PRIMARY MALIGNANT BONE


TUMOURS
Dr. Henry Pebruanto, SpOT

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CURICULUM VITAE

EDUCATION UNDER-POST GRADUATE TRAININGS

p Medical Faculty of Udayana University, Denpasar – Bali; 2005
p Orthopaedic Training (Airlangga Univ., Sby/ Udayana Univ., Dps); Jan 12
p Overseas Observership Programme (Hip and Knee Arthroplasty): Perth – WA; Nov 10
p ASEAN Cadaveric Knee Course: Chiang Mai Univ. – Thailand; Apr 12.
p Australian Educational Program (Knee &Shoulder): Sydney – NSW; Jun 12
p ASEAN Cadaveric Knee Course: Bangkok – Thailand; May 14.
p ASEAN EASE (Express Arthroplasty and Arthroscopy Skills Education) Knee Course : Bangalore – India; May 15.

COURSES
p Advanced Trauma Life Support (Denpasar – Bali); Jan 06
p Basic Orthopedic Skill Course (Surabaya – East Java); Aug 07
p Basic Surgical Skills Course (Surabaya – East Java); Jan 08
p Ultrasonography for Abdominal and Chest Trauma Course (Jakarta); Apr 08
p Total Nutritional Treatment Course (Tanah Lot – Bali); Dec 08
p Basic AO Trauma Course (Nusa Dua – Bali); May 09
p Bali Hand Course (Denpasar – Bali); Jul 09
p Post Graduate Course : Musculoskeletal Trauma (Jakarta); Nov 09
p 8th Annual Meeting of Indonesian Spine Society & 1st International Society for Minimal Intervention in Spinal
Surgery (Denpasar – Bali); Jun 10

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COURSES

p Workshop Hemiarthroplasty and Bone Substitute (Malang – East Java); Jun 10

p Ponsetti Course (Denpasar – Bali); Dec 10

p 4th Arthroplasty Workshop : Jump Start on Total Knee Replacement (Jakarta); Apr 11

p 3rd Arthroplasty Workshop : Jump Start on Total Hip Replacement (Jakarta); Apr 11

p AO Spine Principles Course (Jakarta); Jun 11

p Current Diagnosis and Comprehensive Treatment of Brachial Plexus Injury (Surabaya

– East Java); Oct 11

p 3rd Pelvic and Acetabular Course and Workshop (Surabaya – East Java); Oct 11

p Lower Extremity Trauma Course (Denpasar – Bali); Jan 12

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OSTEOSARCOMA

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OSTEOSARCOMA
p The most common primary malignant bone tumour ~

p bimodal age distribution (adolescence and in the 7th decade)

p High-grade, medullary osteoid-producing tumour spreading rapidly outwards

through the periosteum and into surrounding tissues.

p Typically metaphyseal or metadiaphyseal,

p Distal femur, proximal tibia, proximal femur and humerus

p Worsening pain and swelling (night pain, non-mechanical pain) or joint restriction.

p Fever, elevated alkaline phosphatase (ALP) and lactate dehydrogenase (LDH)

p Skin may be warm and erythematous with venous engorgement.

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OSTEOSARCOMA
p Primary (arising de novo) or secondary (arising in abnormal bone).

p Secondary osteosarcomas ~ related to :

p previous irradiation, Paget’s disease, fibrous dysplasia, bone infarcts,

liposclerosing myxofibrous tumour, chronic osteomyelitis or in
dedifferentiated chondrosarcomas

p less favourable prognosis.

p This explains the secondary elevation in the bimodal.

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OSTEOSARCOMA
X-rays

p Generally diagnostic

p An ill-defined, permeative bone-forming lesion causing cortical

destruction, periosteal reaction and expansion into the soft tissues.

p Codman’s triangle (new bone forms in response to periosteal elevation)

p ‘Sunburst’ appearance (the periosteum does not have enough time to

lay down a new layer and instead the Sharpey’s fibres stretch
perpendicular to the periosteum) (Figure 9.25).

p 80% are extra-compartmental

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OSTEOSARCOMA
X-rays

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OSTEOSARCOMA
MRI

p Delineate the medullary and extra-osseous extent of the tumour.

Nuclear Medicine Bone Scintigraphy

p intensively hot and may identify bone skip lesions, distant bone metastases
or bone-producing chest metastases.

CT Chest

p mandatory as lung metastases are common (up to 15%).

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OSTEOSARCOMA

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OSTEOSARCOMA
Biopsy

p Always be carried out before commencing treatment;

p Carefully planned according to the principles of performing a biopsy

p Neoplastic cells demonstrate severe anaplasia and pleomorphism,

producing primitive woven bone and osteoid and display a permeative
pattern replacing host bone.

p Conventional osteosarcoma is subclassified according to the predominant

extracellular matrix evident in the tissue,

p osteoblastic osteosarcoma, chondroblastic and fibroblastic variants.

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OSTEOSARCOMA
Treatment

p Advances in multi-agent chemotherapy, diagnostic imaging and surgical

reconstruction ~ limb-salvage surgery,

p Limb salvage ~ preferred method of treatment for osteosarcomas

p Amputation ~ when attempted tumour excision would compromise safe

surgical tumour margins.

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OSTEOSARCOMA
Treatment

p The principal first-line chemotherapeutic agents : doxorubicin, cisplatin,

ifosfamide and methotrexate.

p Multi-agent neoadjuvant chemotherapy ~ started immediately after

diagnosis for 8–12 weeks

p After re-staging to evaluate response to induction chemotherapy and

provided the tumour is resectable and there are no skip lesions ! a
wide resection and limb reconstruction are performed.

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OSTEOSARCOMA
Treatment

p The resected specimen ~ histopathological analysis to determine if

satisfactorily wide margins have been achieved and the response to
chemotherapy is judged by the degree of tumour cell necrosis.

p These can predict the risk of local recurrence and survival.

p Minimum 90% necrosis rate is required for a good response.

p Factors predictive of a worse outcome are

p large tumours, ablative surgery, age under 14 years, male gender, high
ALP, local recurrence, p-glycoprotein expression, and absent Erb2
expression.

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OSTEOSARCOMA
Treatment

p If tumour necrosis is marked, chemotherapy is continued for another 6–12

months.

p If the response is poor, a different chemotherapeutic regime is substituted.

p Pulmonary metastases

p small and peripherally situated ~ completely resected with a wedge of

lung tissue.

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OSTEOSARCOMA
Treatment

p Five-year survival after wide resection and chemotherapy over 60%.

p Today, limb-salvage surgery is possible in 90% of cases.

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OSTEOSARCOMA
VARIANTS OF OSTEOSARCOMA

p Parosteal osteosarcoma

p Periosteal osteosarcoma

p Secondary osteosarcoma

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CHONDROSARCOMA

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CHONDROSARCOMA
p Chondrosarcomas ~ the second most common primary malignant bone
tumours

p In adults from the third to the eighth decades

p Peaking between 40 and 70 years of age

p Men > women

p Slow-growing

p Deep pain and/or a gradually enlarging mass

p Metaphyseal lesions

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CHONDROSARCOMA
p Radiographically ~ large, intraosseous, osteolytic tumours with a narrow
zone of transition and irregular, granular calcifications within the matrix
described as ‘honeycomb’ or ‘popcorn’(Figure 9.27).

p Endosteal scalloping of the cortex and eventual cortical destruction can

occur

p A faint periosteal reaction.

p CT ~ matrix calcifications and permeative destruction of the tumour.

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CHONDROSARCOMA
p MRI ~ to define tumour extent and identify more biologically active parts of
the tumour
p These avascular tumours
p Differentiation of low-grade from high-grade chondrosarcomas is essential
before surgery
p MRI can reliably differentiate high-grade from low-grade chondrosarcomas
of long bone.
p Differentiating features are bone expansion, periostitis, soft-tissue mass and
tumour length (mean intramedullary extent 11.8 cm in high-grade tumours;
5.5 cm in low-grade tumours),
p Presence of these four MRI features has a diagnostic accuracy of 96%.

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CHONDROSARCOMA

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CHONDROSARCOMA
p Most common sites are the femur and pelvis.

p Very poor prognosis (5-year survival 7–24%),

p improved only by wide surgical resection

p Worsened by pathological fracture (29% of cases), advancing age and

metastasis at diagnosis.

p Chemotherapy and radiotherapy have not been shown to improve

survival.

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CHONDROSARCOMA
p Full staging ~ X-rays and MRI of the entire bone affected,

p prior to biopsy

p including bone scintigraphy and chest CT.

p A biopsy is essential to confirm the diagnosis.

p Histopathology : macroscopically lobular white hyaline cartilage, areas of

mineralization and cystic changes, erosion of the cortex and soft-tissue
expansion.

p Microscopically : the high cellularity, atypia, mitoses and permeation

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CHONDROSARCOMA
p Resistant to both chemotherapy and radiation
p Surgical excision the only treatment.
p In high-grade tumours ~ wide excision margins
p Prognosis is determined by the cellular grade, stage, tumour size, (axial
versus appendicular) site and the resection margin.
p Overall survival at 5 years :
p Low-grade tumours is 90–100%,

p Grade II tumours approximately 60%

p Grade III approximately 30–40%.

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CHONDROSARCOMA
p In low-grade chondrosarcomas ~ intralesional curettage or radiofrequency
ablation in selected tumours

p this may be associated with higher rates of local recurrence

p recurrences may transition to higher- grade tumours ~ worse prognosis.

p For this reason, others recommend wide resection and reconstruction ~

reduced risk of local recurrence.

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EWING’S SARCOMA

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EWING’S SARCOMA
p Arise from mesenchymal stem cells in the bone marrow

p > males (between 10 and 20 years of age)

p a diaphyseal long-bone location but is equally as common in flat bones.

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EWING’S SARCOMA
p Pain

p Swelling

p Low-grade fever

p ESR and WCC may be elevated ; haemoglobin may be reduced.

p Dd/ include osteomyelitis.

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EWING’S SARCOMA
p Radiographically

p an aggressive, permeative, poorly defined osteolytic lesion with cortical

destruction, periosteal reaction and large, radiolucent soft-tissue mass
may be found.

p Periosteal reaction is common in young patients with the lamellar

‘onionskin’ appearance causing fusiform bone enlargement (Figure
9.29).

p Mimic infection or eosinophilic granuloma in young patients;

p Subtle bone involvement with a large soft-tissue mass could mimic

primary bone lymphoma in older patients.

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EWING’S SARCOMA
p Radiographically

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EWING’S SARCOMA
p Routine local and distal staging includes bone scintigraphy and chest CT.

p MRI ~ the intra- and extra-osseous tumour extent,

p Lesions ~ ‘hot’ with bone scintigraphy.

p Lung, skeletal and lymph node (rare) metastases ~ 20% of cases.

p Bone marrow involvement is a unique feature of Ewing’s sarcoma

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EWING’S SARCOMA

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EWING’S SARCOMA

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EWING’S SARCOMA
p Ewing’s sarcoma is composed of primitive, undifferentiated, small, round
blue cells with large nuclei and scant cytoplasm that bears no resemblance
to normal tissue.

p The characteristic translocation between chromosomes 11 and 22 results in

a fusion gene, EWSR1-FL11, which is present in 90% of cases.

p Other translocations ~ most frequently t(21:22).

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EWING’S SARCOMA
p Preoperative chemotherapy frequently results in extensive tumour necrosis
and shrinkage.

p Include vincristine, ifosfamide, doxorubicin and etoposide.

p Wide excision +/- adjuvant radiotherapy (if surgical margins are poor)

p Definitive radiotherapy ~ non-resectable or metastasis

p Favourable outcome is expected with younger age, distal appendicular

sites, small tumour volume, normal ESR and greater chemotherapy
necrosis response.

p Five-year survival greater than 60% can be expected for appendicular

tumours.

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THANK YOU

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