Principles of diagnosis and treatment of Malignant Bone Tumors

1. Dr.Ledian Fezollari
11/02/2018

2. Introduction
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. 70% secondary, 30 % primary

3. WHO Classification

4. Clinical presantation
Age
Childhood and adolesence:
Most benign, and some malignant (Osteosarcoma, Ewing
Sarcoma)
Decade 4-5 th:
Chondrosarcoma and Fibrosarcoma
Decade 6th:
Myeloma( the commonest primary malignant bone tumor)
Over 70 y.o:
Metastatic lesions are the commonest

5.  Family History : may
be present in tumours
like exostosis/ von
recklenghausen`s
disease etc
Sex : Very few
tumours show sex
prediliction.
Eg GCT is commoner in
females.
Hereditary multiple exostosis

6. CLINICAL PRESENTATION- symptoms :
Pain :
Initially may be activity related, but in case of malignancy
there could be progressive pain at rest and at night.
In benign tumours, pain may be activity related when it is
large enough to compress surrounding soft tissue or when
it weakens bone.

7. Mass/ Swelling
 In case of soft tissue
sarcomas patients may come
with mass rather than pain
but in some exceptions like
nerve sheath tumours, they
have pain and neurological
conditions.
 Limitation of movement
 Pathologic fracture
 General symptoms

8. INVESTIGATIONS
Serological investigations
 FBC
 ERS
 Serum protein
electrophoresis
 Urine Bence Jones
protein
 PSA, prostatic acid
phosphatase
 Serum Calcium
 Serum ALP
 Antisarcoma antibodies
 Osteocalcin – A
 Flow Cytometry

9. Imaging
•
•
•
•
•
Radiography
CT scan
MRI
Radionuclide scanning
PET

10. INVESTIGATIONS: RADIOGRAPHS
• Phemister's Law = the most common site of
infection & tumours is the fastest growing site
of the long bone
• To see a lucent lesion in bone, an estimated
30 to 50 % of the bone must be lost
[Harris & Heaney, N Engl J Med 1969]

11. 1.Site
5.New
matrix
4.Periosteal
reaction
3.Bone
destruction
2.Border
Radiography
Information yielded by radiography
includes :

12. Radiography
I- Site
o Type of bone
Long bone / Flat bone
 Epiphysis /Metaphysis
/Diaphysis
 Intramedullary / Eccentric
/ Cortical lesion
 The epicenter of the
tumor helps to determine
the origin

13. Characteristic Locations
Simple bone cyst
Proximal humerus
Chondroblastoma
Epiphyses

14. Characteristic Locations
Giant Cell tumor
Epiphyses
Chordoma
Sacrum, Pelvis

15. Characteristic Locations
Osteoblastoma
Spine – posterior elements
Adamantinoma
Tibia

16. II-

17. Zone of Transition
Most reliable indicator for benign versus malignant lesions.
“Narrow”, if it is so well defined that it can be drawn
with a fine-point pen.
“Wide”, if it is imperceptible and can not be drawn at all.
An aggressive process should be considered, although
not necessarily a malignant lesion.
Narrow zone Wide zone

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
III- Type of Bone Destruction
Type 1: Geographic destruction

19. Type 2: Moth-eaten destruction
Areas of destruction with
ragged borders
Implies more rapid growth
Probably a malignancy
osteosarcoma

20. Type 3: Permeative Pattern
ill-defined lesion
with multiple “worm-holes”
Spreads through marrow space
Wide transition zone
Implies aggressive malignancy
Round-cell lesions
Leukemia
Ewing sarcoma.

21. IV:

22. Codman’s
triangle
Solid onion-peel Sunburst
Less malignant More malignant

23. V- Types of matrix
Matrix appearance of the bone lesion. A, Solid pattern of radiodensity
indicates a bone matrix. B, Stippled appearance or, C, rings and arcs suggest a
cartilage matrix. D, Hazy, smoky, or ground glass appearance correlates to a
fibrous matrix of the lesion.

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

25. Imaging:
Radionuclide scanning (Bone scan)
• Locate small tumors.
• Identify secondary deposits or skip lesions.
• Computed Tomography (CT)
• Extension and staging.
• Identify metastasis to other organs.
• CT Angio; help operation planning in highly vascular tumors.
Magnetic Resonance Imaging (MRI)
• Assess tumor spreading.
• Detailed view for soft tissue.
• Examining cystic-like lesion contents.

26. Biopsy
• Needle biopsy
• Large bore biopsy needle.
• Ultrasound or CT guided.
• Carried out in the line of any further operation.
• Open biopsy
• More reliable.
• Preferred if there is risk damaging neurovascular structures with the
needle biopsy.
• Preferably from the boundary zone so it will contain (normal tissue,
pseudocapsule, and abnormal tissue).
• Excisional biopsy; for some benign tumors.
• Curettage for cystic lesions.
Core biopsy (Preferred)
Open biopsy:
Incisional (Preferred)
Excisional
Curettage

27. The Enneking staging system has been shown to
prognosticate survival for bone sarcomas !!!

28. Informed Consent
 Natural hx of the disease
The various treatment options
 Limb salvage
 Amputation
 Adjuvant therapy
 Advantages and
disadvantages of Rx

29. Treatment
(General principles)
 Treatment of choice in most bone and soft tissue
sarcomas
 Preoperative radiation – soft tissue sarcomas
 Neoadjuvant chemotherapy – bone sarcomas
Osteosarcoma & Ewing’s sarcoma
Neoadjuvant chemo & surgery
Chondrosarcomas
Not sensitive to chemotherapy or radiotherapy
Treatment is surgical

30. Treatment
(General principles)
 Solitary bone lesion in previous hx of malignancy
 Should not be assumed a metastatic lesion!
 Surgical treatment of metastatic bone disease
 Palliative
 Multiple myeloma
Rx is mainly haematological
Surgery is for fracture & spinal cord compression

31. Treatment
(General principles)
 Epiphyseal & metaphyseal lesions
 Best treated with prosthetic replacement
 In the shoulder
 Prosthetic replacements have poor function
 Internal fixation gives the best results
 In the hip
 Best treatment is arthroplasty

32. Limb salvage or Amputation ???

33.  A set of surgical procedures designed to accomplish removal of a malignant
tumor and reconstruction of the limb with an acceptable oncologic, functional,
and cosmetic result**
Goal of limb salvage surgery
Condition that must achive:
1. Tumor free limb
2. Acceptable degree of function
3. Cosmetic appearance
4. Minimal amount of pain
5. Durable enough to withstand the
demands of normal daily activities
Combines two procedures-
Wide resection
Reconstruction of skeletal defect
Definition of limb salvage surgery

34. Indications
 Every patient with tumor of the
extremity should be considered
for limb salvage if the tumor can
be removed with an adequate
margin and the resulting limb is
worth saving
 No justification for limiting the
limb salvage process based only
on the prognosis
 Survival rates should be no
worse than with amputation
 Oncologic indications
 IA, IIA
 Good response to neoadjuvant
chemotherapy

35. Surgical Resections

36. 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
Treatment depends on the type of tumor and the stage
Not: Radical resection- May involve
amputation, disarticulation

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

38. I. Short diaphyseal segments can be
replaced by vascularized or non-
vascularised bone grafts
II. Longer gaps may require custom –
made implants
III. Osteoarticular segments can be
replaced by endoprosthesis or
allograft-prosthetic composites
Principles of management of
defect after resection

43. Treatment
(Limb Salvage)
Advantage
Long time survival 20%→ 70%
The function of the salvaged limb is better than that of
the amputation but not normal function
Disadvantages
 ↑ morbidity (↑ risk of infection, wound dehiscence,
flap necrosis,blood loss & DVT)
 Multiple future surgery
 33% → amputation

44. Contraindications
Three strike rule
 Bone
 Nerves
 Vessels
 Soft tissue envelope
If three of these key components
are involved, the limb salvage is
probably not worth considering
 Major neurovascular involvement
 Displaced pathologic fracture
 Fungating and infected tumors
 Inability to afford chemotherapy or
poor response to neoadjuvant
chemio
 Recurrence of malignant tumors
 Complications sec to poorly
performed biopsy
 Skeletal immaturity - 60% growth
occur through distal femoral and
proximal tibial epiphysis
 Pulmonary metastasis is not a
contraindication of surgery
 Contraindications of limb salvage
are the indications for amputation

45. 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

46. AMPUTATIONS :
Amputation provides definitive
surgical treatment when Limb
sparing is not a prudent one.
Common amputations in
malignant tumours:
 Proximal humerus : fore
quarter amputation
 Distal femur : hip disarticulation
 Proximal tibia : mid thigh
amputation

47. Treatment
(Chemotherapy)
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

48. 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)

49. Treatment
(Bone metastases)
Aim
 Palliation
1. Prophylactic fixation of metastatic deposits where there is a
risk of fracture
2. Stabilization/Reconstruction following pathological fracture
3. Decompression of spinal cord & nerve roots and stabilization
for spinal instability
Not:Type of fixation depends on location
 IM nailing for peritrochanteric lesions
 Hemiarthroplasty for femoral neck & head lesions

50. 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
 Success depends on prompt detection and early referral by primary care
doctor and on careful and coordinated sequences of events
 Achieving a surgical margin that will ensure a low rate of local
recurrence is paramount
 Amputation still plays an important role & offers
a standard to which other approaches must be
compared

51. References
1. Campbell’s Operative Orthopaedics 13 edition
2. American Academy of Orthopaedic Surgeons
– Text book of Orthopaedic knowledge update 8
series
3. Samuel Turek Text Book of Orthopaedics: 3d
edition
4. www.uptodate.com/musculoskeletal tumours
5. WHO Manual 2001 reprint for Classification of
Musculoskeletal Tumours Ebnezer J. Bone
neoplasias. Textbook of orthopaedics, 2010; 4th
edition