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MANAGEMENT OF
PRIMARY BONE
TUMOURS
DR TELLA A.O.
23/01/13
OUTLINE
• OVERVIEW
• CLINICAL EVALUATION
- History of the patient
- Physical Examination
- Investigations
- Treatment
• FOLLOW-UP
• PROGNOSIS
• CONCLUSION
OVERVIEW
• Bone tumours can be very diverse in
morphology and biologic potential
• Most bone tumours are benign (˃95% of
cases)
• Malignant tumours may be primary or
secondary
- Primary malignant bone tumours are very
rare (˂0.2% of all cancers-NCCN)
OVERVIEW
• Most primaries occur in long bones
• May be quite difficult to diagnose
specifically
• Morbidity and mortality worse in
developing countries
• Multimodal approach to management has
improved survival
CLINICAL EVALUATION
• High index of suspicion is the first step in
management
• Requires a multidisplinary team
• Core Group:
- Orthopaedic oncologist
- Bone pathologist
- Medical/paediatric oncologist
- Radiation oncologist
- Musculoskeletal radiologist
CLINICAL EVALUATION
• Management involves:
○ Meticulous history
○ Thorough physical examination
○ Prebiopsy radiological evaluation
○ Biopsy
○ Other relevant investigations
○ Staging of the tumour
○ Treatment plan
HISTORY OF THE PATIENT
● PRESENTING SYMPTOMS:
Pain
Mass
An abnormal radiographic finding detected
during evaluation for other conditions
Neurological symptoms
Previous radiation exposure
Constitutional symptoms
History of trauma
● AGE OF THE PATIENT – a very important clue
PHYSICAL EXAMINATION
● Evaluation of patient’s general health
● TUMOR MASS should be measured & its
location, shape, consistency, mobility,
tenderness noted
● SKIN & SUBCUTANEOUS TISSUE :
Small dilated superficial veins overlying the
mass are produced by large tumors
Café-au-lait spots & subcutaneous
neurofibromas indicate Von
Recklinghausen’s disease
PHYSICAL EXAMINATION
● REGIONAL LYMPH NODES: sign of
metastatic disease
● Atrophy of surrounding musculature
● Neurological deficits
● Peripheral pulses → Adequacy of
circulation.
● Other systems e.g chest
RADIOGRAPHIC EVALUATION
• Plain X-rays
• Bone Scans
• Computed Tomography (CT)
• Magnetic Resonance Imaging (MRI)
• Angiography
• PET Scans
PLAIN X-RAYS
• Provides most useful diagnostic information
in evaluation of bone tumours
• Guides the selection of other imaging
techniques
• Radiographic parameters are different for
both benign and malignant bone tumours:
- Location and nature of the lesion
- Periosteal reaction
- Soft tissue changes
- Matrix of the tumour
- pathological fracture
 Geographic pattern
 Ex: Most benign tumours
Non-ossifying
fibroma
Unicameral Bone
Cyst
 MOTH-EATEN PATTERN
 Ex: Multiple myeloma, Osteosarcoma
 PERMEATIVE PATTERN
 Ex: Ewing’s sarcoma
PERIOSTEAL REACTIONS
• Benign tumours:
- None
- Solid
• Malignant tumours
- Codman’s triangle
- Sunburst
- Onion skinning or Lamellated
 Codman’s triangle
 Sunburst
 Onion skinning
CartilaginousOsteoblasticNo matrix
EXPANSILE LESIONS OF BONE
Enchondroma
Fibrous
Dysplasia
BONE SCANS
• Uses very low radioactive
material like technetium
to assess spread to other
bones
- Polyostotic involvement
- Skeletal metastases
• Isotope Thallium-201
used to assess tumour
response to
chemotherapy
CT SCANS
• CT depicts transverse
anatomic relationship
of the tumour to
surrounding structures
• 3-D reconstruction
useful in pre-op
planning e.g pelvis
• Helps in evaluation of
pulmonary metastases
MRI
• Has better contrast
discrimination
• Images can be
performed in any
plane
• Useful in detecting
neurovascular
bundles and skip
metastases
ANGIOGRAPHY
• Useful in
determining
relationship of major
vessels to the
tumour
• Pre-op embolization
of a highly vascular
tumour can be done
prior to surgical
resection
ADDITIONAL INVESTIGATIONS
►LABORATORY INVESTIGATIONS:
• FBC, ESR, E/U/Cr
• Serum Cacium & Phosphate
• Serum electrophoresis
• Urinary Bence Jonce Protein estimation
DIFFERENTIAL DIAGNOSES
DIFFERENTIALS
BIOPSY
• Tissue sampling for pathological evaluation
• The planning and technique is important
- Error may have negative impact on survival
• Biopsy of bone tumours can be done open or
closed
- Needle biopsy to be done with image
guidance
• Patient should be well prepared
BIOPSY
• The smallest longitudinal incision compatible with
obtaining adequate sample should be employed
• Knife and bone curette should be used to avoid
crushing the specimen
• Small circular holes minimise stress risers on the bone
• Meticulous haemostasis must be maintained
- Careful use of tourniquet
• Exposure should violate one compartment
• Drain to pass through incision wound
• All biopsies must be cultured and vice versa
BIOPSY
 Enneking surgical staging system
TREATMENT
• INITIAL RESUSCITATION
- Anaemia to be corrected
- Pain control
- Antibiotics
• Palliative treatment for metastatic
disease
CURETTAGE OF BONE TUMOURS
• Used for benign active tumours e.g unicameral
bone cyst, aneurysmal bone cyst, giant cell
tumour
• The technique involves creating a window and
‟scooping” out the tumour
• The bony defect created can then be
reconstructed using bone graft, PMMA or
biologic fillers
• Cryosurgery (use of liquid Nitrogen) is often
used to kill remaining tumour cells
AMPUTATION
• Involves the removal of the entire bone and soft
tissues at a safe proximal level to the tumour
• Often indicated in complex tumours with
neurovascular compromise and tumours
complicated with infection and severe soft
tissue compromise
• Requires careful planning :
- patient’s goal and expectations
- Oncologic and functional outcome
• May entail intralesional, marginal , wide or
radical excision
LIMB-SPARING PROCEDURE
• Currently being employed due to advances in
imaging modalities and availability of tumour
prosthesis
• Requires patient preparation, staging studies,
adequate biopsy, pre-op and post-op
chemotherapy
• Phases of operation:
○ Tumour resection
○ Skeletal reconstruction
○ Soft tissue reconstruction
REQUIREMENTS
• No major neurovascular
involvement
• Wide resection of
affected bone and cuff of
normal tissues
• Resection of bone 3-4cm
beyond abnormal uptake
on bone scan
• Complete soft tissue
coverage
• Contraindications:
1. Major neurovascular
involvement
2. Pathologic fractures
3. Inadequate biopsy
4. Tumour complications
e.g infection, muscle
necrosis
5. Skeletal immaturity
METHODS OF SKELETAL
RECONSTRUCTION
• Autograft (vascularised)
• Allograft (Osteoarticular)
• Modular endoprosthesis
• Allograft-prosthetic composites
• Expandable prosthesis
• Rotationplasty
• Arthrodesis
• Limb lenghtening
LIMB-SPARING VS AMPUTATION
• Survival of the patient
• Functional outcome
• Complications
• Psychological factors
• Cost
CHEMOTHERAPY
• Effective multiagent chemotherapy has improved
overall survival of patients
• Could be given as Noeadjuvant or Adjuvant therapy
• Neoadjuvant chemotherapy avoids tumour
progression and decreases tumour spread at time of
surgery
• Not very useful in cartilaginous and low-grade
tumours
• Patient must be well prepared
• Response to chemotherapy needs to be measured
RADIOTHERAPY
• Most primary malignant bone tumours are
relatively radioresistant
• Useful in multiple myeloma, Ewing’s
sarcoma, spinal tumour
• Brachytherapy or EBRT employed
FOLLOW-UP CARE
• Aim is to detect local recurrence or metastatic
disease
• Patients are seen at regular intervals after
completion of initial treatment
• Entails adequate assessment of the patients
- Physical examination
- Lab investigations
- Imaging studies
• Long term complications of treatment also
evaluated
CONCLUSION
• Treatment of MSS tumours still expanding
• Results better with dedicated centres
- Multidisplinary team
- Research oriented
- Tumour registry
• Early detection and appropriate treatment
remains the key in reducing morbidity and
mortality
Management of primary bone tumours

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Management of primary bone tumours

  • 2. OUTLINE • OVERVIEW • CLINICAL EVALUATION - History of the patient - Physical Examination - Investigations - Treatment • FOLLOW-UP • PROGNOSIS • CONCLUSION
  • 3. OVERVIEW • Bone tumours can be very diverse in morphology and biologic potential • Most bone tumours are benign (˃95% of cases) • Malignant tumours may be primary or secondary - Primary malignant bone tumours are very rare (˂0.2% of all cancers-NCCN)
  • 4. OVERVIEW • Most primaries occur in long bones • May be quite difficult to diagnose specifically • Morbidity and mortality worse in developing countries • Multimodal approach to management has improved survival
  • 5. CLINICAL EVALUATION • High index of suspicion is the first step in management • Requires a multidisplinary team • Core Group: - Orthopaedic oncologist - Bone pathologist - Medical/paediatric oncologist - Radiation oncologist - Musculoskeletal radiologist
  • 6. CLINICAL EVALUATION • Management involves: ○ Meticulous history ○ Thorough physical examination ○ Prebiopsy radiological evaluation ○ Biopsy ○ Other relevant investigations ○ Staging of the tumour ○ Treatment plan
  • 7. HISTORY OF THE PATIENT ● PRESENTING SYMPTOMS: Pain Mass An abnormal radiographic finding detected during evaluation for other conditions Neurological symptoms Previous radiation exposure Constitutional symptoms History of trauma ● AGE OF THE PATIENT – a very important clue
  • 8.
  • 9. PHYSICAL EXAMINATION ● Evaluation of patient’s general health ● TUMOR MASS should be measured & its location, shape, consistency, mobility, tenderness noted ● SKIN & SUBCUTANEOUS TISSUE : Small dilated superficial veins overlying the mass are produced by large tumors Café-au-lait spots & subcutaneous neurofibromas indicate Von Recklinghausen’s disease
  • 10. PHYSICAL EXAMINATION ● REGIONAL LYMPH NODES: sign of metastatic disease ● Atrophy of surrounding musculature ● Neurological deficits ● Peripheral pulses → Adequacy of circulation. ● Other systems e.g chest
  • 11.
  • 12. RADIOGRAPHIC EVALUATION • Plain X-rays • Bone Scans • Computed Tomography (CT) • Magnetic Resonance Imaging (MRI) • Angiography • PET Scans
  • 13. PLAIN X-RAYS • Provides most useful diagnostic information in evaluation of bone tumours • Guides the selection of other imaging techniques • Radiographic parameters are different for both benign and malignant bone tumours: - Location and nature of the lesion - Periosteal reaction - Soft tissue changes - Matrix of the tumour - pathological fracture
  • 14.
  • 15.  Geographic pattern  Ex: Most benign tumours Non-ossifying fibroma Unicameral Bone Cyst
  • 16.  MOTH-EATEN PATTERN  Ex: Multiple myeloma, Osteosarcoma
  • 17.  PERMEATIVE PATTERN  Ex: Ewing’s sarcoma
  • 18. PERIOSTEAL REACTIONS • Benign tumours: - None - Solid • Malignant tumours - Codman’s triangle - Sunburst - Onion skinning or Lamellated
  • 23. EXPANSILE LESIONS OF BONE Enchondroma Fibrous Dysplasia
  • 24. BONE SCANS • Uses very low radioactive material like technetium to assess spread to other bones - Polyostotic involvement - Skeletal metastases • Isotope Thallium-201 used to assess tumour response to chemotherapy
  • 25. CT SCANS • CT depicts transverse anatomic relationship of the tumour to surrounding structures • 3-D reconstruction useful in pre-op planning e.g pelvis • Helps in evaluation of pulmonary metastases
  • 26. MRI • Has better contrast discrimination • Images can be performed in any plane • Useful in detecting neurovascular bundles and skip metastases
  • 27. ANGIOGRAPHY • Useful in determining relationship of major vessels to the tumour • Pre-op embolization of a highly vascular tumour can be done prior to surgical resection
  • 28. ADDITIONAL INVESTIGATIONS ►LABORATORY INVESTIGATIONS: • FBC, ESR, E/U/Cr • Serum Cacium & Phosphate • Serum electrophoresis • Urinary Bence Jonce Protein estimation
  • 31. BIOPSY • Tissue sampling for pathological evaluation • The planning and technique is important - Error may have negative impact on survival • Biopsy of bone tumours can be done open or closed - Needle biopsy to be done with image guidance • Patient should be well prepared
  • 32. BIOPSY • The smallest longitudinal incision compatible with obtaining adequate sample should be employed • Knife and bone curette should be used to avoid crushing the specimen • Small circular holes minimise stress risers on the bone • Meticulous haemostasis must be maintained - Careful use of tourniquet • Exposure should violate one compartment • Drain to pass through incision wound • All biopsies must be cultured and vice versa
  • 34.  Enneking surgical staging system
  • 35. TREATMENT • INITIAL RESUSCITATION - Anaemia to be corrected - Pain control - Antibiotics • Palliative treatment for metastatic disease
  • 36.
  • 37. CURETTAGE OF BONE TUMOURS • Used for benign active tumours e.g unicameral bone cyst, aneurysmal bone cyst, giant cell tumour • The technique involves creating a window and ‟scooping” out the tumour • The bony defect created can then be reconstructed using bone graft, PMMA or biologic fillers • Cryosurgery (use of liquid Nitrogen) is often used to kill remaining tumour cells
  • 38.
  • 39. AMPUTATION • Involves the removal of the entire bone and soft tissues at a safe proximal level to the tumour • Often indicated in complex tumours with neurovascular compromise and tumours complicated with infection and severe soft tissue compromise • Requires careful planning : - patient’s goal and expectations - Oncologic and functional outcome • May entail intralesional, marginal , wide or radical excision
  • 40.
  • 41. LIMB-SPARING PROCEDURE • Currently being employed due to advances in imaging modalities and availability of tumour prosthesis • Requires patient preparation, staging studies, adequate biopsy, pre-op and post-op chemotherapy • Phases of operation: ○ Tumour resection ○ Skeletal reconstruction ○ Soft tissue reconstruction
  • 42. REQUIREMENTS • No major neurovascular involvement • Wide resection of affected bone and cuff of normal tissues • Resection of bone 3-4cm beyond abnormal uptake on bone scan • Complete soft tissue coverage • Contraindications: 1. Major neurovascular involvement 2. Pathologic fractures 3. Inadequate biopsy 4. Tumour complications e.g infection, muscle necrosis 5. Skeletal immaturity
  • 43. METHODS OF SKELETAL RECONSTRUCTION • Autograft (vascularised) • Allograft (Osteoarticular) • Modular endoprosthesis • Allograft-prosthetic composites • Expandable prosthesis • Rotationplasty • Arthrodesis • Limb lenghtening
  • 44.
  • 45.
  • 46.
  • 47. LIMB-SPARING VS AMPUTATION • Survival of the patient • Functional outcome • Complications • Psychological factors • Cost
  • 48. CHEMOTHERAPY • Effective multiagent chemotherapy has improved overall survival of patients • Could be given as Noeadjuvant or Adjuvant therapy • Neoadjuvant chemotherapy avoids tumour progression and decreases tumour spread at time of surgery • Not very useful in cartilaginous and low-grade tumours • Patient must be well prepared • Response to chemotherapy needs to be measured
  • 49. RADIOTHERAPY • Most primary malignant bone tumours are relatively radioresistant • Useful in multiple myeloma, Ewing’s sarcoma, spinal tumour • Brachytherapy or EBRT employed
  • 50. FOLLOW-UP CARE • Aim is to detect local recurrence or metastatic disease • Patients are seen at regular intervals after completion of initial treatment • Entails adequate assessment of the patients - Physical examination - Lab investigations - Imaging studies • Long term complications of treatment also evaluated
  • 51. CONCLUSION • Treatment of MSS tumours still expanding • Results better with dedicated centres - Multidisplinary team - Research oriented - Tumour registry • Early detection and appropriate treatment remains the key in reducing morbidity and mortality