Oncology

Sholahuddin Rhatomy MD
ORTHOPAEDIC
ONCOLOGY
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1. Tumor principle3
2. Tumor work up 5
3. Biopsy tumor 8
4. Treatment principle 11
5. Bone cyst 15
6. Unicameral simple bone 15
7. ABC 18
8. GCT 21
9. Bone tumor index 25
10. Bone producing tumor 26
11. Osteoid osteoma 26
12. Osteoblastoma 28
13. Osteosarcoma 30
14. Parosteal OS 37
15. Periosteal OS 38
16. Pagets sarcoma 39
17. Teleangiectase sarcoma 39
18. Cartilage producing bone tumor 41
19. Osteochondroma 41
20. Enchondroma 44
21. Chondroblastoma 47
22. Chondromyxoid fibroma 48
23. Chondrosarcoma 51
24. Mesenchimal chondrosarcoma 53
25. Fibroue forming bone tumor 54
26. NOF 54
27. Fibrous dysplasia 56
28. MFH 59
29. Fibrosarcoma 61
30. MM 62
31. Limfoma 64
32. Soft bone tumor 66
33. Benign fibrous tumor68
34. Malignant fibrous tumor 72
35. Benign fatty tumor73
36. Malignant fatty tumor 73
37. Benign peripheral nerve tumor74
38. Malignant peripheral nerve tumor 74
39. Benign muscle tumor75
40. Malignant muscle tumor 72
41.
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TUMOUR PRINCIPLES
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Staging Musculoskeletal Neoplasms (Enneking Staging System aka Musculoskeletal Tumour Society
System)
Staging may be useful for

Developing evaluation strategies
Planning Rx
Predicting prognosis
Benign
1
Latent
G0
T0
M0
Active
G0
T0
M0
Aggressive
G0
T1
M0-1
Malignant
Classification based on
1. Histological grade (G)
2. Site (T)
3. Metastases (M)
Enneking staging system was tested retrospectively on 397 cases of bone & soft tissue tumours
Shown that prognosis varied with stage
Aids in assessing prognosis & planning Mx
NB: applies to lesions of connective tissue, not primary lesions of round cell origin (eg.
leukaemias, lymphomas, myeloma or Ewing's)
ENNEKING'S SURGICAL STAGES

STAGE
GRADE
SITE
METASTASES
IA
Low (G1)
Intracompartmental (T1)
None (M0)
IB
Low (G1)
Extracompartmental (T2)
None (M0)
IIA
High (G2)
Intracompartmental (T1)
None (M0)
IIB
High (G2)
Extracompartmental (T2)
None (M0)
III
Low (G1) or High (G2)
Intracompartmental (T1) or Extracompartmental (T2)
Yes (M1)
Grade (biological aggressiveness)
G0: Histologically benign (well differentiated & low cell to matrix ratio)
G1: Low grade malignancy (few mitoses, moderate differentiation & local spread only); low risk of
metasases <10%
G2: High grade malignancy (frequent mitoses, poorly differentiated); high risk of metasases >20%
Features of aggressive tumours
1. Pleomorphism
2. Cellular atypia
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3.
4.
5.
6.
Frequent mitoses
Extensive necrosis
Significant vascularity
Small amounts of immature matrix
Examples
LOW (G1)
HIGH (G2)
Parosteal osteosarcoma
Classic osteosarcoma
Low-grade medullary
osteosarcoma
Radiation osteosarcoma
Paget's osteosarcoma
Secondary chondrosarcoma
Primary chondrosarcoma
Fibrosarcoma
Malignant Fibrous Histiocytoma
Giant cell tumour

Myxoid liposarcoma
Pleomorphic liposarcoma
Neurofibrosarcoma (Shwannoma)
Rhabdomyosarcoma
Synovioma
Clear cell tumour of tendon sheath

Chordoma
Adamantinoma
Site (anatomic setting)
T0: Intracapsular
T1: Intracompartmental (eg. cortical bone, joint capsule or fascia)
T2: Extracompartmental (spreads beyond 'fascial' plane without longitudinal containment)
Sarcoma grows centrifugally & respects anatomical barriers
Intracompartmental
Extracompartmental
Intraosseous
Soft tissue extension
Intra-articular
Deep fascial extension
Intrafascial compartments:
Extrafascial planes/spaces: (neurovascular containing spaces)
ray of hand or foot
mid & hind foot/mid hand
posterior or anterior leg
popliteal fossa
ant, med, post thigh
groin - femoral triangle
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buttocks
intra-pelvic
volar or dorsal forearm
antecubital fossa
anterior or posterior arm
axilla
pericapsular
paraspinal
Metastasis (nodal or blood borne tumour spread)
M0: No evidence of regional or distant metastases

M1: Regional or distant metastases evident
Tumour Workup
Objectives
1. Establish diagnosis
2. Staging
3. Assess general fitnesss
Rx of any potentially malignant bone tumor always begins with
1. Radiographic features
2. Staging
3. Biopsy
Clinical history
o Age, sex, site & past history
o Common presenting features
Pain
Typical deep seated & dull pain, not responsive to NSAIDs & weak
opiods
Occurs at rest, worse at night
Progressive to severe & constant pain
High grade sarcoma: 1-3/12 history of pain
Low grade sarcoma: 6-24/12 history of mild to moderate pain
Mass
# - 5%
Incidental findings
Physical examination
o Examine affected site for
Soft tissue mass
Skin changes
Adenopathy
General musculoskeletal condition
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Metastases suspected
Thyroid
Breasts
Chest
Liver
Kidney
Rectal (prostate & rectal tumours)
Plain radiographs
o Plain X-rays in 2 planes
Formulation of differential diagnosis
o Based on clinical & radiographic features
1. Age
2. Number of bone lesions
3. Anatomic location within bone
4. Effect of lesion on bone
5. Response of bone to lesion
6. Matrix characteristics
7. Soft tissue involvement
Staging
o When malignancy suspected
MRI -> soft tissue extent & association with nerves & vessels
Angiography -> tumour blood supply & relationship to major vessels
CXR
Abdominal ultrasound
CT of lesion & chest
Bone scan -> other sites
Lab tests
o Often nonspecific
o Blood
FBC (leukaemic cells etc)
ESR (often mildly elevated except for multiple myeloma & Ewing's)
Biochemistry (Ca++, PO4, liver enzymes & Alkaline Phosphatase) -> mets
Acid Phosphatase (prostate & increased with metastatic deposits)
Thyroid function tests
PSA
Serum protein electrophoresis (myeloma)
o Urine
Urinalysis
Urine Bence-Jones (myeloma)
Biopsy
o
Radiological Investigations
Plain X-ray
Diagnosis in 85% of bone sarcoma

Points to note
Number of bone lesions
Anatomic location of lesion
Effect of lesion on bone
Response of bone to lesion
Matrix characteristics
Soft tissue involvement
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Benign lesions suggested if

Small lesion
Tumor limited to confines of bone & has not broken through cortex
Well-demarcated border surrounded by thin rim of sclerotic bone
No soft tissue involvement
Malignant lesions
Suggested if
Size of lesion (small is good, big is bad)
Lack of sclerotic margin
Cortical destruction
Soft tissue mass
Malignant tumor cells that extend through cortex
May elevate periosteum & stimulate it to produce a small triangle of reactive
bone -> Codman's triangle
Seen in osteosarcoma & Ewing's sarcoma but can also be found in infections &
hemorrhagic lesions
Formation of new osseous tissue outside involved bone
Suggestive of malignancy but can also be found in infection & myositis ossificans
Sunray effect, frequently found in osteogenic sarcoma, may be caused by other
malignant, & even some benign, processes
Onion skin appearance, seen in Ewing's sarcoma, may also be found in other
conditions that elevate periosteum, eg. infection
CXR for metastases
Skeletal survey for multiple myeloma
MRI
Good soft tissue definition (better than CT)

Able to image in any plane thus good for pelvic/sacral lesions
Roles
Evaluates extent of tumour both intra & extramedullary
Epiphyseal extension & joint involvement
Skip lesions
Extra-osseous extension
Neurovascular involvement
Determines representative area for biopsy
Locate least differentiation & mineralisation site
Avoid necrosis, #, codman's triangle
Evaluates response to Rx
T1 weighted images best for looking at
Anatomy
Extent of marrow involvement
T2 more useful for evaluating cortical bone & soft tissue extent (NB - CT better for showing areas
of calcification/ossification)
Best technique to identify haemorrhage/oedema/inflammation, eg. prior biopsy
Oedema usually surrounds malignant lesions & is unusual around benign tumours
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Bone Scan
Gold standard for primary staging

Diphosphonates labelled with Technetium 99 usually used in dose of 500-600 mBq
Excellent for occult malignancies
Osteoid osteoma -> increased activity in blood pool phase as well as delayed bony phase
Inflammatory lesions also increased activity in blood pool scan
Gallium 67 has also been utilised
See details
CT Scan
Roles
Indicates extent of bony involvement/destruction

Good for detecting subtle cortical disruption, #, calcification or ossification
Not as good as MRI for soft tissue extent; can detect soft tissue masses >= 5 mm
diameter
Useful to stage, eg. lung secondaries
5 mm slices - 93% specificity
Angiography
To identify
Feeding vessels
Tumour proximity to major vessels
Displacement of vessels by tumour -> access for excision of tumour
Embolisation of vascular tumour prior to surgery
Intra-arterial chemotherapy
Biopsy of Bone Tumours
Final step in staging

Crucial step
Violation of anatomical boundaries
Risk of seeding & infiltration -> recurrence
-ve impact on LSS
Principles
Final step in staging, therefore, should know probable diagnosis & stage of tumour before biopsy
Determine exact anatomical approach & identify representative part of lesion
Biopsy may tamper with radiological imaging & definitive surgery
Performed by same surgeon who will perform definitive surgery
Establish surgical plan before biopsy
? biopsy -> frozen section -> definitive surgery during same anaesthetic
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Biopsy tract orientation & location critical

Will need to be included in definitive surgery if lesion is malignant
Longitudinal incision
Direct musculoaponeurotic compartment
Avoid major neurovascular structures
Avoid bursa & joint contamination
Meticulous haemostasis to avoid tracking haematomas
Tourniquet
Can be used but no squeezing
Release before wound closure for haemostasis
Incision through muscles so that layers can be closed tightly
Bone cement may be used
If haemostasis cannot be achieved, drain brought out of corner of wound
Compression dressing
Send samples for microbiological analysis
Open Biopsy
Excisional biopsy when possible in benign lesions

Incisional biopsy preferable in malignant lesions
To minimize contamination of peripheral tissues
To preserve tumor's pseudocapsule (which facilitates future tumor excision)
Need for embolization
Some lesions eg. suspected renal or thyroid carcinoma should have preop embolization
But consideration should be given to embolization if a non compressible lesion is present
(eg. might occur with a pelvic or acetabular lesions)
After consultation with pathologist & radiologist
Small longitudinal skin incision
Sharp dissection should proceed directly to tumour
Through muscle, not between muscle planes
Uninvolved anatomic compartments should not be exposed
Avoid all major neurovascular structures to prevent contamination
Excise a block of reactive tissue, pseudocapsule, capsule & block of tumour (1 x 1 cm) ->
formalin +/- frozen section
Windows in bone should be
As small as possible (<10% of bone diameter - maintains >80% of bone strength)
Oval to avoid stress risers & pathological #
Release tourniquet prior to closure -> haemostasis
Close in layers & with subcutaneous stitch
Drains should come out through wound
Compression dressing
If proceed following biopsy -> new instruments & drapes to stop seeding
Needle Biopsy
As for open biopsy
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Place biopsy tract where it can be excised

Fine needle biopsy
Relies on cytological interpretation by experienced pathologist
Accuracy = 65-95% (determined by adequacy of collected tissue sample)
Does not allow for immunohistochemical analysis
Core needle biopsy
Uses trocar cannula system, with a outer sleeve which closes over trocar, capturing
sample of tissue
Provides more tissue than fine needle & allows for immunohistochemical analysis
Accuracy = 75-95%
Advantage of needle biopsy
Minimal risk of seeding
Disadvantages
Tissue obtained may be from necrotic portion of tumor & therefore not suitable for
diagnosis
Tissue may be reactive in nature & not representative of actual tumor -> frozen section
may thus be beneficial
Material retrieved limited
Frozen Section
Able to determine if specimen is adequate or representative

Can decide if lesion is inflammatory & needs culturing
Can determine if there is need to perform further Ix/tests
Immediate diagnosis possible -> can proceed to definitive surgery
Common biopsy sites

Distal femur
Medial approach
Avoid anterior extensor & joint
Proximal tibia
Anterolateral approach
Avoid anserinus bursa & patellar tendon insertion
Proximal humerus
Anterior deltoid compartment

Avoid deltopectoral approach
TREATMENT PRINCIPLE
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SURGICAL PROCEDURES
Goal of Rx: to remove lesion with minimal risk of local recurrence
Limb Salvage/Sparing
1.
2.
3.
A procedure involving wide resection of local tumour & replaced with either endoprosthesis,
allograft or autograft with aim of preservation of distal part of limb without compromising local
tumour control
Criteria for limb salvage
1. Local control of lesion must be at least equal to amputation
2. Saved limb must be functional
Indications
Optimum oncological margins achievable - stage IIB
Moderate soft tissue extension
Neurovascular bundles not compromised
Free of infection
Metastasis absent or responsive to curative Rx
Patient in good condition
Relative contraindications
Pathological # - spread via haematoma
Inappropriate biopsy site - contamination
Skeletal immaturity
Must be in an anatomical location which favours reconstruction & allows wide surgical
margins
Major neurovascular involvement
Extensive muscle involvement
Infection - implant
Presence of distal metastasis not a contraindication
Limb Salvage vs Amputation
4 main concerns
Survival
No effect on long term survival: 40-70% 5-yr survival
But must have
Adjuvant/neoadjuvant chemotherapy
Wide surgical margins
Local recurrence 5-10% - same as amputation (but not disarticulation)
Immediate & delayed morbidity of reconstruction
Immediate: skin necrosis, infection
Need to raise large cutaneous flaps
Stretched & thinned out by tumour
Long term
Arthrodesis - nonunion, #
Prosthesis with mobile joints -> loosening
Function
NONE of reconstructions will restore a functionally normal limb -> always
partially disabled
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4.
Upper limb
Resection of proximal humerus better than forequarter
amputation
Preservation of hand function
Lower limb
BKA better than foot or distal tibial resection
Oxygen consumption: BKA < prosthesis with mobile knee joint <
arthrodesis < AKA < hip disarticulation
Psychosocial benefits
Upper limb: yes, salvage better
Lower limb: no difference (or no instrument to detect difference)
If patient does not have a premorbid psychological disorder, he or she
will make good adjustment to operation & disease, no matter what type
of procedure is performed
Surgical principles/guidelines
Identification & preservation of key neurologic & vascular structures
Resection of affected tissue should have a wide margin with normal tissue cuff in all
directions
All previous biopsy site & all potentially contaminated tissue removed en bloc
Reconstruction of axial skeleton
Adequate motor reconstruction by regional muscle transfers
Adequate soft tissue coverage to reduce skin flap necrosis & secondary infection
Types of osseous resection
Intercalary (between joints)
Intra-articular (1 side of joint)
Extra-articular (both sides of joint)
Reconstruction options for skeletal defects
1. Resection arthrodesis
2. Allografts: osteoarticular, intercalary
Long lasting
Problems
Infection
Nonunion
#
3. Allograft prosthetic composite
4. Endoprosthesis
Immediate function
Problems
Infection
Service (bushing usually wears out by 7 yrs)
Metallosis
Loosening - 80% survival at 15 yrs
5. Illizarov bone transport
6. Cement spacer for upper limb
Patient & relative counseling
o Small increase in local recurrence 5-10% but no difference in long term survival
o Morbidity increased: hospital stay, operations
o Durability variable esp. with mobile joint
o Function better esp. upper limb but none normal
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o
No matter what type of surgery, if premorbid personality normal, good psychosocial

adjustment
Surgical Margins (Musculoskeletal Tumour Society)
Intra-lesional
Through tumour (within pseudocapsule)
Leaves macroscopic tumour
Not therapeutic; results in bleeding, infection & non-healing wound in malignant tumours
Marginal
Through pseudo-capsule or reactive zone (contains inflammatory cells, oedema, fibrous
tissue, satellites of tumour cells)
Satellite lesions left
Controls non-invasive benign tumours
Recurrence of malignant tumours = 25-50%
Wide
Through normal tissue, leaving a cuff of normal tissue
Skip lesions left
Recurrence of malignant tumours = <10%
Radical
Removal of entire tumour & its compartment en bloc
Distant metastases left
Amputation
Should be thought of as a form of reconstruction where surgical control of tumour
precludes useful function
CHEMOTHERAPY
Multi-agent chemotherapy has significant impact on limb salvage

survival for osteosarcoma & Ewing's
Localised disease = 60-70% long-term disease-free survival
Type
Neo-adjuvant chemotherapy x 8-12/52
Adjuvant chemotherapy x 6-12/12
& disease-free
Neo-adjuvant chemotherapy
= staging -> preop chemo -> restaging -> surgery -> tumour kill rate -> further Rx (maintain or
change chemo or introduce radiotherapy as indicated)
Roles
Enables action of agents against tumour & its metastases to commence immediately
Reduces mass & vascularity of tumour prior to definitive surgery
Enables time for operative planning
Efficacy of chemotherapeutic agents evident at time of tumour resection
Tumour kill rate
Want 90% kill rate
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If <90% -> change agents

No increased survival evident with different agents in those who do not respond to initial
Rx (? just identifies those patients with good prognosis)
Adjuvant Rx
Commence adjuvant Rx once wound has healed

May persist for 2/12 to 2 yrs depending on response
Complications
MSK
Stunting of growth (catch up later)
Osteoporosis
AVN
Specific
Vincristine -> neurotoxicity
MTX -> hepatic damage
Adriamycin -> cardiotoxicity
Cyclophosphamide -> haemorrhagic cystitis
Cisplatinum -> nephrotoxicity & hearing loss
Chemotherapeutic induced malignancy, usually blood-forming eg. leukaemias but also may -> Ca
bladder or skin (particularly a/w cyclophosphamide)
Some side effects occur with many cytotoxic agents
Nausea & vomiting
Bone marrow toxicity
Gastrointestinal toxicity
Alopecia
Gonadal effects
Hyperuricaemia
RADIOTHERAPY
For local control of

Ewing's, lymphoma, myeloma, metastatic bone disease
Adjunct for soft tissue sarcomas
Preop radiotherapy may result in
Decreased size of tumour
Decreased chance of seeding at time of resection
If seeding, decreased chance of viability of shed cells
Effect of radiation
2 modes
Direct = absorption by complex molecules causes rupture of chemical bonds with
damaging effects
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Indirect = by ionisation & formation of highly reactive intracellular free radicals -> DNA
changes -> stop cell reproduction & slow cell turnover -> loss of specific cell function
Effect on cancer cells not due to more rapid growth but better capacity for recovery & repair of
normal tissues cf poor capacity of cancer tissues
Destruction of small blood vessels on which growth of a tumour depends also contributes to effect
& induction of an inflammatory response may -> destruction of cells weakened by radiation
Increased sensitivity of cells in presence of oxygen up to a critical level but above this level (about
that of normal atmospheric pressure) sensitivity does not increase appreciably (hypoxic tissue
has decreased radiosensitivity)
Radiosensitivity of a tissue is directly proportional to its mitotic activity & inversely proportional to
degree of differentiation
Adverse effects
Erythema of skin & hyperpigmentation

Hair loss & skin flaking
Lymphoedema -> need to screen a strip of skin -> lymphatic drainage
Subcutaneous fibrosis
Muscle atrophy & fibrosis
Joint -> stiffness & loss of function -> physio
Enteritis, diarrhoea, obstruction & bleeding
Cystitis & hepatitis
MSK
Scoliosis may develop therefore include both sides of vertebrae
Children -> premature fusion of growth plates
Irradiation induced sarcoma
Late stress #
Definitions
Rad (radiation absorbed dose) is a measure of energy imparted to matter by ionising radiation per
unit mass (1 Rad = 100 erg/gram (0.01 j/kg))
Grays (Gr) = 1 joule of energy absorbed by a mass of 1 kg (equivalent to 100 rad)
Bone Cysts
Unicameral (Simple) Bone Cyst (UBC)
Aneurysmal Bone Cyst (ABC)
Giant Cell Tumour (GCT)
Unicameral (Simple) Bone Cyst (UBC)
Benign lesion which occurs during growth

Cysts may be active or latent
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Active cysts are located near growth plate, but they move further away as child grows & become
inactive (latent)
Aetiology
Unknown
Venous obstruction leading to a transudate of fluid
Fluid contains high levels of IL-1 & IL-6, which stimulate osteoclasts
Incidence
20% of benign bone lesions

Age 5-15 yrs; not found in adults
M:F = 3:1
Sites: proximal humerus (67%) followed by proximal femur (15%)
May be found in unusual sites (eg. calcaneum, pelvis) in patients >17 yrs
Clinical
Asymptomatic
Usually presents as pathological # (~65%)
Differential diagnosis
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Depending on location: fibrous dysplasia, ABC
Radiographic features
Well defined, central osteolytic area with thin sclerotic margin

Symmetric expansion with thinning of cortex
"Falling leaves"
Metaphyseal in young & moves towards diaphysis with growth
It fills & slightly expands juxta epiphyseal metaphysis
CT not helpful unless UBC in pelvis
Pathology
Histologically UBC's are thin walled cavities filled with blood tinged fluid
Lining cells are cuboidal, but not endothelium
There is endosteal osteoclastic activity & periosteal new bone formation
Treatment
Goal: to minimise # risk until cyst heals (but this can take years)
Aspiration to confirm diagnosis
Steroid injection
1-3 percutaneous injections repeated at 2 monthly intervals
60-80% success rate
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Curettage & bone graft

Reserved for recalcitrant lesions
50% recurrence rate & possibility of damage to growth plate
Bone marrow aspirate has recently been used
Aneurysmal Bone Cyst (ABC)
Benign solitary, expansile & erosive lesion of bone
Aetiology
Unknown, but ABC's are thought to be a reactive process secondary to trauma or vascular
disturbance
Primary ABC's
Lesions are secondary to increased venous pressure -> haemorrhage -> osteolysis
This osteolysis can in turn promote more haemorrhage causing amplification of cyst
ABC's can be secondary to an underlying lesion
Kransdorf, Amer J Roentgenol 1995 Mar;164(3):573-80 -> original lesion can be
identified in 1/3 of cases
Most common precursor lesions: giant cell tumor (19-39%), osteoblastoma, angioma, &
chondroblastoma
Less common precursor lesions: fibrous dysplasia, non-ossifying fibroma, chondromyxoid
fibroma, UBC, fibrous histiocytoma, eosinophilic granuloma, & osteosarcoma
Incidence

Most frequent in children (85% cases <20 yrs)
F:M = 2:1
ABC's can be found in any bone in body
Most common location: metaphysis of lower extremity long bones
Vertebral bodies or arches of spine may be involved
~1/2 of lesions in flat bones occur in pelvis
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Clinical
Swelling, tenderness & pain

Occasionally limited range of motion due to joint obstruction
Spinal lesions can cause neurological symptoms secondary to cord compression
Pathological # rare due to eccentric location of lesion
Differential diagnosis
Depending on location: UBC, chondromyxoid fibroma, GCT, osteoblastoma & highly malignant
telangiectatic osteosarcoma
Osteolytic lesion placed eccentrically in metaphysis

Expansile nature of lesion often reflected by "blow-out" or "soap bubble" appearance
Periosteum elevated
'Pencil-in-cup' appearance
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CT scan
Can help delineate lesions in pelvis or spine where plain film may be inadequate
Can narrow differential diagnosis of ABC by demonstrating multiple fluid-fluid levels
within cystic spaces
MRI can also confirm multiple fluid-fluid levels (blood/serum)
Pathology
Macroscopical
ABC is like a blood filled sponge with a thin periosteal membrane
Soft, fibrous walls separate spaces filled with friable blood clot
Microscopical
Cystic spaces filled with blood
Fibrous septa have immature woven bone trabeculae as well as macrophages filled with
haemosiderin, fibroblasts, capillaries & giant cells
Treatment
Rx approach will vary depending of location & aggressiveness of lesion

A slow growing, indolent ABC has been observed to regress spontaneously
Options
Most lesions can be treated with curettage & application of a high-speed burr
Marginal excision or wide excision with bone grafting preferable
Recurrence
Rates vary widely

Statistically related to young age & open growth plates
May be less likely following wide excision than curettage
If recurrence detected, thorough examination of original radiographs & pathology specimens
should be performed to ensure that primary lesion, if any, is discovered, since this may radically
alter Rx plan
Once precise diagnosis known, local recurrences may be retreated by appropriate methods
Wide resection & limb-sparing reconstructions necessary to prevent progressively destructive
recurrence
Curettage & bone graft can be complicated by profuse bleeding from lesion
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Radiation has been used in some cases where operative Rx is not possible, but this adds
additional risk of malignancy
Giant Cell Tumour (GCT)
Benign
Usually solitary
Nearly always located at very end of a long bone (metaphyseal/epiphyseal)
Locally aggressive
Can undergo malignant transformation (5-10%)
Rarely metastasises (<1% to lungs)
Incidence

Not seen until after growth plate closes; age 20-40 yrs
Unlike most bone tumours, more common in females
Most commonly seen in distal femur, proximal tibia & distal radius
In spine, usually anterior part of vertebral body
Most common tumour to occur in distal phalanx
Classification
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Campanacci
1
Lesion confined within bone
Lesion expanding cortex
Breach of cortex
1 - involvement of joint
2 - distant metastases
Ennekings
1 Benign latent GCT
No local agressive activity
2 Benign active GCT
Imaging studies -> alteration of cortical bone structure
3 Locally aggressive GCT
Imaging studies -> a lytic lesion surrounding medullary & cortical bone
May be indication of tumor penetration through cortex into soft tissues
Presentation
Pain - deep, persistent intraosseous pain that mimics internal derangement of knee
Swelling - reactive knee effusion
Pathological # occurs in 10-15%
Differential Diagnosis
FCD
ABC
Hyperparathyroidism
May produce brown tumors that are radiographically & histologically similar to GCT of
bone, with exception that brown tumors tend to be diaphyseal in location
Unlike brown tumors, serum Ca normal in GCT
X-rays
Usually a well defined radiolucent (lytic) lesion in metaphysis/epiphysis extending
up
to joint surface
Eccentric
Junction with normal bone poorly defined with or without marginal sclerosis
Cortex thinned & sometimes ballooned
Soap bubble appearance - scalloping & ridges in wall (septation)
Egg shell
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CT scan
Helps determine extact amount of cortical destruction
Helps determine optimal location of cortical window
Bone scans
May show decreased radioisotope uptake in center of lesion (doughnut sign); also found
with ABC
MRI
Help determine extent of tumor destruction
May be indicated when tumor has eroded through cortex & allows determination of
whether concomitant neurovascular structures involved
May help evaluate subchondral penetration
Pathology
? arises from mesenchymal cells of connective tissue framework

Soft, friable tumour
Cut surface tan in colour, with areas of necrosis & haemorrhage
Histology
Numerous multinucleated giant cells
Stromal cells: homogenous, mononuclear round/ovoid with large nuclei
Nuclei of stromal cells identical to nuclei of giant cells, a feature which distinguishes GCT
from other conditions containing giant cells
Up to 50% have soft tissue extension but does not indicate malignancy
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Treatment
Due to proximity to articular cartilage, excision of GCT of bone difficult

Intralesional excision alone tends to leave tumor cells behind, past attempts of excision a/w high
recurrance rate (40%)
Stage 1 or 2 lesions
Aim: remove lesion with preservation of involved joint
Intra-lesional excision by "extended" curettage - 85-90% success rate of local
control
Excision facilitated by making a cortical window, large enough to allow complete access
to every corner of intra-osseous lesion
Motorized burr & cautery to complete excision
Adjunctive measures
Use of phenol, polymethacrylate, & liquid nitrogen -> increase zone of necrosis at
periphery of excision ("extended" curettage)
Cement
R.J. O'Donnell et al (JBJS 1994) -> use of cement did not appear to
improve results as compared to historic controls with 25% recurrence
rate with long term follow up
Barium impregnated cement allows for accurant determination of local
recurrance
Cement provides mechanical support following curretage & does not
appear to affect cartilage when placed in subchondral region
When cement is applied beneath subchondral cartilage, joint should be
irrigatted with chilled saline
Liquid nitrogren may cause excessive tissue necrosis, extending into normal
tissue
Resulting cavity can be filled with
Bone graft -> infection, resorption
Cement -> immediate strength, easier to detect recurrence
At completion of procedure, window replaced with bone graft or bone graft substitute
Recurrent or stage 3 lesions
This category includes # with major pathologic # or articular destruction
Prior pathologic # should be allowed to heal before surgery attempted
En bloc excision with wide margin along with appropriate major limb reconstruction
Amputation reserved for massive local recurrence, malignant change or infection
Radiotherapy reserved for rare unresectable tumours because of increased risk of
secondary malignancy
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Lung metastases
Resection
Prognosis
Sarcomatous transformation in 5-10%

Recurrence following simple curettage 50%
Recurrence following simple curettage plus adjuvant Rx (cryo etc) 17%
Malignancy more common in recurrent GCT
Bone Tumours - Index

Bone Tumours - Diagnosis
Classification
Histology type
Benign
Malignant
Bone Producing Tumours

Osteoid osteoma
Osteoblastoma
Osteosarcoma
Cartilage Producing Tumours Osteochondroma

Enchondroma
Chondroblastoma
Chondromyxoid Fibroma
Chondrosarcoma
Fibrous Bone Tumours

Fibrous Cortical Defect
Fibrous Dysplasia

Fibrosarcoma
Unknown origin
Ewing's sarcoma
Malignant GCT
Adamantinoma
Giant Cell Tumour
Reticuloendothelial Tumours Eosinophilic

granuloma/Histiocytosis X
Multiple Myeloma
Lymphoma of bone
Vascular Tumours
Haemangioma
Haemangioendothelioma
Haemangiopericytoma
Notochordal
Chordoma
Lipogenic
Lipoma
Neurogenic
Neurilemoma
Liposarcoma
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BONE PRODUCING TUMOURS

Benign
Osteoid osteoma
Osteoblastoma
Malignant
Osteosarcoma
Characteristics
Recognise matrix as woven bone (osteoid)

Osteoid osteoma & osteoblastoma have similar histology, but different clinical, radiological &
gross pathological findings
Stroma
Fibrovascular = benign
Sarcomatous = malignant
Osteoid osteoma
Small, benign, solitary,
painful lesion of bone
Aetiology
Unknown
Incidence
10% of benign bone tumours

M:F 2:1
Peak age 5-25 yrs (85% in this range); rare over 40 yrs
Location
Any bone, rarely multifocal
Tibia & femur in 50%
Spine - posterior elements
Only occurs in bones formed by endochondral ossification
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Clinical
Pain
Commonest presentation
Often worse at night
Relieved by aspirin
10% occur in spine & may -> scoliosis
Other sites may -> joint effusion, synovitis, LLD
Runs a self limiting course
Pain usually decreases as lesion matures lasting 18-30/12
Lesion healed by 3-7 yrs
But usually requires surgery for pain relief
X-rays
Lytic nidus surrounded by intensely sclerotic bone (which may mask nidus)
Centre of nidus may be calcified
CT or tomograms -> diagnosis
Hot spot on bone scan
Bone island (enostosis)

Brodie's abscess
Osteoblastoma
Fatigue #
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Pathology
Nidus usually <1 cm diameter, most <0.5 cm

Nidus composed of thick vascular bars of osteoblastic tissue surrounded by vascular fibrous
tissue finally surrounded by mature reactive cortical bone
Contains fibroblasts, osteoblasts & osteoclasts (woven bone); no marrow element
May have calcified centre in nidus
Pathology Pictures
Treatment
NSAIDs
Relieves symptoms
May take 3-4 yrs for symptoms to resolve
Surgical
Nidus excision -> no recurrence (need only intact rim of reactive bone around nidus to
ensure complete excision)
Intraop localisation with
Bone scan
Tetracycline (4 mg tetracycline per kg qid 1-2/7 preop -> specimen excised under
UV light)
CT
X-ray
Beware dumbbell nidus
Percutaneous radiofrequency coagulation
Percutaneous insertion of biopsy needle under CT scan guidance
Tissue biopsy taken to prove needle is properly located
Radiofrequency electrode with a 5 mm exposed tip introduced through cannula
o
Electrode connected to radiofrequency generator which raises temperature of tip to 90 C
(for 6')
As noted by Rosenthal et al 1998, results comparable to standard open technique
Osteoblastoma
Benign, solitary,
painful lesion of bone
Incidence
<1% of primary bone tumours

Peak age 10-35 yrs, 80% <30 yrs old
40-50% are vertebral posterior elements
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Clinical
Less intense pain than osteoid osteoma

Pain partially relieved by aspirin
Occurs in spine (posterior elements), often a/w scoliosis & may be neurological signs
May occur in long bones or phalanges
Radiology
Well demarcated osteolytic lesion sometimes containing flecks of calcification

Less reactive bone than osteoid osteoma
May have aggressive features
Metaphyseal
Enlarges bone
Periosteum intact
No soft tissue mass
Bone scan -> intense activity
ABC
Osteoid osteoma (spine)
Giant cell tumour
Osteosarcoma (if more aggressive)
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Pathology
As for osteoid osteoma but increased size 2-10 cm

Vascular stroma, abundant irregular areas of mineralised bone & osteoid, more organised than
an osteoid osteoma
No atypia, no cartilage & occasional mitoses
Vascular tumour +/- haemorrhage +/- calcification
Texture gritty & friable
Malignant change has been reported
Spectrum of aggressiveness
Treatment
Intracapsular resection -> 20% recurrence

En bloc resection -> no recurrence
Use cryotherapy (PMMA) as adjuvant
Prognosis
One case of malignant change reported

Tumours aggressive locally but do not metastasize
Osteoid Osteoma
Incidence
Clinical
Pathology
X-ray
Osteoblastoma
Common
Rare
Pain relieved by aspirin
Pain partially relieved by aspirin
Nidus <1-2 cm
Woven bone
Surrounded by reactive bone
Nidus 3-10 cm
Sheets of woven bone
Small central nidus surrounded by

dense reactive bone
Lucent or dense lesion
Osteosarcoma
Primary malignant tumour arising from bone & producing bone

Highly malignant spindle sarcoma variants depending on appearance of prominent cell type (may
look like fibrosarcoma, chondrosarcoma or myxosarcoma)
Most are high grade & intramedullary
Incidence PISASM
Accounts for 21% of malignant primary bone tumours

Affects ~1/200,000 population
M:F 2:1
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Peak incidence 10-20 yrs (age of rapid growth), with 2nd peak at 50-70 yrs (80% <30 & those
>40 usually secondary to Paget's)
3rd most common malignancy in adolescents, after leukaemia & lymphoma
75% occur in distal femur or around knee
90% metaphyseal in long bones
10% present with macroscopic metastatic disease; 90% micro-metastases
Types
Intramedullary (classical or ordinary) osteosarcoma

Surface osteosarcomas
Parosteal osteosarcoma
Periosteal osteosarcoma
Secondary osteosarcomas
Paget's
Post-radiation
Telangiectatic osteosarcoma
Clinical
Pain at rest which is constant & worse at night

May have a tender lump which may lack a definite edge & may be attached to muscle
If vascular may pulsate & feel warm
Pathological #
Rare
Be aware of # following minimal trauma as # secondary to osteosarcoma often -> late
diagnosis of tumour
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Radiology
X-rays
Variable with combination of bone destruction & bone formation
25% lytic
35% sclerotic
40% mixed
Cortical breach common
Sun ray spicules (radial ossification)
Codman's triangle (lifting of periosteum)
Adjacent soft tissue mass
Joint space rarely involved
MRI
Essential to elucidate soft tissue & neurovascular involvement
Skip lesions
CT scan
To detect radiographically occult pulmonary metastases
Helps to determine response to chemotherapy & plan surgical approach
Bone scan
To detect radiographically occult skeletal metastases
Pathology
Oncogenes in osteosarcoma - Retinoblastoma gene & P53 - control restricion point (G1 & G0 to
S)
Most are high grade aggressive tumours usually ~10 cm diameter at diagnosis (~10/12 growth)
50% osteoblastic
25% chondroid
25% fibroblastic
Associated with areas of increased osteoblastic activity, eg. metaphysis, children & Paget's
20% secondary to other conditions, eg. Paget's, enchondromas, osteochondromas, chronic
osteomyelitis, irradiation, fibrous dysplasia, osteopetrosis & bone infarction
Usually occurs in metaphysis
Initially extends within medulla but soon perforates cortex -> raises periosteum -> Codman's
triangle
As tumour mass expands new bone forms along vascular channels -> sunray spicules
Metastasises via blood stream to lung & other bones
Histology
Bone forming cells with +ve ALP staining
Bars of tumour osteoid embedded in a stroma of hyperchromatic, neoplastic,
mesenchymal spindle cells
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Classical osteosarcoma with sunray spicules (sunburst) & Codman's triangle
"Mixed" appearance with both osteoblastic & osteolytic (radioluscent) areas

Note that lesion appears to be stopped from extending distally by open,
Macrosection shows that lesion has penetrate
physeal growth plate
Lesion with areas of amorphous ossification; cortical breakthrough, with soft- Scattered areas of fleshy consistency or hem
tissue extension; sunburst
transphyseal extension
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Bars of tumour osteoid embedded in a stroma of hyperchromatic, neoplastic, mesenchymal cells typical of a high-grade
, classic osteosarcoma
Ewing's sarcoma
Benign bone tumour may have similar appearance especially if X-rayed early
Osteomyelitis or syphilis
Post traumatic callus or myositis ossificans
Stress # - pathology may look similar
Treatment
Consider osteosarcoma as systemic disease, therefore combined therapy

Remove primary site by wide surgical excision
Treat microscopic disease by multi-agent chemotherapy
Chemotherapy
Multi-agent chemotherapy has significant impact on
Limb salvage
Disease-free survival
Pulmonary metastases (from 90% to 30%)
Commonly used agents include doxorubicin, cisplatin, high-dose methotrexate,
ifosfamide, cyclophosphamide
Methotrexate -> 80% response
T10 (MSKCC) regimen (methotrexate, vincristine, adriamycin,
cyclophosphamide)
60-75% survival
45% of patients -> 100% kill rate -> 100% survival
Survival >10 yrs = cure
UMMC regimen
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Neo-adjuvant chemotherapy
8-12/52 (3 cycles)
Good response to chemotherapy, indicated by 90% tumor necrosis on biopsy,
correlates with ultimate survival
Adjuvant chemotherapy
Regimen continued postop or changed to cisplatin depending on histology ->
92% disease free at 2 yrs
Chemotherapy continues for 1 yr in 4/52 cycles (5 cycles)
Intra-arterial chemotherapy used in some centres -> increased dose to site of lesion but
no evidence that this changes outcome as if multiple feeding vessels some of tumour
may be missed
Surgery
Wide resection or amputation
Limb salvage
Requires ability to
Achieve safe, tumour-free margins (wide)
Preserve function
Preserve nerves
Preserve or reconstruct vessels
Preserve sufficient muscle for functional motor power &
soft tissue coverage
Does not seem to have higher local recurrence rate than amputation (510%)
Reconstruction options
Allografts
Endoprosthesis
Expendable bone (fibula, ilium)
Rotationplasty
Surgical resection of pulmonary metastases
Aggressive surgical resection will improve 5-yr survival rate
Resection of pulmonary metastases, together with multiagent chemo, is a/w longterm survival in ~20%
Pulmonary tumor resection requires good local disease control & no extrapulmonary metastasis
Radiotherapy
Relatively radio-resistant tumour
For
Palliation of local pain & to treat surgically inaccessible lesions & painful
metastatic deposits
May also be used preop to decrease size & vascularity of tumour
Prophylactic irradiation of chest has not been shown to be effective
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Prognosis
At time of diagnosis, most osteosarcomas are stage IIB lesions that have infiltrated soft tissue
10% have macro-metastases at presentation; 90% micro-metastases
Untreated, 95% death in 2 yrs
With Rx, even with metastatic (Stage III) disease 5-yr survival now 30-40% (10-20% with surgery
alone)
Prognostic factors ATLS Rs160
1. Age - adults do worse
2. Type
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1. Parosteal - tend to be more low grade tumours ? -> better prognosis

2. Intraosseous (classical) osteosarcoma -> good prognosis
3.
4.
5.
6.
7.
8.
Location (proximal worse than distal, pelvis, spine)

Size of primary tumour (big is bad)
Stage
>16 metastatic deposits -> poor prognosis
Origin of tumour in pre-existing lesion -> worse prognosis
Response to chemotherapy
80-90% 5-yr disease free survival in good response patients
60-70% 5-yr disease free survival in patients with poor response rates
Pathological # does not affect prognosis
100% of bilateral retinoblastomas -> osteosarcoma
Parosteal Osteosarcoma
Incidence
1% of primary malignant bone tumours

Usually patient >20 yrs old
Peak incidence 30-50 yrs
M:F 2:3
Clinical
Present with a constant ache or lump

Usually a long bone juxta metaphyseal
Usually presents as Stage IA lesion (low grade)
Commonest site = posterior aspect of distal femur
Radiology
X-rays
Well circumscribed mass

May be separated from cortex by a lucent line (30%)
Broad based tumour with mottled calcification
Cortex not eroded
Does not invade medullary cavity (unlike chondrosarcoma)
Tends to encircle bone
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CT scan
To differentiate from classical osteosarcoma
Mass separated from cortex, ie. no cortical involvement
Osteochondroma
Myositis ossificans
Treatment
Chemotherapy or radiotherapy not effective in preventing recurrence -> wide surgical resection
Recently, Rx = classical osteosarcoma
Prognosis
Said to be better than classical osteosarcoma

70-80% 5 yr survival
Probably not different in relation to stage of disease at presentation
Periosteal Osteosarcoma
Arises from deeper side of periosteum

Rare
Usually diaphysis of femur or tibia
X-rays
Ill defined swelling
Sub-periosteal new bone
Large external, poorly mineralized mass within a depression of cortical erosion
Rx: preop chemotherapy, wide surgical resection & maintenace chemotherapy
Prognosis intermediate between high grade intramedullary osteosarcoma & low grade parosteal
osteosarcoma
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Paget's Sarcoma
Incidence
Age >45 yrs

M:F 2:1
Occurs in advanced polyostotic disease 10%
Affects 1% of all patients with Paget's
Pathology
50% or more are osteosarcomas

25% fibrosarcomas
Remainder chondrosarcomas or anaplastic tumours
Prognosis
Poor - <50% 1-yr survival
Radiation Sarcoma
Mean latency to onset of 10 yrs

Earlier appearance in younger patients
In excess of 20 Gr radiation given
Criteria for diagnosis
Benign nature of initial lesion must be evident both histologically & radiologically
Secondary malignancy must have arisen within radiotherapy Rx zone
A relatively long latent period must have elapsed (5 yrs) before clinical appearance of secondary
lesion
All secondary sarcomas must be identified histologically & different to that of original pathology
Prognosis
Cumulative disease free survival rate = 17% at 5 yrs
Telangiectatic Osteosarcoma
Rare - 5% of all osteosarcomas
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Aggressive
Presents with pathological #
Arises within diaphysis (= periosteal osteosarcoma)
Femur & tibia most common, then humerus
Radiology
Often entirely osteolytic

Bone & cortex destruction
Periosteal reaction
Codman's triangles
MRI - high intensity with fluid-fluid levels on T1
Pathology
Gross appearance is a multi-cystic "bag of blood"

Microscopically it has large blood filled spaces & thin septation
Within septa there is scanty osteoid production by pleomorphic malignant cells of this high grade
tumor
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Prognosis
Poor
Cartilage Producing Bone Tumours

Benign
Osteochondroma
Enchondroma
Chondroblastoma
Malignant
Chondrosarcoma
Osteochondroma
Cartilage capped bony projection/exostosis

Developmental abnormality of metaphyseal area of any bone formed in cartilage (endochondral
ossification)
Incidence
Commonest benign tumour of bone; 45% of benign bone tumours

12% of all bone tumours
Most become evident <20 yrs
May be solitary or multiple (diaphyseal aclasis)
Any bone developing by endochondral ossification may be involved
50% are distal femur, upper tibia or proximal humerus
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Diaphyseal aclasis/Multiple exostosis

Autosomal dominant
Disordered endochondral growth
Multiple osteochondromas, often sessile & large, & disordered metaphyseal growth
Short stature & bowing of limbs
Treat individual lesions as necessary & observe for malignant change
Malignancy risk ~20% overall or 0.2% per lesion
Trevor's disease
Osteochondroma on epiphyseal side of growth plate
Clinical
Present with
Painless lump
Interference of tendon function
Pain secondary to muscle irritation
Inflammed bursa
Incidental finding on X-ray
May be sessile or pedunculated
Active growth during skeletal growth then become latent (= UBC, FCD)
Move towards diaphysis with growth & usually angle away from growth plate (= UBC, FCD)
During growth period bone scan -> activity at tip
Increased activity on bone scan after maturity suggests malignant change
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Radiology
X-ray hallmark: blending of tumour into underlying metaphysis; cortex of lesion continuous with
that of underlying cortex
Lesion may be flat, sessile (broad base) or peduculated (stalk like)
Pedunculated osteochondromas are oriented in proximal direction
Look for well defined metaphyseal excrescence of bone with mottled density
Cartilaginous cap displays irregular areas of calcification
Pathology
Normal bone covered by a cap of normal cartilage

Cartilage cap resembles layers of normal growth plate
Cartilage more disorganized than normal
Binucleate chondrocytes in lacunae
Covered with a thin layer of periosteum
Treatment
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Nil required unless symptomatic (persistent irritation [from bursitis or tendon] or neurovascular
compromise)
Excise if troublesome in 2nd decade
Extracapsular marginal excision
Including cartilaginous cap & overlying perichondrium
Deep bony base has minimal activity & may be removed piecemeal
Cartilaginous cap should not be traumatised during removal
Recurrence <5%
Decreased risk of recurrence if excised after maturity
Prognosis
Risk of malignant change ~0.2% in a solitary lesion

Risk of malignant change in diaphyseal aclasis 20%
Sarcomatous change usually low grade
Evidence of transformation to chondrosarcoma
1. Cartilaginous cap thicker than 1 cm in adult (in child may be 2-3 cm thick)
2. Cartilage cap >8 cm in diameter
3. Fluffy outline
4. Bone scan - marked increase in uptake in adult
5. CT/MRI - soft tissue mass or displacement of major neurovascular bundle
Enchondroma
Benign tumour of cartilage originating within medullary cavity

Periosteal form originates in periosteum & erodes into cortex
Incidence
Accounts for 10% of benign bone tumours

Long bone chondromas usually >30 yrs
>50% occur in small bones of hands & feet
15% in femur & 12% in humerus
May be solitary or multiple (Ollier's - multiple enchondromatosis, Mafucci's - multiple
enchondromas & hemangiomas)
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Clinical
Most are asymptomatic

Usually metaphyseal
75% solitary
Present with pathological # (60%), lump or as incidental finding
X-rays
Flecks of calcification - sometimes called 'ground glass'

Scalloped erosions on endosteal surface
Cortex remains intact unless #
Periosteal form (juxtacortical) -> shallow crater lined by rim of mature reactive bone, lifts
periosteum
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Enchondroma (typical appearance & site)
Periosteal enchondroma (only differentiate from

parosteal or periosteal osteosarcoma on MRI & biopsy)
Pathology
Macroscopically - bluish white, well demarcated, well encapsulated & often lobulated gritty tissue
Microscopically - hypocellular; nests of mature cartilage cells, nuclei are small & uniform, no
atypia & there may be calcification
Need to section all areas of specimen as sarcomatous change may occur in a benign lesion
Periosteal form less common & has similar pathology but more cellular than usual for a benign
lesion
Ollier's disease -> more cellular & 50% malignant transformation
Mafucci's disease -> a/w multiple haemangiomata & nearly 100% malignant change somewhere
Treatment
Observe - X-ray 6/12 & 1 yr after presentation

Curettage & grafting if latent
If active -> recurrence but this may be better than morbidity of en bloc excision
Periosteal form -> en bloc excision (with a margin)
Prognosis
Risk of malignant change in Ollier's - 50%

Malignant change in Mafucci's - nearly 100%
Multiple enchondromatosis -> increased risk of visceral malignancies, eg. astrocytomas, GI
malignancies
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Chondroblastoma
Benign cartilage tumours centred in epiphysis
Incidence
~1% of benign bone tumours

M:F 2:1
Peak age 10-20 yrs (rare over 30 yrs)
Adult counterpart of chondroblastoma is giant
cell tumour
Clinical
Present with ache of increasing severity

Usually affects proximal humerus, proximal tibia or distal femur
Epiphyseal but may expand into metaphysis
X-rays
Open physis
Well defined area of rarefaction eccentrically placed in epiphysis or across growth plate
Thin rim of sclerotic bone
50% show central calcification
50% show linear periosteal reaction
Bone scan increased uptake at margins
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Pathology
Arises from chondroblasts

Usually active benign lesion (Stage 2)
Histology
Pinkish grey tissue, lobulated, may be haemorrhagic
Richly cellular multinucleate giant cells with polyclonal or round chondroblasts
GCT (adults)
ABC (histology similar)
Clear cell chondrosarcoma
Epiphyseal osteomyelitis
Treatment
Curettage & bone grafting (15% recurrence)

Important to avoid joint penetration because chondroblastoma cells will grow in joint fluid
Use cryotherapy if extension intracapsular to avoid excision of joint
Prognosis
Probably no chance of malignant change
Incidence

Peak age 10-30 yrs (75%)
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Clinical
Present with chronic ache

Usually eccentric metaphyseal lesions
75% lower extremity & 50% tibia
X-rays
Rounded or oval rare area

Usually eccentric
May cross growth plate
Sharp outline & sclerotic rim
Scalloped margin & thin cortex
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Pathology
Variable amounts of chondroid, fibromatoid & myxoid elements

May develop from remnant of growth plate?
Unique histological picture
Firm lobulated jelly like areas of mucoid with condensations of cells on periphery
Areas of chondroid & myxomatous tissue
Contains giant cells, macrophages & monocytes
Usually no bone osteoid
Treatment
Extracapsular marginal excision -> almost no recurrence

If skeletally immature wait until maturity
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Prognosis
Malignant change has been reported, thus where possible it should be excised
CHONDROSARCOMA
Primary malignant tumour whose cells produce cartilage matrix

May arise de novo or secondarily to existing benign cartilaginous tumour (majority)
Incidence
17% of primary malignant bone tumours

M:F 2:1
Sites
Pelvis 30%
Femur 20%
Femoral head 10%
Ribs 10%
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Clinical
Most common malignant tumour of hands & face in middle aged patients
Usually occurs in metaphysis or diaphysis
Presents with constant ache or increased size of pre-existing lump
Metastatic deposits infrequent & usually go to lung
X-rays
Variable appearance with 60-70% have calcification & 50% have subperiosteal new bone
May be a large cystic lesion with cortical destruction & central calcification, endosteal scalloping &
cortical expansion
Popcorn lesions (rings, arcs, stipples)
Chondrosarcoma can also be classified as
Intramedullary, which generally arise from enchondroma
Patients with Ollier's disease (multiple enchondromatosis) or Maffucci's
syndrome (multiple enchondromas & hemangiomas) are at much higher risk of
chondrosarcoma than normal population
Surface, which arise from osteochondroma
Malignant change in osteochondroma: increased size, fuzzy outline, cartilage cap
>1 cm thick, base >6 cm diameter
Pathology
Cellular pleomorphism & increased cellularity with focally calcified matrix
X-ray & CT of a chondrosarcoma involving the right hemipelvis & sacrum.
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Chondrosarcoma involving the ischium of
the pelvis. The tumour is composed of
lobulated glistening white to bluish-white
tissue that breaks through the cortex. Note
the extensive nodules of white to bluishwhite cartilaginous tumour tissue eroding
and extending outward from the bone.
Low power histology. The tissue is

recognizable as cartilage, and there are
chondrocytes in clear spaces, but there is
no orderly pattern. At the bottom, this
neoplasm can be seen invading and
destroying bone.
Treatment
These tumours tend to metastasise late therefore attempt wide local excision initially
However, relatively resistant to chemotherapy & radiotherapy
Chemotherapy for occasional grade 3 dedifferentiated tumors
Radiotherapy useful for Rx of surgically inaccessible sites
Prognosis
Dependant on grade
>90% grade 1 or 2
Low grade - 65-85% 5-yr survival
High grade - 15-25% 5-yr survival
Mesenchymal chondrosarcoma
Rare
Usually occurs in ribs or jaw
Age usually 10-30 yrs
Sheets of small poorly differentiated cells resemble Ewing's but with focal areas of chondroid
matrix
Metastasis usually to lung
Clear Cell Chondrosarcoma
May be aggressive variant of chondroblastoma

Affects upper ends of long bones, usually upper femur
Well circumscribed lucent defects often with thin sclerotic border on X-rays
Pathology
Numerous cells with abundant clear vacuolated cytoplasm
Scattered giant cells & scant chondroid matrix
Locally aggressive
May be confused with renal clear cell tumour
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Fibrous Forming Bone Tumours

Fibrous Cortical Defect
Fibrous Dysplasia
Fibrosarcoma
Fibrous Cortical Defect (Non-ossifying fibroma)
Results from defect of periosteal cortical bone development which leads to failure of ossification
Lesion typically develops in childhood & adolescence
Non ossifying fibroma is an active stage 2 lesion that persists or enlarges throughout childhood
With skeletal maturation, NOF becomes latent & either regresses or ultimately ossifies
Incidence

Children & adolescents
M>F
Clinical
Usually incidental finding in children

Most heal spontaneously
Larger ones may -> pathological # (common presentation)
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X-rays
Metaphyseal
Eccentric
Lucent lesion, with a distinct multilocular appearance
In cortex of a long bone
Margin well defined, sometimes scalloped & often sclerosed
Histology
Whorled fibrous tissue, foam cells

Occasionally, small elongated giant cells
Malignant fibrous histiocytoma

Eosinophilic granuloma
Osteosarcoma
Histiocytic lymphoma
Pyogenic osteomyelitis
Treatment
Most spontaneously resolve or move to diaphysis of bone with growth (= UBC, osteochondroma)
Pathological #
Closed Rx - Rx of choice in most cases
Usually heal with normal amount of callus, but resolution of fibroma may or may not occur
Intracapsular curettage usually sufficient to promote healing of lesion, however, defect may be
supplemented with bone grafts +/- stabilisation
Jaffe-Campanacci syndrome
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Multiple FCDs
Cafe-au-lait spots
Mental retardation
Hypogonadism
Ocular & cardiovascular abnormalities
FIBROUS DYSPLASIA
Definition
Normal medullary bone is replaced by variable amounts of structurally weak fibrous & osseous
tissue
? developmental hamartoma
Incidence
5-20% of benign bone lesions

Relatively common & usually monostotic
Affects children & adolescents
Median age at onset 8 yrs
M > F (Albright - F > M)
Sites
Any bone
Ribs commonest (40%)
Lower limbs > upper limbs
Craniofacial -> skull deformity
Epiphyses usually spared
Prevalence of malignant transformation (chondrosarcoma or osteosarcoma) ~0.4 %
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Clinical
May be monostotic or polyostotic

Polyostotic -> pain, # (85%), deformity & skin pigmentation (coast of Maine)
McCune-Albright Syndrome
Polyostotic disease (unilateral usually)
Skin pigmentation
Cafe au lait spots with serrated borders (called "coast of Maine") that tend to stop
abruptly at midline of body
Precocious puberty (endocrinopathy)
Usually presents earlier, may be unilateral or widespread, affecting long bones, hands,
feet & pelvis
Malignant transformation (chondrosarcoma or osteosarcoma) ~4%
X-rays
Variable appearance
Intramedullary diaphyseal lesion that blends with expansion of cortex
Ground glass or hazy appearance typical
Sclerotic margin with no discernable matrix
Serpinginous margin (scalloping)
No periosteal reaction
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Angular deformity in bone often present at level of lesion, eg. Shepherd's crook deformity of
proximal femur
Pathology
Bone replaced by firm, whitish tissue of gritty consistency
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Vascular tumour with poorly orientated bone trabeculae separated by fibrous tissue
Bone is woven rather than lamellar
Lack of osteoblastic rimming of trabeculae
Paget's disease
FCD
Hyperparathyroidism
Osteoblastoma
Osteosarcoma
Treatment
Monostotic -> curettage & grafting if symptomatic

Polyostotic -> symptomatic Rx
May require osteotomy for deformity or lengthening/shortening procedures
Prognosis
Monostotic lesions cease activity at puberty but may be reactivated by pregnancy

Polyostotic - 85% -> pathological #
Malignant change occurs after radiotherapy
Cell of origin controversial - histiocytic cell, fibroblasts, multipotent mesenchymal cell

20% occur secondary to pre-existing condition
Paget's disease
Bone infarct (eg. sickle cell)
Fibrous dysplasia
Long standing OM
Irradiated bone
Incidence
Occur less often in bone than in soft tissue; 5% of primary malignant bone tumours
Age >30 (often >50)
M>F
40% occur around knee
Metastasises to lung, & other bones via blood
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Classification
Myxoid
Non myxoid
Clinical
Present with pain, swelling

Usually presents as aggressive stage IIB sarcoma
15% present with pathological #
X-rays
Usually metaphyseal
Purely lytic lesion with ill-defined margins

Bone often mottled or moth eaten with extension into soft tissue
Osteolytic lesion may be surrounded by reactive bone
Usually little periosteal reaction
Pathology
Discrete greyish white rubbery tumour

Irregular bony margins
Composed of fibroblasts, myofibroblasts & large foamy histiocytes
Variable amount of fibrous component in a "storeiform pattern" (radiating)
Metastatic carcinoma
Treatment
Radical excision or amputation

Radiotherapy if not amenable to resection
No randomised studies but reports of increased survival with neoadjuvant chemotherapy
(doxorubicin, vincristine, methotrexate, T10)
Prognosis
Better in young
If initial procedure a wide or radical excision: >80% 4-yr survival
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FIBROSARCOMA
Incidence
2% primary malignant bone tumours

M=F
25% have metastasised at presentation
X-rays
Osteolytic lesion
Margins can range from well-defined to ragged & moth-eaten
Periosteal reaction seen with cortical destruction
Extension into soft tissue common
Metastatic carcinoma
Multiple myeloma
MFH
Leiomyosarcoma
Treatment
Stage IA - limb salvaging excision with wide margin
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Stage IIB - radical or wide margins with adjuvant chemotherapy or radiation therapy
Classically considered radio-resistant
May however be useful as adjuvant to surgery & chemotherapy
Useful for irradiation of lung secondary deposits not accessible to surgery
Prognosis
Stage 2 guarded
MULTIPLE MYELOMA (MM)
Malignant tumor of plasma cells that causes widespread osteolytic bone damage
Incidence
Most common primary malignant tumor of bone (~40%)

May affect any bone with haematopoietic red marrow (spine, skull, ribs, sternum & pelvis)
Age 50-80 yr
M:F = 2:1
Presentation
Bone pain related to deposits

Pathological #
Constitutional symptoms related to anaemia, thombocytopenia & renal failure
Other symptoms may include cachexia, spinal cord compression
Amyloidosis in 20%
Bacterial infections common because of lack of normal immunoglobulin production
Investigations
FBC - normochromic, normocytic anaemia

ESR raised ++ (often >100 mm/hr)
Ca & ALP
Hypercalcaemia (20-40%)
Alkaline phosphatase
Marker of osteoblast activity
Usually not increased since little new bone formation, which explains why bone
scans appear cold
Monoclonal immunoglobulin found on serum electrophoresis (90%)
Bence-Jones proteins (light chain subunits of immunoglobulin) in urine (50%)
Radiology
X-rays
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Characterised by irregular lytic defects of different sizes, described as "punched out" &
no periosteal reaction
Over time lesions may change from diffuse osteopenia to more permeative moth-eaten
destructive pattern
Bone destruction occurs with little or no reactive bone formation
Skeletal survey most sensitive Ix
Bone scan can fail to have increased uptake in 25%
MRI useful for delineating spinal lesions
Histology
Biopsy reveals sheets of densely packed plasma cells

>20-30% plasma cells on biopsy diagnostic of myeloma
Degree of cytological atypia has no prognostic value
Osteolytic lesions are caused by increased osteoclastic resorption that is stimulated by cytokines
released by plasma cells
Treatment
Radiotherapy
MM is sensitive to radiotherapy, & reossification of tumour defects may occur within
several months
Recommended for intractable bone pain -> can be dramatically effective in relieving
symptoms
Chemotherapy
Palliative only
Surgery
Prophylactic IM nails for femoral, humeral deposits
ORIF of other pathological #
15% may need spinal decompression due to deposits or #
Bisphosphonates useful in Rx of hypercalcaemia
Prognosis
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Untreated patient with bony lesions will only survive an average of 6-12/12 with cause of death
usually infection or haemorrhage
Improved survival following chemotherapy
Median survival 12-36/12, usually <2 yrs
Solitary lesions - 60% 5-yr survival
Multiple lesions - 5% 5-yr survival
Lymphoma of bone (NON-HODGKIN'S)
Known in the past as reticulum cell sarcoma
Incidence
<5% of primary malignant bone tumors

>20% of patients with lymphoma have secondary bone involvement
Most intraosseous lesions -> non-Hodgkin's lymphoma
Usually middle aged or elderly
40-50% occur around knee; other bones: femur & pelvis
Clinical
Present with pain & swelling

Pathological # may occur
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X-rays
Early -> vague mottled lucent areas

Diffuse destructive lytic lesion with little periosteal reaction
Usually combination of patchy sclerosis & mottled destruction
Plain radiographs often underestimate extent of lesion
Hodgkin's disease -> typical appearance of ivory vertebrae
Osteosarcoma
Ewing's sarcoma
Metastatic Ca
Osteomyelitis
Pathology
Histologically sheets of poorly differentiated cells with irregular nuclei

Usually composed of cells of a mixture of types, reticulum cells, lymphocytes & lymphoblasts
Hodgkin's -> Reed-Sternberg cells histologically (large, sharply delineated cells with abundant
cytoplasm & a double nucleus)
Investigations
FBC & PBF TRO leukaemia

BMA
CT
Abdominal exploration -> splenectomy -> staging
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Treatment
Surgery - wide excision

Radiotherapy for localised lesions
Chemotherapy for systemic involvement
Prognosis
Lymphoma of bone has best prognosis of all primary malignant bone tumors
44% 5 yr survival
Pure Hodgkin's disease or lymphocytic disease -> worse prognosis
Soft Tissue Tumours

INCIDENCE
Benign soft tissue tumours common

Malignant ones rare
Pathological Classification
Benign
Malignant
Reactive tumour like lesions
Tissue of origin
Fibrous
Fibrohistiocytic
Fat
Benign
Fibroma
Malignant
Fibrosarcoma
Nodular fasciitis
Postradiation
fibrosarcoma
Proliferative fasciitis
Fibrous histiocytoma
Atypical
fibroxanthoma
Lipoma (cutaneous,
deep or multiple)
Malignant fibrous
histiocytoma
Reactive tumour like lesions

Palmar & plantar superficial
fibromatoses
Deep extraabdominal
fibromatoses
Intermediatedermatofibrosarcomaprotruberans
Liposarcoma
Angiolipoma
Spindle
cell/pleomorphic
lipoma
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Lipoblastoma
Intra & intermuscular
lipoma
Striated muscle
Smooth muscle
Hibernoma
Rhabdomyoma
Leiomyoma
Rhabdomyosarcoma
Leiomyosarcoma
Blood vessels
Angiomyoma
Haemangioma
Haemangiosarcoma
Glomus tumour
Lymph vessels
Peripheral nerves
Lymphangioma
Traumatic neuroma
Intermediatehaemangioendothelioma
Malignant
haemangiopericytoma
Lymphangiosarcoma
Malignant
schwannoma
Morton's neuroma
Neurilemmoma
(benign
schwannoma)
Peripheral tumours of
primitive
neuroectodermal
tissue
Neurofibroma
Neurofibromatosis
Synovial tissue
Extraosseous bone
& cartilage
Giant cell tumour of

tendon sheath
Myositis ossificans
Synovial sarcoma
PVNS
Malignant giant cell

tumour of tendon
sheath
Ganglia
Synovial osteochondromatosis
Extraskeletal
chondrosarcoma
Fibrodysplasia
Panniculitis
ossificans
Extraskeletal
osteosarcoma
Extraskeletal
chondroma
Uncertain
Extraskeletal
osteoma
Tumoral calcinosis
Alveolar soft part

sarcoma
Myxoma
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Epithelioid sarcoma
Clear cell sarcoma of
tendons &
aponeuroses
Extra-skeletal Ewing's
STAGING
Benign
Enneking's Stage
1. Latent/inactive
2. Active/growing & symptomatic
3. Aggressive
Malignant
Enneking's Classification
DIAGNOSTIC CLUES SSCCSI
Size
A small mass (<5 cm in its greatest dimension) is unlikely to be malignant, while a mass
>5 cm has at least 20% chance of being soft tissue sarcoma1
Size of lesion can be determined by physical exam if lesion subcutaneous & easily
palpable, or by US, CT or MRI
Superficial or deep?
2
Superficial lesions more likely to be benign &, when malignant, may have better
3
prognosis than deep lesions
Depth of lesion best determined by physical exam, US or MRI
Thigh & buttocks - 2 most common sites for soft tissue sarcomas
Any large deep mass in thigh or buttocks should be considered at high risk for being a
malignant lesion
Consistency
Soft tissue sarcomas tend to be firm & not very painful until very large & compromise
their vascular supply or adjacent neural structures
Lipomas are usually nontender & soft; deep lipoma (intramuscular or infiltrating) may feel
firm when muscular compartment is contracted
Infectious & inflammatory lesions tend to be painful to palpation, may feel warm & cause
certain amount of apprehension
Pseudoaneurysm - unusual lesions that can get very large, look like a sarcoma on
imaging studies -> pulsatile & audible bruit
Cystic or solid
Most cystic lesions are inflammatory or benign lesions, eg. ganglion cysts or soft tissue
abscesses
Solid lesion could represent either a benign or malignant neoplasm
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Attempt transillumination
If deep -> US or MRI
Length of symptoms
A mass that has rapidly increased in size over 2/12 is more likely to be a sarcoma than
lesion that has slowly enlarged over 20 yrs
A mass that increases & decreases in size is usually cystic
However, caution should be taken with masses that have been present for a long time
Soft tissue sarcomas occasionally present with a history of many years duration
Plain X-ray findings
Every soft tissue mass that is going to undergo intervention should have a plain
radiograph
Fat density lesion (lipoma)
Punctate calcifications (synovial cell sarcoma, soft tissue chondrosarcoma or
hemangioma)
Ossification (soft tissue osteosarcoma or myositis ossificans)
Skeletal abnormalities (osteomyelitis, primary bone lesion or periosteal reaction from soft
tissue tumor)
MR scan findings
MRI gives the most information of any radiographic study but should be reserved for
large lesions or those that are ill defined
Clearly delineate whether lesion is bony with very large soft tissue component or whether
lesion is a primary soft tissue lesion
Low T1 & low T2 sequence - either extra-abdominal desmoid tumor, extensive scar
tissue, cortical or dense bone or foreign material eg. bone cement or air
High T1 & high T2 sequence - most likely lipoma, liposarcoma, haemangioma
DIAGNOSTIC STEPS
History: painless swelling (bone tumours: pain -> swelling)

Examination +/- transillumination
Plain radiography & US
Subcutaneous lesion
<5 cm (lipoma, cyst, inflammatory)
Observe
If patient keen on removal, excisional biopsy (longitudinal incision, good
haemostasis, ensuring incision can be incorporated in later excision)
>5 cm
Biopsy if not lipoma
Deep lesion
<5 cm (lipoma, schwannoma)
>5 cm, or not cystic, or painful
MR scan, then refer to tumour specialist for incisional/trucut needle biopsy/fine
needle aspiration
If possibility of malignancy exists
Isotope bone scan for other lesions
FBC, ESR
Chest X-ray
CT scan of chest, for metastases
See Principles of Tumours
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TREATMENT
Principles
Local control of soft tissue sarcomas with
Surgical resection or amputation
Radiation therapy
Combination of both
If lung metastases present, systemic control with chemotherapy
Use of adjuvant chemotherapy for nonmetastatic soft tissue sarcomas controversial because it
has not been shown to be very effective in improving survival & is a/w significant morbidity & cost
Local recurrence common
See Principles of Tumours
PROGNOSTIC FACTORS
Stage (Enneking's stage)

Metastatic disease from soft tissue sarcomas is most frequently identified in lungs, less
frequently in draining lymphatics & skeleton
Standard staging studies include physical exam of lymph nodes, CXR, chest CT, whole
body bone scan & possibly gallium scan
Patient with non-metastatic disease at presentation has far better prognosis than one
with metastatic disease
Histologic grade
High-grade lesions have worse prognosis
Size of lesion
Although small lesions (<5 cm) are rarely malignant, when they are, they have better
prognosis than larger lesions
Size cut-off is somewhat arbitrary, but small is considered <5-8 cm in most studies
Depth of lesion
Superficial (subcutaneous) soft tissue sarcomas have better prognosis than deep (below
muscle fascia) lesions
Age
Surgical margins
Lung metastases resection
SPECIFIC SOFT TISSUE TUMOURS

Benign fibrous tumours
1. Calcifying aponeurotic fibroma
Slow growing, painless mass

Ages 3-30
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X-rays: faint mass with stippling

Histology: fibrous tumour with some calcification & cartilage formation
50% recurrence after excision
Resolves with maturity
2. Nodular fasciitis
Painful reactive rapidly enlarging lesion in young person

50% in upper limbs
Histology: short irregular bundles & fascicles, only small amounts of mature collagen
Treat with excision with marginal resection
3. Fibromatosis
Refers to multiple benign fibrous tumors which are locally aggressive

Although generally superficial, tumors are locally invasive, frequently involving adjacent
neurovascular structures
More aggressive than solitary fibroma, but does not metastasize
Often develop in proximal limbs or trunk, rarely occurs distally
In hand: Dupuytren's
In foot: plantar fibromatosis
In abdomen: desmoid tumors
Palmar (Dupuytren's) & plantar (Ledderhose) fibromatosis
Consists of firm nodules of fibroblasts & collagen
Nodules & fascia hypertrophy -> contractures
Extraabdominal desmoid tumour
Most locally invasive of benign soft tissue tumours
Most common in adolescents & young adults
Rock hard on palpation
May be multiple lesions
Histologically well differentiated fibroblasts & abundant collagen infiltrating surrounding
tissues
Rx
Nonoperative
Begins with construction of a well molded, padded shoe & orthosis
Transference of weight away from prominent nodules important
Radiotherapy may be used as an adjunct to surgical excision in Rx of aggressive
fibromatosis
Surgical
Indications
Major indication: pain
Nodules become large & painful enough to be disabling on
standing/walking
Plantar fibromatosis
Tumors may involve neurovascular structures as well as infiltrating
surrounding muscle
Care must be taken to place incision away from weight-bearing region
Use a longitudinal medial incision, S-shaped incision on plantar surface
of foot
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Incision can be made 2 cm posterior to head of 1st metatarsal, with

extension posterolaterally, then posteromedially, & again
posterolaterally, finishing anterior to weight-bearing surface of calcaneus
Total excision of fascia necessary to reduce chance of recurrence
Resection & recurrence
Recurrence after incomplete excision very common
Rx of choice: excision with a wide margin
Even if this will require SSG
Use at least 2 cm margin of normal fascia proximal & distal to
nodule
Recurrence are difficult to distinguish from scarring of previous excisions,
thus making excision even more hazardous
Adjuvant radiotherapy reduces recurrence rate after marginal or even
after intracapsular excision
Malignant fibrous tumours

1. Fibrosarcoma
Clinical (similar to MFH)

Age group 30-80
Enlarging painless mass
Usually 10 cm in size before symptoms
Radiological (similar to MFH)
Plain X-ray: usually normal unless encroaching on bone -> erosion/destruction
MRI: deep-seated inhomogenous mass
Histology slightly different
Fasciculated growth pattern with fusiform or spindle shaped cells, scanty cytoplasm,
indistinct borders
Cells separated by interwoven collagen
May have herring bone appearance
Rx (similar to MFH)
Wide local excision
If >5 cm, add radiotherapy, preop +/- postop +/- periop
2. Malignant fibrous histiocytoma (Pleomorphic sarcoma)
Malignant cells are of histiocytic derivation

Incidence
Most common soft-tissue sarcoma in adults
Age group 30-80
Histology type & prognosis
Pleomorphic (commonest) - 55% 5-yr mortality
Giant cell - 55%
Myxoid - 23%
Inflammatory -33%
Clinical
Enlarging painless mass (painful mass in osseous tumour)
Usually 10 cm in size before symptoms
Early osseous invasion & metastases to regional lymph nodes common
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Paraneoplastic syndromes
Eosinophilia
Hypoglycemia
Fever
Abnormal LFT
Radiological
Plain X-ray
Usually normal
If encroaching on bone -> erosion/destruction
MRI
Deep-seated inhomogenous mass
Mixed density signal
Areas of internal hemorrhage
Areas of myxoid density (signal)
Occasionally lobular growth
Pathology
A pleomorphic, high grade, bimodal sacroma in which only differentiating feature is
collagen
Spindle & histiocytic cells arranged in storiform (cartwheel) pattern
Short fascicles of cells & fibrous tissue that appear to radiate about a common centre
around slit-like vessels
Chronic inflammatory cells may be present
Rx
Wide local excision
Stage I tumors usually develop in more peripheral & superfical locations than
high grade tumors
Wide surgical excision usually adequate
Amputation reserved for multiple recurrences
Stage II tumors require excision with wide margins or amputation
If >5 cm, add radiotherapy, preop +/- postop +/- periop
3. Dermatofibrosarcoma protruberans
Rare, nodular cutaneous tumour

Occurs in early adult life
Intermediate in grade
Recurs locally but only rarely metastasises
Treat with wide resection
Benign fatty tumours

1. Lipomas
Subcutaneous/intramuscular or intermuscular tumours of mature fat

Most are not painful
Plain X-rays may show a radiolucent region in soft tissues
MR or CT scan show a well demarcated lesion with exactly same signal as fat
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If no symptoms & definite radiological diagnosis, leave alone

If mass growing or causing symptoms excise with a marginal line of resection
Recurrence uncommon
Subgroups: spindle cell lipoma & pleomorphic lipoma
Malignant fatty tumours

1. Liposarcomas
Malignant tumours with differentiation towards fatty tissue

Heterogenous group of tumours with presence of signet ring type cells (lipoblasts) in common
Lipoma like, sclerosing, inflammatory, dedifferentiated, myxoid, round cell, pleomorphic
Range from low grade to high grade
Can be difficult to differentiate between a benign lipoma & a low grade liposarcoma
Low grade liposarcomas treated with wide local excision +/- radiotherapy
High grade liposarcomas treated with wide local excision + radiotherapy
Benign peripheral nerve tumours

1. Neurilemmoma (benign schwannoma)
Benign nerve sheath tumour

Young to middle aged patients
Usually asymptomatic apart from mass
MR: eccentric mass arising from a peripheral nerve
Histologically contains Antoni A or Antoni B cells
Antoni A - compact spindle cells, twisted nuclei, indistinct cytoplasm, clear vacuoles
Antoni B - less cellular
Treat by excision leaving nerve intact
2. Neurofibroma
Solitary or multiple (see neurofibromatosis)

Most are superficial, grow slowly & painless
When they involve a major nerve they can expand nerve in a fusiform fashion
Histologically, interlacing bundles of elongated cells with wavy dark staining nuclei
Rx - excision with a marginal line
In neurofibromatosis, malignant change occurs in 5-30% of patients
Malignant peripheral nerve tumours

1. Neurofibrosarcoma
Rare
Can arise de novo or in neurofibromatosis
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Tend to be high grade, therefore treated with wide surgical resection +/- radiotherapy
Benign tumours of muscle tissue
Leiomyoma, rhabdomyoma
Seldom occur in extremities
Malignant tumours of muscle tissue

1. Leiomyosarcoma
Can be high or low grade

May be a/w blood vessels
Wide/radical surgical excision & radiotherapy
2. Rhabdomyosarcoma
Most common sarcoma in young patients

Highly malignant
Grows rapidly
Histology
Spindle cells in parallel bundles, multinucleated giant cells & racquet shaped cells
Cross striations within tumour cells (rhabdomyoblasts)
Sensitive to multiagent chemotherapy
Treat with preop chemo, followed by wide surgical excision & radiotherapy
Benign vascular tumours

1. Haemangioma
Seen in children & adults

Regresses with age
Can be cutaneous, subcutaneous, intramuscular
If large, patients complain due to symptoms of venous engorgement (aching, heaviness, swelling)
Plain X-ray can show small pleboliths
MR scan shows a heterogenous lesion with many small blood vessels
Treat nonoperatively if possible
Wide surgical resection if symptomatic
Local recurrence rate high
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Malignant vascular tumours

1. Haemangiopericytoma
Rare tumour of pericytes of blood vessels

Can be benign or malignant & from intermediate to high grade
Slowly enlarging painless mass
Rx based on grade of lesion
2. Angiosarcoma
Rare
Tumour resembles endothelium of blood vessels
Rx depends on grade & location of lesion
Benign synovial tumours

1. Ganglia
Malignant synovial disorders

1. Synovial sarcoma
Highly malignant tumour derived from synovial tissues found along fascial planes, periarticular
structures, & rarely, in joints
May involve sheaths & bursae of tendons
Incidence
4th most common soft-tissue sarcoma (5-10% of all cases)
Most often occurs in adolescents & young adults
Typically arises in legs & knee
Occurs in close proximity to joints but rarely from an intraarticular lesion
Clinical
Slowly enlarging, painless juxtaarticular mass
Usually presents as stage IIB lesion in lower limbs
May presents as stage I tumor in hands or feet where it may be confused with a ganglion
Evidence of regional lymph node involvement strongly supports diagnosis
Metastasizes to lymph nodes, bones, & lungs
Radiology
X-rays
Hazy, soft tissue density with discrete intrinsic calcifications in 30% of cases
Periosteal reaction or even bone erosion or invasion
Differential diagnosis: spotty calcification may indicate chondroma or
hemangioma
Bone scan: marked radioisotope uptake
MRI: lesion is often adjacent to major neurovascular structures
Histology
2 forms
Biphasic with a spindle cell component & an epithelial component
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Monophasic can be of either epithelial-cell or spindle-cell type

Treatment
Low grade lesions: wide excision
High grade tumors: either radical resection or wide surgical excision + radiotherapy
Tumors >8 cm: consider administering chemotherapy & radiotherapy
Radiotherapy may provide local control & can create a pseudocapsule around tumour
Chemotherapy for metastatic disease
Prognosis
High recurrence rate
5-yr survival rate 25-55%
Synovial proliferative disorders

1. Pigmented villonodular synovitis
Not a true neoplasm

Exuberant proliferation of synovial villi & nodules
Most common in knee, followed by hip & shoulder
Symptoms include painful swollen joint
Aspiration reveals a bloody effusion
X-ray may show cystic
Histologically, highly vascular villi lined with plump hyperplastic synovial cells, hemosiderin
stained multinucleated giant cells & chronic inflammatory cells
Treat with complete synovectomy
Local recurrence common
More Detail
2. Giant cell tumour of tendon sheath (see Hand Tumours)
Benign but highly recurrent lesion originating in tendon sheaths or joint synovium
Usually seen on palmar surface of digits especially PIP joint of index & middle fingers
Recurrence rate of 10%
2nd most common hand mass
3. Synovial chondromatosis
Typically affects young adults complaining of pain, stiffness & swelling

X-rays show fine stippled calcification
Histologically varies between metaplasia of synovial tissue to firm nodules of cartilage
Treat with synovectomy & removal of loose bodies
More Detail
Other rare Sarcomas

1. Epitheloid Sarcoma
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Nodular tumour occuring in hands of young adults, buttock, thigh, knee or foot
May ulcerate & mimic a granuloma or rheumatoid nodule
Lymph node metastases may occur
Histologically ovoid to polygonal with eosinophilic cytoplasm
Wide surgical excision required
2. Clear cell sarcoma
Slow growing painless mass in young adults

In region of tendons or aponeuroses
Treat with wide surgical resecion with adjuvant radiotherapy
3. Alveolar Cell Sarcoma
Most common in anterior thigh

Treat with wide surgical excision & radiotherapy
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Oncology

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Oncology

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Sholahuddin Rhatomy MD

Staging may be useful for

ENNEKING'S SURGICAL STAGES

Low (G1) or High (G2)

Intracompartmental (T1) or Extracompartmental (T2)

Grade (biological aggressiveness)

Malignant Fibrous Histiocytoma

Giant cell tumour

Clear cell tumour of tendon sheath

Site (anatomic setting)

Soft tissue extension

Deep fascial extension

Extrafascial planes/spaces: (neurovascular containing spaces)

ray of hand or foot

mid & hind foot/mid hand

posterior or anterior leg

ant, med, post thigh

groin - femoral triangle

volar or dorsal forearm

anterior or posterior arm

Metastasis (nodal or blood borne tumour spread)

M0: No evidence of regional or distant metastases

Diagnosis in 85% of bone sarcoma

Benign lesions suggested if

Good soft tissue definition (better than CT)

Gold standard for primary staging

Indicates extent of bony involvement/destruction

Biopsy of Bone Tumours

Final step in staging

Biopsy tract orientation & location critical

Excisional biopsy when possible in benign lesions

As for open biopsy

Place biopsy tract where it can be excised

Able to determine if specimen is adequate or representative

Common biopsy sites

Anterior deltoid compartment

Goal of Rx: to remove lesion with minimal risk of local recurrence

No matter what type of surgery, if premorbid personality normal, good psychosocial

Surgical Margins (Musculoskeletal Tumour Society)

Multi-agent chemotherapy has significant impact on limb salvage

If <90% -> change agents

Commence adjuvant Rx once wound has healed

For local control of

Erythema of skin & hyperpigmentation

Unicameral (Simple) Bone Cyst (UBC)

Benign lesion which occurs during growth

20% of benign bone lesions

Depending on location: fibrous dysplasia, ABC

Well defined, central osteolytic area with thin sclerotic margin

CT not helpful unless UBC in pelvis

Curettage & bone graft

Aneurysmal Bone Cyst (ABC)

Benign solitary, expansile & erosive lesion of bone

1% of benign bone lesions

Swelling, tenderness & pain

Osteolytic lesion placed eccentrically in metaphysis

Rx approach will vary depending of location & aggressiveness of lesion

Rates vary widely

Giant Cell Tumour (GCT)

10% of benign bone lesions

Lesion confined within bone

Lesion expanding cortex

? arises from mesenchymal cells of connective tissue framework

Due to proximity to articular cartilage, excision of GCT of bone difficult