Oncology PDF

Sholahuddin Rhatomy MD
ORTHOPAEDIC
ONCOLOGY
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1. Tumor principle…3
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 tumor…68
34. Malignant fibrous tumor 72
35. Benign fatty tumor…73
36. Malignant fatty tumor 73
37. Benign peripheral nerve tumor…74
38. Malignant peripheral nerve tumor 74
39. Benign muscle tumor…75
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
2 Active G0 T0 M0
3 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. Frequent mitoses
4. Extensive necrosis
5. Significant vascularity
6. Small amounts of immature matrix
• Examples
LOW (G1) HIGH (G2)

Parosteal osteosarcoma Classic osteosarcoma
Low-grade medullary
Radiation osteosarcoma
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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o 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
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
• 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
1. 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)
2. 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
3. Function
• NONE of reconstructions will restore a functionally normal limb -> always
partially disabled
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• 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
4. 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 & disease-free
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
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

2 Lesion expanding cortex
3 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
Orthopaedi UI 25
• 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 Osteosarcoma
Osteoblastoma
Cartilage Producing Tumours Osteochondroma Chondrosarcoma
Enchondroma
Chondroblastoma
Chondromyxoid Fibroma
Fibrous Bone Tumours Fibrous Cortical Defect Malignant Fibrous Histiocytoma
Fibrous Dysplasia Fibrosarcoma
Unknown origin Giant Cell Tumour Ewing's sarcoma

Malignant GCT
Adamantinoma
Reticuloendothelial Tumours Eosinophilic Multiple Myeloma
granuloma/Histiocytosis X Lymphoma of bone
Vascular Tumours Haemangioma Haemangioendothelioma
Haemangiopericytoma
Notochordal Chordoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemoma
Orthopaedi UI 26
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
Orthopaedi UI 27
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 #
Orthopaedi UI 28
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
• Electrode connected to radiofrequency generator which raises temperature of tip to 90 C
o
(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
Orthopaedi UI 29
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)
Orthopaedi UI 30
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 Osteoblastoma

Incidence Common Rare
Clinical Pain relieved by aspirin Pain partially relieved by aspirin
Nidus <1-2 cm
Nidus 3-10 cm
Pathology Woven bone
Sheets of woven bone
Surrounded by reactive bone
Small central nidus surrounded by
X-ray Lucent or dense lesion
dense reactive bone
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
Orthopaedi UI 31
• 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
Orthopaedi UI 32
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
Orthopaedi UI 33
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
Orthopaedi UI 34
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
Orthopaedi UI 35
• 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 (5-
10%)
• 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 long-
term survival in ~20%
• Pulmonary tumor resection requires good local disease control & no extra-
pulmonary 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
Orthopaedi UI 36
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
Orthopaedi UI 37
1. Parosteal - tend to be more low grade tumours ? -> better prognosis

2. Intraosseous (classical) osteosarcoma -> good prognosis
3. Location (proximal worse than distal, pelvis, spine)
4. Size of primary tumour (big is bad)
5. Stage
6. >16 metastatic deposits -> poor prognosis
7. Origin of tumour in pre-existing lesion -> worse prognosis
8. 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
Orthopaedi UI 38
• 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
Orthopaedi UI 39
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
Orthopaedi UI 40
• 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
Orthopaedi UI 41
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
Orthopaedi UI 42
• 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
Orthopaedi UI 43
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
Orthopaedi UI 44
• 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)
Orthopaedi UI 45
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
Orthopaedi UI 46
Periosteal enchondroma (only differentiate from

Enchondroma (typical appearance & site)
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
Orthopaedi UI 47
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
Orthopaedi UI 48
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%)
Orthopaedi UI 49
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
Orthopaedi UI 50
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
Orthopaedi UI 51
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%
Orthopaedi UI 52
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.
Orthopaedi UI 53
Chondrosarcoma involving the ischium of

Low power histology. The tissue is
the pelvis. The tumour is composed of
recognizable as cartilage, and there are
lobulated glistening white to bluish-white
chondrocytes in clear spaces, but there is
tissue that breaks through the cortex. Note
no orderly pattern. At the bottom, this
the extensive nodules of white to bluish-
neoplasm can be seen invading and
white cartilaginous tumour tissue eroding
destroying bone.
and extending outward from the 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
Orthopaedi UI 54
Fibrous Forming Bone Tumours

Fibrous Cortical Defect
Fibrous Dysplasia
Malignant Fibrous Histiocytoma
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)
Orthopaedi UI 55
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
Orthopaedi UI 56
• 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 %
Orthopaedi UI 57
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
Orthopaedi UI 58
• 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
Orthopaedi UI 59
• 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
Malignant Fibrous Histiocytoma

• 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
Orthopaedi UI 60
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
Orthopaedi UI 61
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

Orthopaedi UI 62
• 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
Orthopaedi UI 63
• 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
Orthopaedi UI 64
• 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
Orthopaedi UI 65
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
Orthopaedi UI 66
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 Benign Malignant Reactive tumour like lesions

Fibrous Fibroma Fibrosarcoma Palmar & plantar superficial
fibromatoses
Nodular fasciitis Postradiation
fibrosarcoma Deep extraabdominal
Proliferative fasciitis fibromatoses
Fibrohistiocytic Fibrous histiocytoma Malignant fibrous Intermediate-
histiocytoma dermatofibrosarcomaprotruberans
Atypical
fibroxanthoma
Fat Lipoma (cutaneous, Liposarcoma
deep or multiple)
Angiolipoma
Spindle
cell/pleomorphic
lipoma
Orthopaedi UI 67
Lipoblastoma
Intra & intermuscular

lipoma
Hibernoma
Striated muscle Rhabdomyoma Rhabdomyosarcoma
Smooth muscle Leiomyoma Leiomyosarcoma
Angiomyoma
Blood vessels Haemangioma Haemangiosarcoma Intermediate-
haemangioendothelioma
Glomus tumour Malignant
haemangiopericytoma
Lymph vessels Lymphangioma Lymphangiosarcoma
Peripheral nerves Traumatic neuroma Malignant
schwannoma
Morton's neuroma
Peripheral tumours of
Neurilemmoma primitive
(benign neuroectodermal
schwannoma) tissue
Neurofibroma
Neurofibromatosis
Synovial tissue Giant cell tumour of Synovial sarcoma PVNS

tendon sheath
Malignant giant cell Ganglia
tumour of tendon
sheath Synovial osteochondromatosis
Extraosseous bone Myositis ossificans Extraskeletal

& cartilage chondrosarcoma
Fibrodysplasia
Extraskeletal
Panniculitis osteosarcoma
ossificans
Extraskeletal
chondroma
Extraskeletal
osteoma
Uncertain Tumoral calcinosis Alveolar soft part
sarcoma
Myxoma
Orthopaedi UI 68
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?
• Superficial lesions more likely to be benign &, when malignant, may have better
2
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
Orthopaedi UI 69
• 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
Orthopaedi UI 70
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
Orthopaedi UI 71
• 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
Orthopaedi UI 72
• 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
Orthopaedi UI 73
•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
Orthopaedi UI 74
• 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
Orthopaedi UI 75
• 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
Orthopaedi UI 76
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
Orthopaedi UI 77
• 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
Orthopaedi UI 78
• 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
Orthopaedi UI 79

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Sholahuddin Rhatomy MD

• Staging may be useful for

ENNEKING'S SURGICAL STAGES

IB Low (G1) Extracompartmental (T2) None (M0)

IIB High (G2) Extracompartmental (T2) None (M0)

Grade (biological aggressiveness)

LOW (G1) HIGH (G2)

Site (anatomic setting)

ray of hand or foot mid & hind foot/mid hand

posterior or anterior leg popliteal fossa

ant, med, post thigh groin - femoral triangle

volar or dorsal forearm antecubital fossa

anterior or posterior arm axilla

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

Biopsy of Bone Tumours

• 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

Distal femur Medial approach

• Reconstruction options for skeletal defects

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

Surgical Margins (Musculoskeletal Tumour Society)

• If <90% -> change agents

• Commence adjuvant Rx once wound has healed

• Erythema of skin & hyperpigmentation

Unicameral (Simple) Bone Cyst (UBC)

• 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)

• 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

1 Lesion confined within bone

1 Benign latent GCT

• ? arises from mesenchymal cells of connective tissue framework

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

• Sarcomatous transformation in 5-10%

Bone Tumours - Index

Unknown origin Giant Cell Tumour Ewing's sarcoma

BONE PRODUCING TUMOURS

• Recognise matrix as woven bone (osteoid)

• 10% of benign bone tumours

• Bone island (enostosis)

• Nidus usually <1 cm diameter, most <0.5 cm

• <1% of primary bone tumours

• Less intense pain than osteoid osteoma

• Well demarcated osteolytic lesion sometimes containing flecks of calcification

• As for osteoid osteoma but increased size 2-10 cm