Clinical

1. PRINCIPLES OF
MANAGEMENT OF
MALIGNANT BONE
DISEASES
Dr Arojuraye S.A
National Orthopaedic Hospital
Dala – Kano
3rd February, 2017

2. Outline
 Introduction
 Principles of management
 Resuscitation
 Diagnosis
 Staging
 Counselling
 Treatment
 Follow-up
 Prognosis
 Conclusion
 References

3. Introduction
 Most common MBT are 20
 Vertebrae, Pelvis
 Proximal humerus
 Proximal femur
 Unusual: distal to elbow & knee
 Spread
 Usually via Blood stream
 Occasionally by direct invasion
 Very rarely via lymphatics
 Lesions: Osteolytic / Osteoblastic

4. Introduction…

5. Introduction…
 Common source bone met.
 Breast
 Prostate
 Lung
 Renal
 Thyroid
 Metastasis are rare in children
 Neuroblastoma
 Rhabdomyosarcoma
 Clear cell ca of the kidney

6. Introduction…
 10 MBT are very rare
 0.2% of all malignancies
 5% of all malignancies in children
 Most common 10 MBT
 Multiple myeloma
 Osteosarcoma
 Chondrosarcoma
 Ewing’s sarcoma

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

8. Management
(Multidisciplinary/Multiprofessional)
 Orthopaedic surgeon
 Radiologist
 Pathologist
 Oncologist
 Physiotherapist
 Occupational therapist
 Prosthetist
 Rehabilitation nurses
Consultation & Cooperation

9. Diagnosis
(History)
 Age
 Ewing sarcoma (<10yrs)
 Osteosarcoma (2nd & 4th decades)
 Chondrosarcoma & fibrosarcoma (4th decade)
 Multiple myeloma (6th decade)
In patients over 70 years of age, metastatic bone
lesions are more common than primary
tumours.

10. Diagnosis…
(History)
 Sex
 M > F (osteosarcoma)
 F > M (GCT)
 Race
 Ewing sarcoma is rare in African descent
 Family hx
 R/o multiple hereditary exostosis & NF

11. Diagnosis…
(History)
 Pain
 Common, at rest & at night
 May be due to expansion
 Central haemorrhage
 Tumour degeneration
 Incipient pathological fracture
MBT may remain silent if they are
slow-growing & situated where there is room for
inconspicuous expansion (e.g. pelvis)

12. Diagnosis…
(History)
 Swelling (Size & progression)
 Anorexia, weight loss, fever
 Neurological (paraesthesia or numbness)
 Fracture (trivial injury)
 Hx of trauma (frequent)
whether the injury initiates a pathological change or
merely draws attention to what is already there
remains unanswered!!!

13. Diagnosis
(Examination)
 General
 Anaemia, dehydration
 Wasting, Jaundice
 LN metastasis
 Rare in most sarcoma
 Synovial sarcoma
 Rhabdomyosarcoma
 Epithelioid sarcoma

14. Diagnosis…
(Examination)
 Swelling
 Size
 Location, shape
 Consistency, mobility
 Tenderness, temp.
 Neurological deficit
 Joint stiffness

15. Diagnosis…
(Examination)
 Chest
 Abdomen
 Rectum
 Spine
 Spine lesions →
 Muscle spasm
 Back stiffness
 Painful scoliosis

16. Differential Diagnosis
 Hypertrophic nonunion
 Clotted haematoma
 Chronic OM
 Bone infarct
 Stress fracture
 Myositis ossificans

17. Investigations
(Plain Radiographs)
 Most useful investigation
 Abnormalities:
 Cortical thickening
 Discrete lump, Cyst
 Ill-defined destruction
 Location:
 Metaphysis or diaphysis
 Margins:
 Well-defined or ill-defined

18. Investigations
(Plain Radiographs)
 Medullary & cortical destruction
 Periosteal reaction
 Sunburst
 Codman triangle
 Soft-tissue mass
Osteosarcoma

19. Investigations…
(Plain Radiographs)
 Medullary destruction
 Calcifications
 Annular, punctate
 Popcorn or comma
 Cortical thickening
 Endosteal scalloping
Chondrosarcoma

20. Investigations…
(Plain Radiographs)
 Destructive lesion in
the diaphysis
 “Onion skin”
periosteal reaction
Ewing sarcoma

21. Investigations…
(Plain Radiographs)
 Multiple punched-out
 Sharply demarcated
 Purely lytic lesions
 No reactive sclerosis
 Biconcave vertebral bodies
Multiple myeloma

22. Investigations…
(Plain Radiographs)
 Multiple, sharply
demarcated radiolucent
lesions in the tibial
diaphysis separated by
areas of dense, sclerotic
bone
Adamantinoma

23. Investigations…
(Plain radiographs)
 Purely lytic lesions with
indistinct borders
 Absence periosteal reaction
unless with a pathological #
Malignant fibrous
histiocytoma &
Fibrosarcoma

24. Investigations…
(Plain radiographs)
 Ill-defined area of bone
destruction (diaphyseal)
with permeative
appearance
 Cortex may be thickened
but periosteal reaction is
rarely seen
Lymphoma

25. Investigations…
(Plain radiographs)
 Destructive lesions
 Calcifications
Chordoma

26. Investigations…
(Plain radiographs)
 Destructive lesions
 Geographic
 Moth-eaten
 Permeative
 Indistinct borders
 Little soft tissue lesion
 No periosteal reaction
 Blastic or lytic
Bone metastasis

27. Investigations…
(Radionuclide scanning)
 Site of small tumour
 Silent 2° deposit
 Skip lesions

28. Investigations…
(CT Scan)
 Extends range of x-ray
 Lesion in inaccessible
sites (spine, pelvis)
 Reliable in pulmonary
metastasis

29. Investigations…
(MRI)
 Relation to soft tissues
 Tumour staging

30. Investigations…
(Arteriogram)
 Planning limb salvage
 Therapeutic embolization
 Assess vascularity of tumour

31. Investigations…
(Laboratory)
 FBC
 ESR
 Serum ALP
 Serum protein electrophoresis
 Urine Bence Jones protein
 PSA
 Antisarcoma antibodies

32. Investigations…
(Biopsy)
 Biopsy may alter the CT & MRI features
 Delay the procedure until after all imaging
 Important to take a representative sample
 Frozen section may be needed
 Suspected infection
 Sample for microbiology.

33. Investigations…
(Biopsy)
 Needle (Jamshidi)
 USS guided
 CT guided
It’s essential that the biopsy is carried out in the line of
any further surgical incision so that the tract can be
excised at the time of definitive surgery

34. Investigations…
(Biopsy)
 Open biopsy
 NOT a minor procedure!
 Significant morbidity
 Reliable in obtaining a representative sample
 Indicated if needle biopsy is dangerous
 Or diagnosis not made with needle

35. Investigations…
(Biopsy)
 Open biopsy…
 Selected site ↔ definitive surgery
 Longitudinal incision
 Minimal tumour exposure
 Block of tissue is removed (boundary zone)
 Deflate tourniquet & secure haemostasis
 Drains should be avoided

36. Investigations…
(Biopsy)
 Open biopsy…
 Biopsy should be round or
oval (↓stress concentration)
 Keep defects < 10% of bone
diameter
 Torsional strength ↓ to 50%
with defect > 20% of bone
diameter

37. Investigations…
(Biopsy)
 Intramuscular dissection
 Specimens → fresh, unfixed & uncrushed
 Experienced pathologist
 Histologic diagnosis
 Immunohistochemistry
 Cytogenetic analysis
 Request form
 Site of the tumour
 The patient’s age
 Radiological differential diagnosis

38. Investigations…
(Poorly performed biopsy)

39. Staging…
 Enneking`s Staging
 AJCC System
 TNM Staging
The Enneking staging system has been shown to
prognosticate survival for bone sarcomas

40. Staging
(Enneking)

41. Staging…
(AJCC)

42. Staging…
(TNM)
Union International Cancer Centre Geneva
Staging System
 Primary Tumour – T0 to T4
 Nodal involvement – N0 to N3
 Metastasis – M0 to M2

43. Informed Consent
 Natural hx of the disease
 The various treatment options
 Limb salvage
 Amputation
 Adjuvant therapy
 Pros & Cons of Rx

44. Treatment
(Resuscitation)
 Anaemia
 Dehydration
 Electrolyte imbalance
 Infection
 Pathological fracture

45. Treatment…
(General Guide)
 Osteosarcoma & Ewing’s sarcoma
 Neoadjuvant chemo & surgery
 Chondrosarcomas
 Not sensitive to chemotherapy or radiotherapy
 Treatment is surgical
 Multiple myeloma
 Rx is mainly haematological
 Surgery is for fracture & spinal cord compression

46. Treatment…
(General Guide)
 Solitary bone lesion in previous hx of malignancy
 Should not be assumed a metastatic lesion!
 Surgical treatment of metastatic bone disease
 Palliative
 Epiphyseal & metaphyseal lesions
 Best treated with prosthetic replacement

47. Treatment…
(General Guide)
 Diaphyseal lesions
 Intramedullary nail
 In the shoulder
 Prosthetic replacements have poor function
 Internal fixation gives the best results
 In the hip
 Best treatment is arthroplasty

48. Treatment
(Surgical Resections)
Objective
 Tumour free resection
margins
 Preserving maximum
function

49. Treatment...
(Surgical Resections)
 Stage IA:
 Wide excision
 Stage IIA:
 Wide excision + Adjuvant
 Stage IIB:
 Radical resection + Adjuvant
 Stage III:
 Neoadjuvant, Radical resection
 Adjuvant Chemo radiation

50. Treatment…
(Management of defect after resection)
 BG (Vascularized / non-vascularized)
 Customized implants
 Allograft
 Custom made prosthesis
 Arthrodesis
 Distraction osteogenesis

51. Treatment…
(Limb Salvage)
 Considerations:
 Survival rates should be no
worse than with amputation
 Reconstructed limb must provide
satisfactory function
 Indications
 IA, IIA, IIIA
 Good response to neoadjuvant
chemotherapy

52. Treatment…
(Limb Salvage)
 Relative Contraindications
 Major neurovascular structures involvement
 Pathologic fractures
 Biopsy-site complications
 Severe infection in the surgical field
 Immature skeletal age with predicted LLD >8 cm
 Extensive soft-tissue involvement
 Poor response to neoadjuvant chemotherapy

53. Treatment…
(Limb Salvage)
 Advantage
 Long time survival 20% → 70%
 Disadvantages
 ↑ morbidity
 Multiple future surgery
 33% → amputation

54. Treatment…
(Limb Salvage)
Musculoskeletal Tumor Society Functional Evaluation
 Lower Extremity Data
 Pain
 Function
 Emotional acceptance
 Use of supports
 Walking ability
 Gait
Each of the six factors is graded 0 to 5
Maximum Extremity Score of 30 points
Outcome is reported as a percentage of the Score

55. Treatment…
(Limb Salvage)
Musculoskeletal Tumor Society Functional Evaluation
 Upper Extremity Data
 Pain
 Function
 Emotional acceptance
 Hand positioning
 Manual dexterity
 Lifting ability
Each of the six factors is graded 0 to 5
Maximum Extremity Score of 30 points
Outcome is reported as a percentage of the Score

56. Treatment…
(Amputation)
 Indications
 Late presentation
 Significant NV damage
 Poor extremity function
 Failed attempt at salvage
 Pathological #
 Poorly performed biopsy
 Persistent local recurrence
Definitive surgical Rx when limb
sparing is not visible

57. Treatment…
(Chemotherapy)
 Introduced in 1970
 Advantages
 Reduces the size of 10 lesion
 Prevents metastatic seedlings
 Allows easy resectability
 Improves chances of survival
 Drugs in use
 Methotrexate, Doxorubicin,
 Cyclophosphamide, vincristine
 Cisplatin, Etoposide

58. Treatment…
(Radiotherapy)
 Poorly effective
 Neoadjuvant / adjuvant Rx
 ↓ vascularity pre-op
 Extracorporeal Irradiation
 Microscopic margins
 Tumour site not accessible for surgery
 Relief of symptoms (pain, bleeding)

59. Treatment…
(20 malignant bone diseases)
 Aim
 Palliation
 Goals of fixation
 Immediate mobilization
 Protect the entire bone
 Type of fixation depends on location
 IM nailing for peritrochanteric lesions
 Hemiarthroplasty for femoral neck & head lesions

60. Treatment…
(20 malignant bone diseases)
 Harington's criteria for prophylactic fixation
 > 50% destruction of diaphysis
 > 50-75% destruction of metaphysis
 Permeative destruction in high stress
 Persistent pain following irradiation

61. Treatment…
(20 malignant bone diseases)

62. Treatment…
(20 malignant bone diseases)
 Bisphosphonates
 Highly effective inhibitors of bone resorption
 Pamidronate & zoledronic acid – most effective
 Prevents bone met in operable breast cancer

63. Treatment…
(20 malignant bone diseases)

64. Follow-up
 To detect
 Local recurrence
 Metastatic disease
at a time when Rx is possible & might be effective
 What?
 Physical examination of the tumour site
 Assessment of the function
 Possible complications of any reconstruction

65. Prognosis
 Metastasis
 Size
 Grade
 Location
 Response to chemo
 Surgical resection
 Age

66. Conclusion
 The surgical management of malignant bone
tumors presents many challenges
 With advances in chemotherapy, radiographic
imaging & reconstructive surgery; most patients
now can be offered limb-sparing surgery
 Amputation still plays an important role & offers
a standard to which other approaches must be
compared

67. References
 Will A, Timothy B, Louis S. Tumours. Apley’s System Of Orthopaedics &
Fractures, 11th Ed. Hodder Arnold 2010:(9)187 – 224
 Robert K. Heck J. General principles of tumours. Campbell's operative
Orthopaedics, 11th ed. Mosby Elsevier 2007;(19) 775 – 840
 Ebnezer J. Bone neoplasias. Textbook of orthopaedics. Jaypee brothers
2010; 4th ed. 43: 615 – 640
 Enneking WF. Principles of musculoskeletal oncologic surgery. Surgery of the
musculoskeletal system. 2nd ed. Churchill livingstone 1990; 5 (175): 4647 -
4669
 Ilana L, Paul C. Inflammation and infection and musculoskeletal tumours.
Bailey & Love’s short practice of surgery. Hodder Arnold 2008; 25th ed. 37:
539 - 563

68. References…
 Allan L, James R, Jean-Jacques B, Brendan F, Oyvind S, Michael A et al.
Advances in Treating Metastatic Bone Cancer. Clin Cancer Res. 2006;
12(20 Pt 2): 6209s–6212s
 Rong-Sen Y. Limb Salvage Operations for Patients with Malignant Bone
Tumors in the Extremities. Tzu Chi Med J 2005; 17:389-396
 Mccarroll HR. Practical Considerations In The Management Of Malignant
Bone Tumors. Jama. 1953;152(4):297-300
 Matthew RD, Gary EF. Malignant Bone Tumors: Limb Sparing Versus
Amputation. J Am Acad Orthop Surg 2003;11:25-37