Bone tumor

By

Daniel A. (Orthopedic
Surgeon)
Outline
Introduction
Classification
Diagnosis
Clinical
Imaging
Laboratory
Biopsy
Staging
Principles of management
Introduction
Bone tumor is an abnormal growth of cells within
the bone that may be malignant or benign.
 Etiology = Unknown
• Inherited mutation
• Radiation
• Predisposing conditions:
 Paget’s disease
 Fibrous dysplasia
 Retinoblastoma
 Syndromes eg. Gardner's, Ollier’s disease
 Primary bone tumor = rare accounting 0.2%
 Secondary bone neoplasms = more common
 Cortical bone (compact
bone)
• High quantity of bone
tissue and forms the lining
of bones
• Provide rigidity &
structural soundness
 Cancellous bone (spongy
bone)
• Present at the end of long
bones that forms the joint
and covered by hyaline
cartilage
Classification of bone tumor
 Bone tissue consists of
 Cartilaginous tissue
 Ostoid
 Fibrous
 Bone marrow element
 Neoplasm of the bone
 Primary bone tumor
• Benign
• malignant
 Secondary bone tumor
 Primary bone neoplasms are classified based on
 Cell type
 Products of the proliferating cells
Cell type Benign Malignant
Osseous Ostoid osteoma Osteosarcoma
Osteoblastoma
Osteoma
Cartilagenous Chondroma chondrosarcoma
Chondroblastoma
CMF
Osteochondroma
Fibrous NOF Fibrosarcoma
Fibrous dysplasia MFH
Myelogenous Eosinophilic Ewing’s
granuloma sarcoma
Multiple
myeloma
Vascular hemangioma Angiosarcoma
Unknown Simple bone cyst Malignant GCT
GCT Chordoma
Adamantinoma
Diagnosis
Diagnosis of bone tumor based on
1. Clinical examination
2. Imaging
3. Lab. Investigation
4. Biopsy
1. Clinical Examination
History Osteochondroma
 Age
 Useful clue
 Pain
 Common feature
 Causes of pain
 Stretching
 Central hge
 Pathological #
 Swelling
 Neurologic Sx
• Paresthesia & numbness
• Progressive dysfunction
 Pathological #
 Hx of malignancy
Age in yrs Benign Malignant

0-5 EG Leukemia
UBC

6-18 UBC, CMF ES

ABC, NOF OS
CB,OB

19-40 GCT ES
EG OS

40+ Mets
MM,
CS,Lymphoma
P/E
 Lump
 Size
 Consistency
 Tender
 Mobility
 Site
 Boundary
 Near the joint
 Effusion & LOM
 Spine
 Stiffness
 Scoliosis
 Examine the breast,
thyroid, lung…….
2. Imaging
 Important clues for Dx, Rx and prognosis
 Modalities
 Plain X-ray
 CT
 MRI
 Bone scan
 Ultrasound
 Arteriogaphy
Plain Radiography
Bone Real Estate
 Most useful
 Systematic approach

1. Anatomic site
Simple bone cyst
Chondroblastoma
GCT
Parosteal OS
Adamantinoma
 Non-ossifying fibroma
2. Borders of the lesion

 Growth rate & response

of the bone
 Benign lesion
 Well defined
 Narrow transition
 Malignant lesion
 Poorly delineated or absent
margins
Tal. osteosarcoma
GCT
 Fibrous dysplasia
3. Number of lesions

Is it monostoitic or
polyostoitic?
Examples of polyostoitic
• Olier’s d/se
• Polyostoitic FD
• Mets
• MM
• OS(rare)
Oliers dse
M. myeloma
F. dysplasia
4. Bone destruction CMF

 Reflects the increasing

growth rate
 Three patterns
• Geographic
• Moth-eaten
• Permeative
 Geographic:NOF

Geographic

 Destructive lesion with sharply

defined border
 Implies a less-aggressive,
more slow-growing, benign
process
 Narrow transition zone
 Examples
 Non-ossifying Fibroma
 Chondromyxoid Fibroma
 Eosinophilic Granuloma
 Chondromyxoid Fibroma
Moth eaten Moth eaten: M.myloma

 Areas of destruction with

ragged borders
 Implies more rapid growth
 More likely chance of
malignancy
 Example
 Multiple Myeloma
[Plasmacytoma]
 Metastases
 Lymphoma
 Ewing’s Sarcoma
 Permeative: leukemia
Permeative

 ill-defined lesion with multiple

“worm-holes
 Spreads through marrow space
 Wide transition zone
 Implies an aggressive
malignancy
 Lymphoma,
 Leukemia
 Ewing’s Sarcoma
 Multiple Myeloma
 Osteoblastic:OS
5.Matrix formation

Matrix is internal tissue

of tumor.
 Cartilage matrix
• Calcification
 Ossific matrix
• New bone formation
Cartilaginous:CS
6. Periosteal reaction
Solid periosteal rxn COM

 Indicative of malignancy
 Not pathognmonic
 Benign
 None
 Solid
 Aggressive/malignant
 Lamellated/”Onion-skinning
 Sunburst
 Codman’s Triangle
Sunburst : OS
Codmann’s:ES
Onion-skin:ES
CT & MRI
 Subtle cortical disruption,calcification and
ossification
 Intraosseous & extraosseous extension
 R/ship to the surrounding structure
 Inaccessible area like pelvis & vertebra
 Detecting pulm. Mets.
 Tumor spread
• Within the bone
• Into the nearby joint
• Into the soft tissue
 Blood vessels & the r/ship of the tumor to
the perivascular space
FIBROUS DYSPLASIA CT
GCT
MRI skip lesion
Ostoid osteoma
Bone scan

 Polyostoitic inv’t
 mets
 intraosseous
extension
3. Lab. Investigation
CBC, ESR
Calcium, inorganic
phosphate
VDRL
Alk. Phosphatase
Serum acidic phosphatase
Prostatic specific antigen
Serum protein
electrophoresis
Bence Jones protein
4. Biopsy
• Crucial in the Dx
• Types
 Open biopsy
• Conventional & reliable method
 Closed biopsy
• Tissue specimen tru a needle
• Multiple sample can be obtained from
same puncture
• Cannot be done in osteosclerotic tumor
Staging of bone tumor
 Enneking (1980)- surgical staging, then adopted by
American joint committee for cancer in 1997
 Based on three factors
Histological grade(G)
• G-0 benign & well differentiated
• G-1 lesion with low grade malignancy
• G-2 lesion with high grade malignancy
Anatomical location(T)
• T-0 intracapsular
• T-1 intracompartmental
• T-2 extracompartmental
Presence of distant metastasis(M)
• M-0 no metastasis
• M-1 distant metastasis
Stage Grade Site Metastas
is
IA Low (G1) Intracompartmental(T None
1)
IB Low (G1) Extracompartmental(T None
2)
IIA High(G2) Intracompartmenta None
l(T1)
IIB High (G2) Extracompartmenta None
l(T2)
IIIA G1or G2 Intracompartmental(T Yes
1)
IIIB G1 or G2 Extracompartmental(T Yes
2
Principles of Management
Multidisplinary
• Orthopedic surgeon
• Oncologist
• Radiologist
• Pathologist
• Prosthetic designer
• Rehabilitation therapist
Modalities of therapy
• Surgery
• Chemotherapy
• Radiotherapy
Asymptomatic benign lesions
Conservative
Excision/ curettage
Symptomatic benign lesions
Biopsy
Excision/ curettage
Suspected malignant lesions
Admit the pt
Confirm the Dx and staging
Treatment
• Surgical
• Adjuvant therapy
Surgical treatment
Tumor excision
Intralesional excision
Includes excision &
curettage
Passes through
pseudocapsul
Incomplete
Macroscopic tumor
remain &
contaminated
operative field
 Applicable for benign
lesion with low risk of
recurrence
Marginal excision
 Entire tumor is
removed in a single
piece
 Goes beyond the
tumor-plane of
dissection through
reactive zone
 Leaves mac. Lesion
 High risk of recurrence
in malignant lesion up
to 50%
 Suitable for benign
lesion
Wide Excision
 En block resection
which includes
• The entire tumor
• The reactive zone
• A cuff of normal
tissue
 Dissection – through
normal tissue
 It can be done
• Stage IA lesion- risk
of recurrence 10%
• Stage IIA lesion
along with
chemotherapy
Radical Excision

The entire compartment is removed en

bloc with out exposing the lesion
Plane of dissection is beyond the limiting
fascial and bony borders
Can be done for Stage IIB
Limb salvage surgery

Developed in early 1970s

Successful surgery depends on
• Accurate staging
• Accurate Dx – biopsy
• Good surgical technique
• Pre & postoperative chemotherapy
Phases of operation

1. Resection of the
tumor
 Avoid recurrence
 Decide how much
tissue to be
removed
2. Skeletal Reconstruction prosthesis

 The skeletal defects are

reconstructed by diff.
Modalities
• Bone graft-
vascularized or non
vascularized
• Endoprosthesis
• Allograft-prosthetic
composite
• Grafting &
arthrodesis
Allograft prosthesis
composite
Grafting & arthrodesis
3. ST & muscle transfer

 To cover &
close resected
site
 To restore
motor power
St & muscle
reconstruction
Contraindication to limb salvage procedure

Major NV inv’t
Pathologic #
Inappropriate biopsy site
Infection
Skeletal immaturity
Extensive muscle inv’t
Amputation

Significant number of still require

amputation
Indication
• High grade tumors
• Limb salvage procedure is not good
option
Chemotherapy
Multidrug chemotherapy
• Neoadjuvant
• Adjuvant
Effective for sensitive tumor
• Osteosarcoma
• MFH
• Ewing’s sarcoma
• Multiple myeloma
uses
• Reduce the tumor size
• Prevent metastatic seeding
• Improves chances of survival
Neoadjuvant chemotherpy
 Uses
• Facilitates limb sparing surgery by necrosis the
tumor
• Treatment of micrometastasis
• Powerful prognostic factor
 Protocol
• Cisplatin
• Doxorubicin
• methotrexate
 Rx started 8-12 wks preoperatively
 Assessment of the response
• Clinical
• Radiologic
• Histopathologic
 Degree of cellularity
 Necrosis of the resected specimen
Radiotherapy

Used to destroy radiosensitive

tumors or as adjuvant before
operation
• Ewing’s sarcoma
• Pnet
Palliative
Tumor in inaccessible area – axial
Benign bone tumor
Osteochondroma
Definition

OC is a cartilage capped bony

projection arising on external surface
of bone containing a marrow cavity.
Continuous with the underlying bone.
Developmental abn.
May occur as solitary or multiple.
Epidemiology

 Commonest benign tumor-

45%
 Accounts 10-15% of
primary bone t.
 Age: majority of cases
occur in the first 3
decades.
 Sex: M> F
 Osteochondroma

Sites

 Most common:
metaphyseal
• Distal femur
• Upper humerus
• Prox. Tibia & fibula
Clinical feature
 Assymptomatic- incidental
 Symptomatic
 Swelling, hard & painless
 Pain
• Nerve impingement
• Bursa
• Infarction
• Fracture of the stalk
 Deformity
 Limb length discrepancy
 Limitation of motion-
juxtaarticular
 Neurologic manifestation- spinal
lesion
Imaging
Osteochondroma
Plain X-ray
 Pedunculated or
sessile
 Metaphyseal
 Pedunculated
 Grows away from
the epiphysis
 Stalk continuous
with cortex
Sessile OC
Osteochondroma

Flat, plateau like

 protuberance well
demarcated outline
CT & MRI
 Shows continuity of
marrow space
 Thickness of
cartilaginous cap
 Evaluate surrounding
soft tissue
Treatment
 Asymptomatic OC
None
 Surgical removal symptomatic OC
Indication
• Pain
• Nerve irritation
• Continued growth
• Pseudoaneurysm
• Malignant change
Extracapsular marginal excision
• Include the cap,perichondrium, and deep
bony base
• Recurrence = 5%
alignant change suspected

 Adequate imaging studies

 Biopsy
 Treat the pt as conventional CS
 Wide excision

Prognosis
 Risk of malignant change~ 0.2% in solitary OC, 1-
3% in multiple OC
 Sarcomatous change = low grade CS
 Evidences
Cartilage cap >1cm in adult, >2-3cm in children
Cap diameter >8cm
Soft tissue mass in CT & MRI
Fluffy outline
Chondroma

Background
Chondromas are benign
cartilaginous tumor.
Two types
Enchondroma – originates within
medullary cavity
Periosteal Chondroma- originates in
the periosteum and erodes the cortex.
Enchondroma
Solitary or multiple
Epidemiology
• 10-25% all benign
tumors
• Age ranges 5-80yrs
 Majority 20-40
• sex: equal
 Enchondroma

sites
>50% in hands &
feet
Long bones
• Femur
• Humerus
Clinical feature
Enchondroma

Assymptomatic
in most
Swelling with/
without pain
Pathological #
Plain X-ray

Enchondroma
 Central,radiolucent
at the meta-
diaphyseal area
 Flecks of
calcification
 Narrow zone of
transition and
sclerotic borders
Enchondroma
Treatment
Observation
Assymptomatic enchondroma
• Serial radiography & CT
• Tumor size & cortical destruction
Surgery
Curettage & grafting
• Incr. in size
• Pathological #
• Sarcomatous tumor
En bloc excision
Periosteal chondroma

• <2% of all
chondroma
• Long bones & small
tubular bones
Chondromyxoid fibroma

Definition

CMF is a rare benign tumor xized by

lobules spindle cell with myxoid or
chondroid matrix.
Epidemiology
CMF
Rare tumor
 <1% of bone tumor
 <2% of benign
bone tumor
Age : 75% 10-
30yrs
Sex: M=F
Sites of inv’t
CMF

Any bone can be

affected
75% in lower limb
Proximal tibia
Distal femur
Ilium
metatarsal
Clinical feature
Pain
Most common feature
Mild, long standing
Swelling
Infrequent
Hands & feet
Pathological #
Plain X-ray

 Metaphyseal
 Eccentric
 Round,
radiolucent
 Well defined
margin with
surrounding
sclerosis.
Treatment
Surgical Rx
Curretage & grafting
 Recurrence 15%
Extracapsular marginal excision
 No recurrence

Prognosis
Malignant change : extremely rare
Chondroblastoma

Definition

Chondroblastoma is a benign
cartilage forming tumor usually
arising in the epiphysis of skeletally
immature patients.
Epidemiology

Rare accounting
<1% of all
tumor
Age: peak 10-
20yrs
Sex: M>F
Sites

Arise from epiphysis

of long bone(75%)
Distal & proximal
femur
Proximal tibia
Proximal humerus
Other sites
Talus
Calcaneous
Acetabulum &
ilium
Clinical feature
Localized pain-majority of pts
• Mild /severe & sharp
• Several months
Swelling – less common
Joint stiffness & LOM
Joint effusion -rare
Imaging Chondroblastoma

Plain X-ray
 Well defined, oval,
radiolucent lesion in the
epiphysis with a thin
rim of sclerosis and
cortical expansion.
 25% stippled
calcification
 Periosteal rxn may be
present
chondroblastoma
CT & MRI
Calcification
proximity of tumor
to the epiphysis &
articular surface
Host response to the
lesion
Treatment
Surgery
Curettage & grafting
Recurrence –15%
En bloc excision
• Pelvis lesion
Marginal excision
• Adults
Adjuvant
Phenol & liquid nitrogen
Prognosis
 Recurrence 15%
 Pul. Mets is documented but rare and non
progressive
Ostoid osteoma
Definition

OO is a benign bone forming lesion

called nidus, surrounded by a
dense reactive zone of host bone.
Epidemiology

Accounts 10% of
benign bone tumor
2-3% of all primary
bone tumor
Age
 Children &
adolescent
 Peak 5-25yrs (85%)
Sex : M>F
Sites Ostoid osteoma

Located
• Cortex
• Intramedullary
cavity
• Periosteum
Any bone can be
affected
• Tibia & femur
• Spine- post.
element
Clinical feature
Pain (80%)
• Commonest
• Worse at night
• Relieved by ASA
Limping
Swelling
Scoliosis –spine inv’t
Leg length discrepancy
Imaging Ostoid osteoma

Plain X-ray
Dx can be made
with radiography
alone
Diphysis of long
bone
Lytic nidus
surrounded by a
sclerotic margin
Size of nidus –
varies upto 2.5cm
CT & MRI
 Display the nidus
& sclerosis
 In difficult area
like spine
Treatment
Medical
 NSAIDs
• Relieves Sx
Surgical
 En bloc excision of the nidus
 IntraOP localization of the nidus
Percutaneous radiofrequency coagulation
 Best for spine
 CT guidance
Prognosis
 excellent
 Recurrence rare
Osteoblastoma

Definition

OB is a rare benign bone forming

tumor which forms woven bone,
which are bordered by prominent
osteoblasts.
Epidemiology

Rare –1% of
primary bone
tumor
Age
 Range 5-70yrs
 Peak 10-35yrs
Sex : M>F
Sites 0steoblastoma

Post. Segment of
the spine (40-50%)
Appendicular bone
 Prox. Femur
 Distal femur
 Prox. Tibia
 Tarsal bones- talus,
Calcaneous
Clinical feature

Pain
 most common
 Less severe
 Unpredictable response to ASA
Swelling and atrophy
Scoliosis
LOM of the spine
Neurologic manifn -
uncommon
Imaging Osteoblastoma

Plain X-ray
 Radiolucency
• Larger than OO, 2-
10cm
• Irregular
 Less reactive bone
than OO
 Intact periosteum
 No soft tissue mass
CT & MRI

Determine extent of
mineralization &
sclerosis
Anatomic
relationship
Treatment
Surgery
Intracapsular resection
• Recurrence 20%
En bloc resection
• No recurrence
Radiation
Rarely indicated
If surgical removal is not possible
Prognosis : excellent
Non ossifying fibroma

Definition

NOF is the commonest benign lesion

of the bone in which a nest of
fibrous tissue appears in the bone
persist for years before ossifying.
Epidemiology

Incidence: 20% of
benign bone t.
Age
• NOF peak 10-20
yrs
Sex M>F
Sites

Metaphysis of the
bone
• Distal femur
• Prox. tibia
• Distal tibia
Clinical feature
NOF

 Usually assymptomatic
– incidental
 Pathological # in 20%
Plain X-ray NOF

 Radiolucent area
surrounded by thin ,well
defined,margin of dense
bone,
 Metaphyseal, eccentric
NOF
Treatment
Conservative Mx
Spontaneous resolution
Cast splint – in pathological #
Surgical Mx
Curettage & grafting
 Lager lesion (50% of diameter)
weakening the bone
 Recurrence unusual
Fibrous
dysplasia

Definition

FD is a benign medullary fibro-

osseous lesion which may involve
one or multiple bone.
– A dev’tal abnormality than a true
neoplasm
Epidemiology

Incidence:
• 25% of all benign
bone t.
• 7% of all bone tumor
Age :10 – 30 yrs
Sex :F>M
Sites
 In women
• long bones are more affected
 In men
• ribs & skull are favored
 Monostoitic
• 35% skull
• 35% femur & tibia
• 20% ribs
 Polyostoitic
• Femur
• Pelvis
• tibia
Clinical feature
Three clinical
syndrome
Monostoitic FD
• 2-3 decades
• Confined to one
extremity
• Sx are related
to deformity
and
pathological #
• Cranial lesion
=>progressive
visual or
hearing loss
Polyostoitic FD

Younger age
Presentation
• Pain
• Bony
enlargement
• Deformity
• Pathological #
MacCune Albright
syndrome
Polyostoitic dse
Skin pigmentation
• Café au lait spot
with serrated
borders (Coasts of
Maine)
Precocious puberty
Unilateral or
widespread
Plain X-ray

Lucent or “ground
glass” appearance,
thinning of cortex
Sclerotic margin
with no matrix
 No periosteal rxn
The bone may be
enlarged or
deformed
Management

Conservative Mx
Observation
• Asymptomatic pt
• < 18yrs of age
• Monostoitic FD
Surgical Mx

 Aim
• Maintain strength
& integrity of the
bone
• Correct deformity
 Modalities
• Curretage &
grafting
• Wide excision
• Internal fixation
Unicameral bone cyst

Definition
UBC is an intramedullary ,usually
unilocular bone cyst filled with
serous or serosanginous fluid.
Etiology : unknown
Epidemiology

20% 0f benign
tumors
Age
• 85% in the first
two decades = 5-
15yrs
Sex : M>F
Sites

Long bones
• Prox. Humerus (40-
60%)
• Prox. femur
• Prox. Tibia
Pelvis & Calcaneous
Clinical
feature
Pathological # -
frequent
Pain - rare
Swelling - rare
Imaging
Plain X-ray
 Metadiaphyseal,
central, radiolucent
lesion, well
demarcated
 Thinned cortex but
intact
 Partial /complete
septation s of cavity
CT & MRI

 Fluid content
 Distinguish from
ABC, GCT, FD
Management
Conservative Mx
No treatment
• Asymptomatic cyst in older children
Aspiration and injection of steroid
• Active cyst in young children
• Methyl prednisolone 80 –120mg
Surgical Mx
Indication
• Non responsive to steroid
• Pathological #
• Growing cyst
Curettage & grafting
• Prognosis
– recurrence 10-20%
Aneurysmal bone cyst

Definition
ABC is a benign tumor like
condition of unknown origin
composed of blood filled spaces,
separated by septa.
ABC can arise
• De novo =>primary ABC (70%)
• Secondarily from other tumor
=>secondary ABC(30%)
Epidemiology

 Rare
• 1.5% of primary
bone tumor
 Age
• all age
• Most common in
first two decades
 Sex: equal
Sites

Any bone can be

affected
Metaphysis of long
bones
• Femur
• Tibia
• Humerus
Vertebral bone
Clinical feature

Pain & swelling- most common

Neurologic symptoms - vertebral
inv’t
Imaging

Plain X-ray
 Metaphysis,
occasionally
epiphysis
 Eccentric,lytic,
expansile lesion with
thinning of cortex
 Well defined,
trabeculated
CT & MRI

 Internal septa & fluid

fluid level
 Evidence of the
underlying primary
lesion in secondary
ABC
Treatment

Surgery

Curettage & grafting

• Recurrence 20-70%
Marginal excision
Wide excision- in rib
GCT
Definition

GCT is a benign, locally aggressive

neoplasm which is composed of
neoplastic ovoid mononuclear cells
interspersed with osteoclast giant
cell.
• Appears in mature bone
• Unknown etiology
Epidemiology

4-5% of all primary

bone tumor
20% of benign bone
tumor
Age
• Peak age 20-45 yrs
• Rare in immature bone
Sex : F>M
Sites
Ends of long
bones
Distal femur
Prox. Tibia
Distal radius
Prox. humerus
Clinical feature

Pain
Swelling
LOM
Pathological # 5-10%
Imagin
g
Plain X-ray
 Subarticular,
eccentric,expanding
zone of radiolucency
 Well delineated with
irregular endosteal
margin
 Trabeculated with
soap bubble
appearance
 No Periosteal rxn
 CXR
• Pul. Metastasis 1-2 %
• 25% die from pul.
mets
CT & MRI

Accurate cortical
thinning
Joint & soft tissue
inv’t
Management
Establish firm Dx
Aggressive nature
High potential for local recurrence
Rx modalities
Curettage alone
• Recurrence rate 50%
En bloc excision
• Expendable bone
Curettage with adjuvant therapy

Eliminate microscopic dse

Cryotherapy
 Liquid nitrogen
 1-2cm osteonecrosis
Phenol
 1-2mm osteonecrosis
PMMA
 2-3mm osteonecrosis
Malignant bone tumor
Metastatic bone tumor

Definition
It is a malignant tumor involving the
bone which has originated from distant
site.
• Commonest cause of bone
destruction in adults
• Third common site after lung and
liver
Sites

Persistent red marrow

vertebra, sternum, ribs, pelvis,
skull, shoulder girdle
• Axial skeleton - 44.8%
• Appendicular - 28.8%
• Multiple bone - 26.9%
Rare distal to the wrist and
ankle
• Clinical feature
 Bone pain
 Swelling
 Fracture
 neurological Sx
• Paresthesia & weakness
• Bladder & bowel dysfunction
• Investigation
 ESR
 CBC
 Alk. Phosphatase
 Acidic phosphatase
 Prostatic specific antigen
Plain X-
ray
Osteolytic lesion arising
in the medulla and
extend in all direction.
Osteoblastic
No periosteal rxn
ST extension -
uncommon
hepatoma
Breast
Multiple and
several cm in
diameter
 Osteolytic – 80%
 Oseoblastic - 10%
 Mixed – 10%
Prostate

Commonest in
male
Osteoblastic
Spine & pelvis
Kidney

 Solitary
 Lumbar spine &
pelvis
 Expansile
Lung

2nd most
common in men
Osteolytic
Small bones of
the hand and
feet
Treatment
Non operative Rx
 Irradiation and protected wt bearing
 Hormonal & chemotherapy
Operative Rx
 Rigid fixation and full wt bearing
 Devices: IMN, Plates, prosthetic
devices
 Wide resection
Osteosarcoma
Background
OS is a malignant tumor of the bone in
which tumor cells form neoplastic
Ostoid or bone or both.
Variant
 Conventional (IM, Classical )
 Surface OS
 Parosteal
 Periosteal
 High grade surface OS
 Talengiectatic OS
 Small cell OS
 Multifocal OS
Conventional OS

Definition
It is a primary intramedullary
high grade malignant tumor in
which the neoplastic cells
produce ostoid.
• A dise of the young
Epidemiology

Most common primary malignant

tumor accounting 19%.
Age
 10-25yrs
 85% develop < 30 yrs
Sex : M>F (2:1)
Sites
 Metaphysis 91%Long
bones
 Distal femur
 Proximal tibia
 Proximal humerus
 Rare distal to the ankle
& wrist
Clinical feature
Pain
 Deep, severe and boring
 Constant & worse at night
Mass
 Hard, fixed, warm, and tender
Pathological # (4%)
P/E
 LOM
 Edema
 bruit
Imaging
Plain film
Metaphyseal
Variable combination
of bone destruction
and formation
 25% lytic
 35% sclerotic
 40% mixed
Sun ray specules
Codeman’s triangle
Soft tissue extension
CT & MRI

Delineate intra-
and extra-osseous
extent of the
tumor
Distant metastasis
Management

Modality of treatment
surgery
Adjuvant measures
 Chemotherapy
 Radiotherapy
Chemotherapy
1970s  5yrs survival rate of 45% -
60%.
Component of OS treatment protocols
Effective agent
 Methotrexate
 Cisplatin
 Doxorubucin
 Vincristin
 Isosfamide
Chemotherapy
 Neoadjuvant
 adjuvant
Neoadjuvant chemotherapy
Reduction of tumor size and pain
Assessment
Clinical & radiological
• Reduction of ST mass, ossification and # healing
Histological
• Extent of tumor necrosis
Regimen
Rosen 1982
• High dose methotrexate, Cyclophosphamide,
bleomycin and actinomycin D given for 9-12wks
Adjuvant chemotherapy
Tumor assessment
 Good >90% necrosis
 Poor <90% necrosis
Pt’s with good response - the same
regimen postOP
Pt’s with poor response – replace high
dose methotrexate with cisplatin or
doxorubucin postOP.
Survival
 5 yr disease free = 60-90%
 Long term survival (5yrs) =50-70%
Surgery
Limb salvage surgery
Adequate tumor removal
Reconstruction
85% of OS
Amputation
Indication
 Large lesion with NV inv’t
 Path.# with contamination
 Lesions in a very young
 Distal portion of extremity
Level = staging of tumor and its extent
 10 cm or one joint above
Radiation

Less often used

Palliative of local pain
Surgically inaccessible lesion
Painful met.deposits
Prognosis

Untreated = 95% die in 2 yrs

10% macro- and 90% micrometastsis at
presentation
Factors
 Age
 Size of primary tumor
 Location
 Type
 Stage
 Response to chemo.
 >16 mets deposits
Parosteal OS

4% of all OS
Peak – 30-50 yrs
70 % in distal femur
X-ray
 well circumscribed
mass
 cleavage line
• Rx – wide resection
Periosteal OS

 2% of all OS
 Peak 10-30 yrs
 Metadiaphyseal
region of long bone
 X-ray
 subperiosteal new
bone formation
 Rx – wide excision
Telangiectatic OS

 4% of all OS
 2nd decade of life
 Metadiaphyseal area
of long bone
 C/f – pain, swelling,
and path. #
 X-ray often Lytic
 Rx – chemo +
surgery
Chondrosarcoma
Background
CS is a malignant tumor of cartilage
differention.
Variant
 Central
 Secondary
 Juxtacortical
 Mesenchymal
 Clear cell
 dedifferentiated
Conventional CS
Definition
Conventional CS is a
variant CS arising
centrally in a previously
normal bone.
Epidemiology
 20% of all malignant
bone tumors.
 90% of all CS.
 Age
 Tumors of adulthood
 Peak 5th to 7th decades
 Sex M>F
Sites

Pelvis
Proximal femur
Proximal humerus
Distal femur
Ribs
Clinical features

Local swelling
Pain
Imaging
Plain film
 Metaphysis or
diaphysis
 Cortical destruction
with ill-defined
margin
 ST mass
 Calcification
 Periosteal rxn
minimal or absent
CT & MRI
Matrix calcification
Extent of the tumor
and ST extension
Management

Surgery
Limb salvage
Amputation
Ewing’s sarcoma

Definition
Ewing’s sarcoma is sarcoma of the
bone which arises from the
medullary cavity.
Within the group of small round blue
cell tumor.
Epidemiology

 6-8% of primary
malignant bone
tumor
 Age
 Common in
children
 Second decade of
life
 80% < 20 yrs of
age
 Sex M>F
 Sites

Diaphysis/metadiaphy
sis
Pelvis & ribs
Clinical feature
Pain
 Intermittent & gradual
Mass
Systemic Sx
 Fever, malaise, wt loss, and weakness
Investigation
Anemia
Leukocytosis
Elevated ESR
Imaging
Plain X-ray
 Moth eaten or
permeative
destruction
 Onion skin type of
periosteal rxn
 Large ill-defined ST
mass
 Pathological # <5%
CT & MRI
 Delineate the extent of
ST extension
Management

Multidrug chemotherapy
 Vincristine, Cyclophosphamide,
Actinomycin D, Doxorubucin
Radiotherapy
Surgery
 Resection of expendable bone
 Limb salvage
 Amputation
Prognosis
Favorable prognostic factors
 < 10yrs of age
 Distal extremity
 Tumor size <100ml
 Chemotherapy response
 <10% viable tumor
Unfavorable prognostic factors
 Pelvis
 Tumor size >100ml
 Elevated WBC & ESR
 Chemotherapy response
 >10% viable tumor
Fibrosarcoma

Definition
Fibrosrcoma is a rare
malignant bone tumor
xized by the
proliferation of spindle
cells with no matrix
production.
 4% of malignant
neoplasm of the bone
 Age 20-60 yrs
 both sexes equally
affected
Site
 Femur & tibia

C/F
 Pain
 Swelling
 Pathological # (15%)
Plain X-ray

 Eccentric with
permeative or moth
eaten patterns of
destruction
 No calcification or
ossification
Management

Surgery
 Wide resection
 Limb salvage
 Amputation
Chemotherapy
 Neoadjuvant
Radiation ineffective
 palliative
Prognosis
Five yrs survival rate ~ 45%
Prognostic factors
 Histologic grade
 Age >40 yrs
 Location
 metastasis
References

• WHO, Pathology of MS diseases

• Chapman, orthopedics
• Campbell’s system of orthopedics
• Appley’s system of orthopedics
• Devita cancer principles & practice
of oncology
• Grainger ‘s diagnostics radiology
• Enneking, MS tumor surgery
• Internet
Thank you