase Acidic phosphatase Prostatic specific antigen 65

65. Plain X-ray Osteolytic lesion (kidney,lung tumors, melanoma) Osteoblastic or can be both No periosteal rxn ST extension - uncommon 66

66. Breast Multiple and several cm in diameter Osteolytic – 80% Oseoblastic - 10% Mixed – 10% 67

67. Prostate Osteoblastic Spine & pelvis Kidney Solitary Lumbar spine & pelvis Expansile 68

68. Lung 2nd most common in men Osteolytic Small bones of the hand and feet Treatment Non operative Rx Irradiation and protected wt bearing Hormonal & chemotherapy Operative Rx Rigid fixation and full wt bearing Devices: IMN, Plates, prosthetic devices Wide resection 69

69. Osteosarcoma OS is a malignant tumor of the bone in which tumor cells form neoplastic Osteoid or bone or both Variant Conventional (IM, Classical ) Surface OS Parosteal Periosteal High grade surface OS Talengiectatic OS Small cell OS Multifocal OS 70

70. Conventional OS It is a primary intramedullary high grade malignant tumor in which the neoplastic cells produce osteoid. A disease of the young Epidemiology Most common primary malignant tumor accounting 19%. Age 10-25yrs 85% develop < 30 yrs Sex : M<F (2:1) 71

71. Sites Metaphysis 91% Long bones Distal femur,Proximal tibia,Proximal humerus Rare distal to the ankle & wrist Clinical feature Pain Deep, severe and boring Constant & worse at night Mass Hard, fixed, warm, and tender Pathological # (4%) P/E LOM,Edema,bruit 72

72. Imaging Plain film Metaphyseal Variable combination of bone destruction and formation 25% lytic 35% sclerotic 40% mixed Sun ray specules Codeman’s triangle Soft tissue extension 73

73. 74 CT & MRI Delineate intra- and extra-osseous extent of the tumor Distant metastasis

74. Management Modality of treatment surgery Adjuvant measures Chemotherapy Radiotherapy Chemotherapy 5yrs survival rate of 45% -60%. Component of OS treatment protocols Effective agent Methotrexate,Cisplatin,Doxorubucin,Vincristin Isosfamide Chemotherapy Neoadjuvant adjuvant 75

75. Surgery Limb salvage surgery Adequate tumor removal Reconstruction 85% of OS Amputation Indication Large lesion with NV inv’t Path.# with contamination Lesions in a very young Distal portion of extremity Level = staging of tumor and its extent 10 cm or one joint above 76

76. Radiation Less often used Palliative of local pain Surgically inaccessible lesion Painful met.deposits Prognosis Untreated = 95% die in 2 yrs 10% macro- and 90% micrometastsis at presentation Factors Age,Size of primary tumor,Location Type ,Stage,Response to chemo. <16 mets deposits 77

77. Ewing’s sarcoma Ewing’s sarcoma is sarcoma of the bone which arises from the medullary cavity. Within the group of small round blue cell tumor. Epidemiology 6-8% of primary malignant bone tumor Age Common in children Second decade of life 80% < 20 yrs of age Sex M<F 78

78. Sites Diaphysis/metadiaphysis Pelvis & ribs Clinical feature Pain Intermittent & gradual Mass Systemic Sx Fever, malaise, wt loss, and weakness Investigation Anemia Leukocytosis Elevated ESR 79

79. Imaging Plain X-ray Moth eaten or permeative destruction Onion skin type of periosteal rxn Large ill-defined ST mass Pathological # <5% 80

80. Management Multidrug chemotherapy Vincristine, Cyclophosphamide, Actinomycin D, Doxorubucin Radiotherapy Surgery Resection of expendable bone Limb salvage Amputation 81

81. Prognosis Favorable prognostic factors < 10yrs of age Distal extremity Tumor size <100ml Chemotherapy response <10% viable tumor Unfavorable prognostic factors Pelvis Tumor size <100ml Elevated WBC & ESR Chemotherapy response <10% viable tumor 82