NEOPLASM OF BONE AND SOFT TISSUE

Widiyatmiko A.P
Department of Orthopaedic and Traumatology
Fakultas Kedokteran UNIVERSITAS SWADAYA GUNUNG JATI

Outline

Introduction
Classification
Epidemiology
Evaluation
Staging
Principles of management
Selected tumors
Therapeautic advances

Introduction
Forms 0.2% of human tumor burden
Primary malig bone tumors make 1% of
all malignant tumors
Carcinoma commonly metastasize to LN
except BCC
Sarcomas commonly metastasize
hematogenously
Most have male predominance excep
GCT, ABC

Classification
Based on tissue of origin
Bone
Cartilage
Fibrous tissue
Bone marrow
Blood vessels
Mixed
Uncertain origin

Evaluation
History
Physical examination
Investigations; labs, imaging
Biopsy

Analytic approach to evaluation

of the bone neoplasm

Evaluation; history
Prolonged delayed in obtaining
treatment
Age : useful clue
Childhood & adolescence : benign
lession, also : malignant tumors (Ewings
& osteosarcoma)
Chondrosarcoma & Fibrosarcoma 4th
6th decade
Myeloma seldom before 6th decade
Age > 70 : metastasis more common

Clinical Presentation
History
Pain : common complaint
Progressive & unremitting pain sinister
symptom, caused by :
Rapid expansion with stretching of surrounding
tissues, central haemorrhage or degeneration
of tumour
An incipient pathological fracture

Tiny lesion very painful if encapsulated

in dense bone

Clinical Presentation
History
Neurological symptoms (paraesthesia /
numbness) pressure upon or stretching
of peripheral nerve
Progressive dysfunction invasion by an
aggressive tumour
Pathological fracture:
First (& only) clinical sign
Injury was slight
Elderly people with any break (fracture) of midshaft

Clinical Presentation
Examination
Lump : where, discrete / ill-defined,
soft / hard, pulsatile,
tenderness
Swelling : distinguish it from infection
or
haematoma
Near joint : effusion & / limitation of
movt
Spinal lesions : muscle spasm, back
stiffness, painful scoliosis

Evaluation; imaging
Plain radiograph
CT scan
MRI
Radionuclide scanning
PET

Radiography
Information yielded by radiography
includes :
Site of the Lesion
Borders of the lesion/zone of
transition
Type of bone destruction
Periosteal reaction
Matrix of the lesion
Nature and extent of soft tissue

Radiographic features of bone tumors

IMAGING
Question to ask when looking at an xray
(Watt, 1985)
Is the lesion solitary or multiple ?
What type of bone is involved ?
Where is the lesion in the bone ?
Are the margin of the lesion well- or illdefined ?
Is there cortical destruction ?
Is there a bony reaction ?
Is there centre calcified ?

Site of the lesion.

Parosteal
osteosarcoma

Chondroblastom
a

Adamantinoma

Site of the lesion.

Distribution of various
lesions in a long
tubular bone in a
growing skeleton

Distribution of various
lesions in a long tubular
bone after skeletal
maturity

Site of the lesion.

Location of epicenter of lesion usually

determines site of its origin (medullary, cortical,
periosteal, soft tissue, or in the joint)

tribution of various lesions in a vertebra

Malignant lesions are
seen predominantly in
its anterior part (body)

Benign lesions
predominate in its
posterior elements.

Borders of the lesion

determine its growth rate.
sharp sclerotic

sharp lytic

ill-defined.

Borders
of
the
lesion.
B: A wide zone of transition
A: Sclerotic border
typifies a benign
lesion e.g
nonossifying fibroma
in the distal femur.

typifies an aggressive or
malignant lesion e.g
plasmacytoma involving the
pubic bone and
supraacetabular portion of
the right ilium

Type of Bone Destruction

Mechanisms of bone destruction

Direct effect of tumor cells
Incr osteoclastic activity

Cortical bone is destroyed less rapidly than trabecular

bone.
Loss of cortical bone appears earlier on radiography
trabecular bone must be destroyed (about 70% loss of
mineral content) before the loss becomes
radiographically evident

Bone destruction can be described as

geographic (type I) - benign lesions
moth-eaten (type II) and
permeative (type III) - rapidly growing infiltrating
tumors

Patterns of bone destruction.

geographic

moth-eaten

a uniformly affected
area within sharply
defined borders

rapidly growing
infiltrating lesions

giant cell tumor.

myeloma

permeative type
characteristic of
round cell tumors

Ewing sarcoma

Types of periosteal reaction.

An uninterrupted periosteal reaction usually indicates a benign
process, whereas an interrupted reaction indicates a malignant
or aggressive nonmalignant process

Examples of Nonneoplastic and Neoplastic

Processes Categorized by Type of Periosteal
Reaction

Interrupted type of periosteal reaction

sunburst
pattern
-osteosarcom
a

lamellated or
onion-skin
type in ewing
sarcoma

Ewing sarcoma
-lamellated type

Codman
triangle
(arrow)

Types of matrix: osteoblastic

The matrix of a typical osteoblastic lesion is characterized by
the presence of the following features
A. fluffy, cotton-like
densities within the
medullary cavity, e.g
in this case of
osteosarcoma of the
distal femur

B. presence of the
wisps of tumor-bone
formation, like in this
case of osteosarcoma
of the sacrum

C. by the presence of
a solid sclerotic
mass, such as in
parosteal
osteosarcoma

Types of matrix: chondroid matrix

A: Schematic representation of various
appearances of chondroid matrix calcifications.

B: Enchondroma
displays a typical
chondroid matrix

C: Chondrosarcoma
with characteristic
chondroid matrix

OTHER IMAGING
Radionuclide scanning (99mTcHDP)
Shows non-specific reactive changes
in bone
Helpful in revealing the site of small
tumour
Skeletal scintigraphy detecting
skip lesions / silent secondary
deposits

OTHER IMAGING
CT-Scan
Excelent method cortical erosion /
#
Shows intra- & extra-osseos
extension of tumour & relationship to
its surroundings
Also reveal suspected lesions in
accesible sites (spine, pelvis)
Reliable method of detecting
pulmonary metastases

OTHER IMAGING
MRI
Its greatest value is in the assessment of
tumour spread :
Within the bone
Into nearby joint
Into soft tissues

Blood vessels & tumour relationship to

perivascular space well defined
Best method for evaluating soft tissue
tumours

Benign Versus Malignant

Nature
clusters of features that can be gathered from
radiographs can help in favoring one designation over
the other .
Benign lesions usually have

well-defined sclerotic borders

exhibit a geographic type of bone destruction
the periosteal reaction is solid and uninterrupted, and
there is no soft tissue mass.

Malignant tumors often

exhibit poorly defined borders with a wide zone of transition;
bone destruction appears in a moth-eaten or permeative
pattern, and
the periosteum shows an interrupted, sunburst, or onion-skin
reaction with an adjacent soft tissue mass.

NB-benign lesions may also exhibit aggressive features

Staging of bone tumors

Benign tumors (Enneking staging of benign
tumors)
Stage 1 - latent
Stage 2 - active
Stage 3 - aggressive

Malignant tumors
TNM staging
AJCC staging system
Musculoskeletal tumor society staging
system(enneking)
Surgical staging

Note
Benign tumors - classified using Arabic numerals(1,2,3)
Malignant tumors - classified using roman
numerals(I,II,III)

William F. Enneking M.D

Enneking classification
systems
Enneking classification of benign tumors
Latent, active, aggressive
Enneking surgical staging of malignant
tumors
Enneking classification of local
procedures
Intracapsular, marginal, extended,
radical
Enneking classification of amputations
Intracapsular, marginal, extended,

nneking classification of local procedures

Enneking classification of amputations

Enneking staging of benign tumors

Stage 1; Latent
Well defined margin
Grows slowly and then stops
Heals spontaneously eg osteoid osteoma
Neglible recurrence after intracapsular resection
Stage 2; Active
Progressive growth limited by natural barriers
Well defined margin but may expand thinning cortex e.g
ABC
Negligible recurrence after marginal excision
Rx marginal resection
Stage 3; aggressive
Growth not limited by natural barriers e.g GCT
Mets present in 5% of these pts
Have high recurrence after intracapsular or marginal
resection
Extended resection preferred

Enneking surgical Staging of malignant

tumors
Incorporates
degree of differentiation
Low grade(stage I) or
High grade(stage II)
Local extent of tumor
Intracompartmental - A
Extracompartmental - B
distant spread
metastasis

Enneking surgical Staging of

malignant tumors

AJCC staging for bone

sarcomas
Based on
Tumor grade
Low grade(I)
High grade(II)

Tumor size
<8cm -A
>8cm -B

Presence and location of mets

Skip mets -III
Pulm mets -IVA
Non-pulm mets -IVB

Bone biopsy
Options
Needle biopsy
90% accuracy at determining malignancy
Accuracy at determining specific tumor much lower
Absence of malignant cells less re-assuring than incisional
biopsy

Core biopsy
Provides accurate diagnosis in 90% of cases
incisional

biopsy

Primary resection instead of biopsy can be done in;

Small(<3cm) subc mass- marginally resected if likely
malignant
Characteristic radiographic appearance of benign lesion
Painful lesion in an expendable bone e.g prox fibula,
distal ulna

Tumour Biopsy Principles

1
1.Biopsy done only after evaluation & imaging is complete.
determine xteristics and local extent of the tumor and mets
Staging helps determine the exact anatomic approach to
tumor
Biopsy superimposes radiologic changes at the biopsy site,
and there4 can alter the interpretation of the imaging studies.
2. Place small incisions whenever possible- skin & capsule
3. The biopsy track be considered contaminated with tumor
cells.
Track excised en bloc with the tumor subsequently.
4. The surgeon should be familiar with incisions for limb
salvage surgery, and also with standard and nonstandard
amputation flaps.

Tumour Biopsy Principles

2
5. If a tourniquet is used;
The limb is elevated before inflation
Avoid exsanguination by compression.

6. contaminate as little tissue as possible.

Avoid transverse incisions
The deep incision should go thru single muscle
compartment (muscle belly) rather than through
an intermuscular plane.
Major neurovascular structures should be
avoided.
Care should be taken not to contaminate flaps.
Minimal retraction should be utilized to limit
soft tissue contamination.

Example of poorly performed biopsy

Transverse incisions should not be used

Tumour Biopsy Principles

3
7. If possible soft tissue extension of a bone lesion should
be sampled
8. If a hole must be made in the bone, it should be round or
longitudinally oval to minimize stress concentration and
prevent a subsequent fracture.
A fracture may preclude a subsequent limb salvage
surgery.
PMMA is plugged into the hole to contain a hematoma
- minimal.

9. Biopsy should be taken from the periphery of the

lesion, which contains the most viable tissue.
Biopsy material may be sent for M/C/S if in doubt
regarding infection

If hole must be made in bone during biopsy,

defect should be round to minimize stress
concentration, which could lead to
pathological fracture

Examples of poorly performed biopsies

Biopsy resulted in irregular defect in
bone, which led to pathological
fracture

Tumour Biopsy Principles

4
10. A frozen section should be sent intraop to
ensure that diagnostic tissue has been
obtained.
If a tourniquet has been used it should be deflated
and meticulous haemostasis ensured before closure.

11. Drains should not be used routinely.

If a drain is used, it should exit in line with the
incision.
The wound should be closed tightly in layers.

12. operating surgeon should accompany

specimen to pathologist if feasible
Discuss with the pathologist about clinical findings,
imaging, intraop findings and the specimen

Example of poorly performed biopsy

Drain site was not
placed in line with
incision

DIFFERENTIAL
DIAGNOSIS
Soft tissue haematoma

Large, clotted subperiost / soft tissue haematoma

painful lump
Clues : history & rapid onset of symptoms

Myositis ossificans

Following an injury tender swelling in the vicinity of

joint
X ray : fluffy density in soft tissue adjacent to bone
become less painful & new bone better define

Stress #

A young adult, w/ localized pain near large joint

X ray : dubious area of cortical destruction
overlying periosteal new bone
Healing callus histologically may resemble
osteosarcoma

DIFFERENTIAL
DIAGNOSIS
Tendon avulsion injuries
Children & adolescent w/ vigorous sports
Osgood-Schlatter disease, but lesions at less familiar
sites escape immediate recognition

Bone infection
Osteomyelitis pain & swelling near large joint
X ray: destruction of mhysis w/ periosteal new bone
Tissue bacteriological & histological exam

Gout
Large tophus painful swelling at one of bone ends
X ray: large, poorly defined excavation, may need biopsy

Principles of
management
Multidisciplinary team approach
Benign asymptomatic tumors
If certain observe
If in doubt biopsy
Benign symptomatic or enlarging tumors
Biopsy
Excision/ curretage
Suspected malignant tumors
If primary admit for work-up
Staging
Choices; amputation, limb sparing surgery,
adjuvant therapy

PRINCIPLES OF
MANAGEMENT
Benign, Symptomatic or Enlarging
Tumour
Painful lesion or enlarging tumour
biopsy & confirmation diagnosis
Usually not aggressive local
(marginal) excision or curettage
(cyst)

PRINCIPLES OF
MANAGEMENT
Suspected Malignant Tumour
Primary malignant tumour px admitted
for detailed examination, blood tests,
chest x-ray, & biopsy
Firm diagnosis & staging
Choice of treatment :
Amputation
Limb-sparing operations
Adjuvant therapy

METHODS OF
TREATMENT
Tumour Excision
more aggressive lesion wider
excision
Multi-Agent Chemotherapy
Radiotherapy

Tumour Excision

Intracapsular (intralesional) excision & curettage

incomplete form of tumour ablation; only for benign lesion
w/ low risk of recurrence; or incurable tumour debulking
(relieving local symptoms)

Marginal Excision
beyond tumour, but only just.
if dissection malignant lesion through the reactive zone
risk of recurrence (50%)
Suitable method for benign lesion
Cavity filled w/ bone gaft

Tumour Excision

Wide excision
Dissection carried out well clear of the tumour, through
normal tissue
Appropriate for stage IA (<10% recurrence)
In conjungtion with chemotherapy in stage IIA

Radical resection
entire compartement removed en bloc w/o exposing the
lesion
still possible to spare the limb, but must sacrifice the
surrounding muscle
True radical resection amputating above the
compartment involved
For stage IIB

Tumour Excision

Limb-sparing surgery
Amputation no longer for stage II
Preferred for intra-compartmental lesion wide excision w/ pre &
post-op chemoth.
Short diaphyseal segment vascularized or non-vascularized
bone graft
longer gaps custom-made implants
Osteoarticular large allograft, custom-made prostheses,
allograft-prosthetic composites
growing children extendible implant
Sarcoma of the hip & shoulder difficult
Principles : No skip lesion & limb must be viable & functional
Outcome : massive prosthesis risk of wound breakdown &
infection

Amputation : doubt whether intra or extra-compartment tumor

extension

Tumour Excision

Multi-Agent Chemotherapy
For sensitive tumour reduce the size,
prevent metastatic seeding, improve
survival
Drugs : MTX, Doxorubicin (adriamycin),
cyclophosphamide, vincristine, cisplatin
8 12 weeks pre-op, if not effective
change to different drug for post-op
treatment
Maintenance chemotherapy Another 6
12
months

Radiotherapy
Indication more restricted
Highly sensitive tumour alternative to
amputation, combined w/ adjuvant
chemotherapy
For tumour in inaccessible site, inoperable
because of size or local spread, metastatic
deposits or marrow-cell tumour (myeloma,
malignant lymphoma)
Given in devided dose over 4 weeks up to
total 6000cGy

Benign tumors - not

aggressive

Bone-forming tumors
Osteoid osteoma
Bone island
Cartilage lesions
Chondroma
Osteochondroma

Fibrous lesions
Nonossifying fibroma
Cortical desmoid
Benign fibrous
histiocytoma
Fibrous dysplasia
Osteofibrous dysplasia
Desmoplastic fibroma

Cystic lesions
Unicameral bone cyst
Aneurysmal bone cyst
Intraosseous ganglion cyst
Epidermoid cyst
Fatty tumors
Lipoma
Vascular tumors
Hemangioma
Other nonneoplastic lesions
Paget disease
Brown tumorhyperparathyroidism
Bone infarct
Osteomyelitis

Fibrous Dysplasia
Developmental disorder areas of
trabecular bone are replaced by cellular
fibrous tissue containing flecks of osteoid
& woven bone
Monostotic, monomelic & polyostotic
Large lesion pathologic # & progressive
deformity
Small, single lesion asymptomatic
Large, monostotic pain, pathologic #
Polyostotic childhood / adolescence w/
pain limp, bony erlargment, deformity,
pathologic #

Fibrous Dysplasia
McCune - Albrights Syndrome

Polyostotic disease (unilateral usually)

Skin pigmentation

cafe au lait spots with serrated borders (called "coast of

Maine") that tend to stop abruptly at the midline of the body

Precocious puberty (endocrinopathy)

usually presents earlier, may be unilateral or widespread,
affecting long bones, hands, feet & pelvis
Malignant transformation (chondrosarcoma or
osteosarcoma) is about 4 %;

Treatment : large, painful, tend to # curetted &

grafted
strong tendency to recur MMA cement (very large
lesion)
Large cyst beware of bleeding at operation

Fibrous Dysplasia

X-ray: radiolucent cystic area in

metaphysis or shaft, lucent patches
ground glass appearance,
weight bearing bone bent
(shepherds crook proximal femur)
Scintigraphy : activity

Fibrous lesions
Nonossifying fibroma

Polyostotic Fibrous dyspalsi

Fibrous dysplasia

Shepherds crook
appearance

Osteoid Osteoma
Tiny tumour, regardless its size
symptoms
Mostly at femur / tibia, except skull
Persistent pain relieved by salicylates
Delayed diagnosis limp, muscle
wasting, spinal lesion, muscle spasm,
scoliosis
DD/ bordies abscess biopsy, Ewings
sarcoma, chronic periostitis

Osteoid Osteoma
Pathology : dark-brown / reddish nucleus
surrounded by dense bone,
central area osteoid & bone cells
No risk of malignant
Treatment : complete removal of nidus
excised in the small block of bone,
specimen x-rayed to confirm
weak bone prophylactic internal fixation

Osteoid Osteoma
X-ray : nidus (small radioluscent
area)

Diaphysis : surrounded by dense

sclerosis / cortical thickening
Metaphysis : less cortical thickening
99mTc-HDP scintigraphy : intense,
localized activity

Compact Osteoma
(Ivory Exostosis)

Localized thickening on the outer or inner surface of

compact bone
Adolescent / young adult
Painless, ivory hard lump usually : skull,
subcutaneous
surface of tibia
Inner table skull focal epilepsy
X-ray : sessile plaque of exceedingly dense bone w/
well-circumscribed edge
Parosteal sarcoma have no long history, absence
of pain, smooth outline
Treatment : if not impinges need not be removed,
excision is easier if a margin of normal bones is
taken

Compact Osteoma
(Ivory Exostosis)

Chondroma (Enchondroma)
Persistent of islands of cartilage formed by
endochondral ossification
Asymptomatic (incidental x-ray finding), or after
pathologic #
Commonly : tubular bones of hands & feet
Solitary / multiple or part of generalized dysplasia
Ollier's disease multiple enchondromatosis;
50% ->malignant transformation
Mafucci's disease - associated with multiple
haemangiomata and associated with nearly
100% malignant change somewhere

Chondroma (Enchondroma)
Treatment : if there is enlargement,
pathologic # curettage
High recurrence rate & seeding to nearby
bone & tissue
Malignant change (< 2%)
Signs of malignant change (>30y.o.):
Onset of pain
Enlargement of the lesion
Cortical erosion

Chondroma (Enchondroma)
X-ray : well-defined, centrally placed
radiolucent area at the junction of metaphysis
& diaphysis
Pathognomonic : flecks of calcification w/i
lucent area

Periosteal Chondroma
Rare developmental lesion in the deep layer of
periosteum
Cartilaginous lump bulging from bone soft
tissue alarming the px
Uncalcified not shown on x-ray, surface of bone
irregular / scalopped
Histologically : highly cellular cartilage
Treatment : best marginal excision (taking a
rim of normal bone
recurrent lesion may aggressive but not change
to malignant form

Osteochondroma
(Cartilage-Capped Exostosis)
Commonest tumour of the bone
Developmental lesion, starts as a small
overgrowth of cartilage at the edge of
physeal plate & develops by endochondral
ossification bony protuberance still
capped by cartilage
Commonest sites : fast-growing end of
long bones & iliac crest
Bony growth stop at the end of normal
growth period, any further enlargement
suggestive malignant

Osteochondroma
(Cartilage-Capped Exostosis)
Usually : teenager young adult
Pain, due to overlying bursa /
impingement on soft tissue /
stretching of an adjacent nerve

Osteochondroma
(Cartilage-Capped Exostosis)
Pathology : cap surmounting a narrow base /
pedicle of bone, consist of simple hyaline
cartilage
bony part may be pedunculated, sessile, or cauliflower
malignant transformation (1% solitary, 6%
multiple)
Treatment : should be excised if symptomatic
in adult (urgent) if it become bigger / painful
suggest malignancy (most often: pelvic exostosis)
if histology benign but enlarging after end of
growth period treated as chondrosarcoma

Osteochondroma
(Cartilage-Capped Exostosis)

X-ray (pathognomonic) :
well-defined exostosis emerging from
metaphysis, its base co-extensive w/
the parent bone
look smaller cartilage cap cant
seen on x-ray, but if undergo
cartilage degeneration & calcification
clouds of calcified material

CARTILAGE LESIONS
Osteochondroma

Cystic lesions
Unicameral bone cyst

Aneurysmal
bone cyst

Aggressive benign
tumors
Giant cell tumor
Chondroblastoma
Chondromyxoid fibroma
Osteoblastoma
Langerhans cell histiocytosis

Chondroblastoma
Benign tumour of immature cartilage cells
appear in epiphysis (prox. Humerus,
femur & tibia)
The end of growth period / early adult life
Symptom : constant ache in the joint,
tender spot adjacent bone
Treatment : children risk of physeal
damage, delayed removal of lesion until
end of growth period by marginal
excision / curettage
Incomplete removal high risk of recur
If needed Joint reconstruction

Chondroblastoma
X-ray : well-demarcated
radiolucent area in epiphysis,
articular surface breached
Can extend across physeal
line
sometimes resemble ABC

Chondromyxoid Fibroma
Adolescent / young adult
More common in lower limb
Px seldom complaint, discovered by
accident / after pathologic #
Pathology : contains of mucinous material
& cartilage
Micros : 3 types of tissue
Patches of myxomatous tissue w/ stelate cells
Island of hyaline cartilage
Fibrous tissue (varying degree of maturity)

Osteoblastoma
(giant Osteoid Osteoma)
Similar w/ osteoid osteoma but its larger
more cellular, smtimes more ominous in
appearance
Tend to occur in spine & flat bones
X-ray : well-demarcated lytic lesion w/ small
flecks of ossification, surrounding
sclerosis
Radioisotop scan : hot area
Treatment : Excision & bone grafting
Lesion in vertebral pedicle / floor of
acetabulum incomplete removal
Local recurrence common

Osteoblastoma
(giant Osteoid Osteoma)

The microscopic features

resemble those of osteoid
osteoma. Osteoblasts and
osteoclast-like giant cells rim
interconnected spicules of
osteoid and woven bone. The
intervening fibrous stroma

Aggressive benign tumors

Giant cell tumor

Chondroblastoma

Aggressive benign tumors

Chondromyxoid fibroma

Malignant Tumors of
Bone
Osteosarcoma
Chondrosarcoma
Ewing sarcoma
Chordoma
Adamantinoma
Malignant vascular tumors
Malignant fibrous histiocytoma and
fibrosarcoma
Multiple myeloma and plasmacytoma
Lymphoma
Metastatic carcinoma

Chondrosarcoma
Can occur as primary or secondary
Highest incidence: 4th & 5th decade
Slow growing & present many month
before discovered
Dull ache / gradually enlarging lump
Medullary lesions pathologic #
Slow-growing & metastasize late

Chondrosarcoma
Primary Chondrosarcoma
Usually seen in metaphysis of tubular
bones
X-ray: radiolucent area w/ central flecks of
calcification
Rarely, appear as a globular mass on the
surface of the bone
Peripheral & central (common)
chondrosarcoma

Chondrosarcoma
Secondary chondrosarcoma
Usually arises in cartilage cap of an
exostosis (osteochondroma)
More susceptible: pelvis & scapula
X-ray: bony exostosis, often surmounted
by clouds of patchy calcification
Clearest sign of malignant change
progressive enlargement of
osteochondroma after end of normal bone
growth

Chondrosarcoma
Staging: full staging must be employed
Pathology:
low grade = aggressive benign cartilagenous
High grade = more cellular, plumpness,
hyperchromasia, mitosis
dedifferentiated chondrosarcoma =
assoc w/ area that look more malignant
Treatment : wide excision & prosthetic
replacement
does not respond to radiotherapy or
chemotherapy

Chondrosarcoma

Chondrosarcoma

Osteosarcoma
Classic (intramedullary) form
highly malignant tumour arising w/i
the bone & spread rapidly
periosteum & surrounding soft tissue
Predominantly in children &
adolescent
Most common at long-bone
metaphysis (knee & proximal end
humerus)

Osteosarcoma
Clinical feature
first symptom constant, worse at night,
severity
Lump
rarely pathological #
physic exam : local tenderness (palpable
mass, swollen & inflamed)
Lab : ESR & Alkali Phosphatase

Osteosarcoma

Osteosarcoma
X-rays: appearance variable
hazy osteolytic area alternate w/ unusually
dense osteoblastic area
endosteal margin poorly defined
cortex breached & tumour extend to
adjacent soft tissue
streaks of new bone, radiating outwards
from cortex sunburst effect
reactive new bone form at angles of
periosteal elevation Codmans triangle

Osteosarcoma

Osteosarcoma
Diagnosis can be made based on x-ray
Must exclude : post-traumatic swelling,
infection, stress #, aggressive cystic
lesion
Other imaging studies staging
radioisotope scan skip lesions
CT &MRI tumour extension
Chest x-ray / CT lung metastases (10%)
Biopsy must carefully planned

Osteosarcoma
Pathology :
Macros: tumour destroy & replace normal bone, alternate
area of bone loss & cavitation w/ dense patches abnormal
new bone
extend w/i the medulla & across physeal plate
spread to soft tissue w/ ossification at periosteal margin &
streaks of new bone exrta-osseus

Micros: show considerable variation

spindle cells w/ osteoid matrix
cartilage cells / fibroblastic w/ little or no osteoid
several sample must be examined

Osteosarcoma

Osteosarcoma
Treatment :
prognosis markedly improved because : chemotherapy &
metastatic control
Important : eradicate primary lesion completely
Recurence lesion worse mortality
After diagnosis & staging may be stage IIA or IIB
Preo-op chemotherapy for 8 -12 weeks if no skip lesion,
tumour is resectable wide resection
Prepare for bone-graft or custom-made implants
If tumour necrosis is marked continue w/ post-op
chemotherapy for 6 12 months, if not change
Pulmonary metastases if small & peripheral wedge
resection

Osteosarcoma
Outcome :
After wide resection & chemotherapy
50 60%
Implants function well, but theres
fairly high complication rate (wound
breakdown & infection)
Incidence of local recurence :same as
amputation

Variants of Osteosarcoma
Parosteal osteosarcoma
Periosteal osteosarcoma
Pagets sarcoma

Parosteal osteosarcoma
Low-grade sarcoma on surface of one of tubular
bone (distal femoral or proximal tibial metaphysis
Young adult w/ slowly enlarging mass near bone
end
Pathology:
Macros : hard mass
Micros : consist of well-formed bone w/o any
regular trabecular arrangement
Cellular fibroblastic tissue
a few atypical cell & mitotic figures
more aggressive : dedifferentiated parosteal
osteosarcoma

Parosteal osteosarcoma
X-ray: dense bony
mass on the surface of
bone or encircling it
cortex not eroded,
thin gap remain
between cortex &
tumour
easily mistaken w/
benign lesion, until
recur
low-grade
intracompartemental
tumour stage IA

Parosteal osteosarcoma
Treatment:
Wide excision w/o adjuvant therapy
recurence rate < 10%
dedifferentiated parosteal
osteosarcoma same as
intramedullary sarcoma

Periosteal osteosarcoma
More like classic osteosarcoma, but situated on
the surface of the bone
Occurs in young adult
Local pain & swelling
X-ray: cortex superficial defect, but CT / MRI
shows larger soft-tissue mass
can be like periosteal chondroma performed
biopsy
Pathology: true osteosarcoma w/ section of
prominent cartilagenous element
Treatment : same as classic osteosarcoma

Osteosarcoma

Fibrosarcoma of the Bone

Rare in the bone, arise in preveiously abnormal
bone (a bone infarct, fibrous dysplasia, after
irradiation)
Adult px w/ pain & swelling, may be pathologic #
X-ray:
undistinctive area of bone destruction
CT / MRI : soft tissue extension
Pathology : masses of fibroblastic tissue w/
scattered atypical & mitotic cells, vary from welldiff to highly-undiff

Fibrosarcoma of the Bone

Treatment :
low-grade, well-confined
(stage IA) : wide excision &
prosthetic replacement
high-grade (IIA or IIB)
radical resection or
amputation
If cant local excision w/
radiotherapy

Malignant Fibrous
Histiocytoma
Tends to occur in previously normal bone
Middle-aged adult
X-ray: destructive lesion adjacent to an old
area of medullary infarction
Staging almost invariably spread
beyond the bone
Treatment: wide / radical resection /
amputation & adjuvant chemotherapy
inaccessible lesion local radiotherapy

Malignant Fibrous
Histiocytoma
Pathology: fibrous
tumour,
interweaving
bundles,
histiocytes & giant
cells distinguish
from fibrosarcoma

Ewings Sarcoma

Arise from endothelial cells in bone marrow

Common between the age of 10 20 years
Usually in tubular bone (tibia, fibula & clavicle)
Presents w/ (throbbing) pain & swelling
Generalized illness, pyrexia, warm, tender
swelling, ESR suggest osteomyelitis
Pathology
macros: lobulated & fairly large, may look grey or
red (haemorrhage)
micros: sheet of small dark polyhedral cells w/ no
regular arrangement & no ground substance

Ewings Sarcoma
DD/ bone infection, osteosarcoma,
retculum-cell sarcoma & metastatic
neuroblastoma
Treatment: prognosis poor
Surgery little effect
Radiotherapy overall survival not
enhanced
Chemotherapy 5-year survival = 50%
Best combination pre-op
chemotherapy, wide excision / amputation
or radiotherapy w/ local excision &
chemotherapy for 1 year

Ewings Sarcoma

X-ray: an area of bone destruction (middiaphysis)

new bone formation extend along the shaft
fusiform layer of bone around the lesion
onion-peel effect
also common sunray appearance &
Codmans triangle
CT & MRI large extra-osseous component
Scintigraphy multiple areas of activity

Ewing Sarcoma
may be confused with osteomyelitis
Commonly affects diaphysis with onion
skin appearance

MULTIPLE MYELOMA
(MM)
MM is a malignant tumor of plasma cells
that causes widespread osteolytic bone
damage
Most common primary malignant tumor of
bone (~ 40%)
May affect any bone with haematopoietic
red marrow (spine, skull, ribs, sternum and
pelvis)
Age 50-80 year
M:F = 2:1

MULTIPLE MYELOMA
(MM)
Presentation
bone pain related to the deposits
Pathological fractures
Constitutional symptoms related to anaemia,
thombocytopenia and renal failure
Other symptoms may include cachexia, spinal
cord compression
Amyloidosis in 20%
Bacterial infections are common because of a
lack of normal immunoglobulin production.

MULTIPLE MYELOMA (MM)

Investigations
FBC
normochromic,
normocytic anaemia
ESR
raised
++
(Often
>100mm/hour)
Hypercalcaemia (20-40%)
Monoclonal immunoglobulin
found
on
serum
electrophoresis (90%)
Bence Jones proteins (light
chain
subunits
of
immunoglobulin) present in
urine (50%)

Solitary lesions - 60% 5 year

survival
Multiple lesions 5% , 5 year
survival

MALIGNANT FIBROUS
HISTIOCYTOMA

5% of primary malignant bone tumours

Age more than 30 (often more than 50)
Male > Female
80% are primary bone tumour
20% occur secondary to a pre-existing
condition
Cell of origin controversial-options- histiocytic
cell; fibroblasts; multipotent mesenchymal cell
Can produce the osteolitik lession that spread
to soft tissue

MALIGNANT FIBROUS
HISTIOCYTOMA
Clinically
Present with pain, swelling
15% present with a pathological fracture
May arise in previously abnormal bone eg
Pagets, fibrous dysplasia, long standing
osteomyelitis or irradiated bone
40% occur around the knee
Metastasises to the lung, and other bones via
the blood

MALIGNANT FIBROUS
HISTIOCYTOMA
X-Rays
Usually metaphyseal
around knee
bone often mottled or moth eaten with
extension into soft tissue
Osteolytic lesion may be surrounded by
reactive bone
Destructive appearance radiologically

FIBROSARCOMA
25% have metastasized at presentation and rare
2% primary malignant bone tumors
Male = Female
X-Rays
Osteolytic lesion
Margins can range from well-defined to ragged
and moth-eaten
Periosteal reaction is seen with cortical
destruction
Extension into the soft tissue is common

FIBROSARCOMA
Differential Diagnosis:
Metastatic carcinoma
multiple myeloma
MFH
leiomyosarcoma

SOFT-TISSUE TUMOURS
Benign common
Feature of malignancy :
Pain in previously painless lump
Rapid increase in size
Poor demarcation
Attachment to surroundings structure
Sonogram: discrete echo pattern

Biopsy excisional biopsy when

possible

SOFT-TISSUE TUMOURS
Fatty Tumours

Blood Vessel Tumors

Fibrous Tumours

Nerve Tumours

Lipoma
Liposarcoma

Fibroma
Fibromatosis
Fibrosarcoma

Synovial Tumours

Pigmented Villonodular
Synovitis & Giant-Cell
tumour of tendon
sheath
Synovial Sarcoma
(Malignant Synovioma)

Haemangioma
Glomus Tumour

Neuroma
Neurilemmoma
Neurofibroma
Neurosarcoma
(malignant
Schwannoma)

Muscle Tumours

Rhabdomyoma
Rhabdomyosarcoma

Lipoma

Arise in subcutaneous layer

One of the commonest
Almost anywhere, sometimes multiple
Px age usually > 50 y.o. w/ painless
swelling
Micros: lobules of fat w/ capsule, welldefined edge
Treatment: marginal excision
atypical staging & biopsy

Liposarcoma
Rare
Fatty tumour (esp. in buttock, thigh)
growing & painful
Lump firm, not translucent
CT & MRI extent of tumour
Treatment:
low-grade wide excision
high-grade radical resection

Fibroma
Solitary, benign tumour of fibrous
tissue
Small asymptomatic nodule / lump
Treatment: not essential
(marginal excision adequate)

Fibromatosis
More aggressive than fibroma
Young adult w/ thick cord or plaques
in subcutaneous tissue (limb or
trunk) grow into featureless
masses w/ ill-defined margins
After local excision tend to recur
Can be highly aggressive

Fibromatosis
Wide excision threaten to involve
pelvis / axilla
Micros: vary, from clearly benign cell
to multinucleated cells w/ many
mitosis (suggest malignancy)

Fibrosarcoma
Occur in conective tissue, common in
extremity
An ill-defined, painless mass
considerable size
Diagnosis after biopsy
Local extent MRI
High grade : atypical spindle cell
low grade: difficult to distinguish from
fibromatosis
Treatment: low grade wide excision
high grade WE + pre & post-op
radiotherapy

Pigmented Villonodular Synovitis &

Giant-Cell tumour of tendon sheath
Benign disorders wherever synovial membrane is
found (joint, tendon sheath, bursae)
PVNS
Long-standing boggy swelling of the joint (hip,
knee or ankle)
Adolescent or young adult
X-ray: excavation in juxta-articular bone on either
side of the joint contain clumps of friable
synovial material
Macros: synovium : swollen & hyperplastic, villi,
golden-brown colour

Pigmented Villonodular Synovitis &

Giant-Cell tumour of tendon sheath
Tendon sheath lesion
Mainly in the hand & feet nodular
thickening
X-ray: presure erosion of adjacent
bone surface
Macros: when boggy synovial tissue :
yellow xanthoma of tendon sheath

Rhabdomyoma
Rare, often confuse w/ muscle
rupture
Muscle rupture: sudden, depression
proximal or distal of the lump,
swelling doesnt grow
Early exploration & biopsy
(malignant change)
Treatment: excision

Rhabdomyosarcoma
Occasionally seen in muscles around hip &
shoulder
Young adult w/ ache, enlarging & illdefined lump, move w/ affected muscle
Tend to spread along the fascial plane
Biopsy: clusters of highly abnormal muscle
cells
Treatment :
radical muscle resection (muscle group
excision)
amputation
adjunctive radiotherapy

The end
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