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
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
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!!!
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
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
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
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
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
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
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
Editor's Notes
The incidence
of malignant bone tumors shows a striking age-specific
distribution: in the age group 0–40 years, there is an incidence
peak between 10 and 20 years (primarily osteosarcoma
and Ewing’s sarcoma) and for the age group
above 40 years there is a steady increase in incidence up
to 80 years (primarily chondrosarcoma and to a lesser
degree Paget’s related osteosarcoma) (Dorfman and
Czerniak 1995, 1998; Unni et al. 2005).
The three most common genuine primary bone
malignancies (osteosarcoma, chondrosarcoma, and Ewing’s
sarcoma) account for only 0.2% of all malignancies
in the UK and USA; however, in children (< 15 years)
malignant bone tumors account for approximately 5%
of all malignancies (Dorfman and Czerniak 1995,
1998; Unni et al. 2005).
As the management of cancer becomes more complex, it becomes impossible for any individual clinician to have the intellectual and technical competence that is necessary to manage all the patients presenting with a particular type of tumour.
Accurate diagnosis is the key to the successful management of
cancer
The pathologic diagnosis of primary bone tumors poses
particular problems:
1. Their rarity prevents most pathologists from gaining
sufficient diagnostic experience.
2. There is an unusual need for the pathologist to be familiar
with and to integrate clinical, laboratory, and
imaging findings in the final diagnosis.
3. Despite their rarity, there is a wide spectrum of bone
lesions with overlapping morphologic features.
4. The distinction between neoplastic, reactive/inflammatory,
and metabolic bone lesions as well as some
developmental disorders is sometimes difficult.
5. The diagnosis of malignant bone tumors, which
frequently involve children or young adults, often
has dramatic consequences in terms of surgical and
adjuvant treatment. Moreover, there are a number
of rare hereditary and non-hereditary conditions
associated with increased risk of developing bone
tumors that the pathologist needs to be aware of.
Within these teams the
pathologists have the important role to establish the
correct diagnosis, to arrange for and interpret required
adjunctive diagnostic tests (immunohistochemistry, cytogenetic/
molecular analyses), to provide prognostic
information, to identify patients that should be considered
for adjuvant treatment protocols or trials, and to
assess treatment response.
Pathologic fracture violates compartment boundary
Bisphosphonates are a class of pyrophosphate analogues
that bind with high affinity to mineralized bone surfaces
and inhibit osteoclastic bone resorption. They are used
extensively to treat patients with diseases of bone loss,
such as osteoporosis and Paget’s disease, and cancers that
cause osteolysis.Common examples of bisphosphonates
are pamidronate (Aredia), alendronate (Fosamax),
zoledronate (Zometa) and clodronate (Bonefos). The
bisphosphonate market for these diseases is over US $1.5
billion dollars annually. The newer bisphosphonates act
by inhibiting specific enzymes in the mevalonate
pathway of cholesterol biosynthesis in osteoclasts,which,
in turn, leads to impaired prenylation of important small
GTP-binding proteins such as RHO, and to subsequent
changes in the cytoskeletal function that promotes
osteoclast apoptosis.
The following are prognostic and predictive factors for bone cancer.
Extent of the tumour
The extent of the tumour is the most important prognostic factor for bone cancer. Metastases are associated with a poorer prognosis. However, the location of metastases is also important in determining prognosis.
Lung metastases have a more favourable prognosis than metastases to other distant sites, such as the brain and other bones.
Metastases to the lymph nodes or bone marrow are a less favourable prognostic factor.
Size of the tumour
Smaller tumours have a more favourable prognosis than larger tumours.
Grade of the tumour
Low-grade tumours are associated with a more favourable prognosis than high-grade tumours.
Location of the tumour
Tumours that occur in bones farther away from the centre of the body, such as the limbs (distal tumours), have a more favourable prognosis than tumours that occur close to the central part of the body (proximal tumours), such as the skull, vertebrae, sternum, ribs and pelvis.
Response to chemotherapy
Chemotherapy is often given before surgery.
After surgery, the tumour is examined to see how many cells were killed by chemotherapy (necrosis).
In people with Ewing sarcoma, response to chemotherapy can often be assessed by an MRI before surgery to see how much the tumour has shrunk.
People with at least 90% necrosis in the primary tumour after chemotherapy have a more favourable prognosis than those with less necrosis.
Surgical resection
Tumours that are completely removed, with no cancer cells in the margins around the tumour, have a much more favourable prognosis.
Age
Age is a prognostic factor for osteosarcoma and Ewing sarcoma. Younger people may have a more favourable prognosis.
Read more: http://www.cancer.ca/en/cancer-information/cancer-type/bone/prognosis-and-survival/?region=on#ixzz4WzKpR4Q9