management of primary bone tumours

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:PainMassAn abnormal radiographic finding detected

during evaluation for other conditionsNeurological symptomsPrevious radiation exposureConstitutional symptomsHistory 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 tumorsCafé-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

ALGORITHM

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

LOCATION OF THE TUMOUR

Geographic patternEx: Most benign tumours

PATTERN OF BONE DESTRUCTION

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

TUMOUR MATRIX

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

STAGING OF THE TUMOUR

TREATMENT

• INITIAL RESUSCITATION- Anaemia to be corrected- Pain control- Antibiotics

• Palliative treatment for metastatic disease

TYPES OF TUMOUR EXCISION

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

CURETTAGE OF BONE TUMOURS

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

AMPUTATION

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 involvement2. Pathologic fractures3. Inadequate biopsy4. Tumour complications e.g infection, muscle necrosis5. Skeletal immaturity

METHODS OF SKELETAL RECONSTRUCTION

• Autograft (vascularised)• Allograft (Osteoarticular)• Modular endoprosthesis• Allograft-prosthetic composites• Expandable prosthesis• Rotationplasty• Arthrodesis• Limb lenghtening

ROTATIONPLASTY

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

MANY THANKS