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BONE TUMORS
&ITS GENERAL
PRINCIPLES OF
MANAGEMENTDR.PRASHANTH KUMAR
SEC.DNB RESIDENT
HINDU RAO HOSPITAL
DELHI
Introduction :
• Neoplasia : It is defined as a mass of tissue
formed as result of abnormal, excessive,
uncoordinated, autonomous and purposeless
proliferation of cells.
• Term Neoplasia includes both Benign and
Malignant.
FEATURES BENIGN (DIFFERENTIATED) MALIGNANT (UNDIFFERENTIATED)
MACROSCOPIC FEATURES
Boundaries Encapsulated or well
circumscribed
Poorly circumscribed and irregular
Surrounding tissue Often compressed Usually invaded
Secondary Changes Occur less often Occur more often
MICROSCOPIC FEATURES
Pattern Usually resemble tissue of origin No resemblance
Nucleo – Cytoplasmic
Ratio
Normal Increased
Pleomorphism Absent usually Often present
Anisonucleosis Absent Generally present
Hyperchromatism Absent Often present
Growth Rate
Usually Slow Rapid
Metastasis Absent Frequently Present
Contrasting Differences between Benign and MalignantTumours
Routes of Metastasis :
• Haematogenous Spread : Most common spread in
musculoskeletal tumours
• Lymphatic Spread : rare in musculoskeletal tumours.
• Seen in Rhabdomyosarcoma, Synovial sarcoma,
Malignant fibrous histiocytoma .
• Direct Implantation :
– Through surgeons scalpel, needles, sutures, FNAC,
diagnostic or excision biopsy
• Spread through CSF :
– malignant tumours of ependyma and leptomeninges
rarely spread through CSF to vertebrae.
Classification of Tumours
1. WHO Classification :
widely accepted and is based on histogenesis
and histological criteria.
2. Classification based on origin of tumours
3. Classification based on site of lesions.
Modified WHO Classification of BONE TUMOURS
• 1. Bone forming tumours :
Benign :
Osteoma
Osteoid Osteoma
Osteoblastoma
Intermediate
Aggressive
Osteoblastoma
Malignant
Osteosarcoma
Conventional osteosarcoma
Telangiectatic osteosarcoma
Juxta cortical or Parosteal
Osteosarcoma
Periosteal Osteosarcoma
2. Cartilage forming Tumours
• BENIGN :
• Chondroma
• Enchondroma
• Osteochondroma
• Chondroblastoma
• Chondromyxoid fibroma
• MALIGNANT :
• Chondrosarcoma
• Juxtacortical
chondrosarcoma
• Mesenchymal
chondrosarcoma
• Clear cell
chondrosarcoma
• De differentiated
chondrosarcoma
• Malignant
chondroblastoma
3. Giant Cell Tumours :
– Osteoclastoma
– Malignant giant cell tumour
– Giant cell tumour in Pagets disease
– GCT occurring in non epithelial region
– Giant cell variants : tumors which histologically show the osteoclastic
giant cells
1 - aneurysmal bone cyst
2 osteoclastoma
3 chondroblastoma
4 unicameral bone cyst
5 chondromyxoid fibroma
6non osteogenic fibroma
7- fibrous dysplasia
8- brown tumor of hyperparathyroidism .
4. Marrow tumours
•Ewing sarcoma
•Reticulosarcoma
•Lymphosarcoma
•Myeloma
5. Vascular Tumours
– Benign:
» Haemangioma
» Lymphangioma
» GlomusTumour
–Intermediate or inderminate variant :
» Haemangioendothelioma
» Hemangiopericytoma
–Malignant :
» Angiosarcoma
» Malignant haemangiopericytoma
6. Other Connective tissue tumours
• Benign :
» Benign fibroushistiocytoma
» Lipoma
• Intermediate :
» Desmoplasticfibroma
• Malignant :
» Fibrosarcoma
» Liposarcoma
» Malignantmesenchymoma
» Malignant fibroushistiocytoma
» Leiomyosarcoma
» Undifferentiatedsarcoma
7. Other Tumours
• Chordoma
• Adamantinoma
• Neurilemmoma
• Neurofibroma
8. Unclassified Tumours
9. Tumour Like Lesions
• Solitary Bone cysts
• Aneurysmal bone cyst
• Juxta articular bone cyst (Intra osseous ganglion)
• Metaphilic fibrous defect ( non ossifying fibroma)
• Eosinophilic granuloma
• Fibrous dysplasia
• Myositis ossificans
• Brown Tumour or hyperparathyroidism
• Intraosseous epidermoid cyst
• Giant cell granuloma
Classification based on origin of tumours
1. Primary Bone tumours : Derived from bone
2. Metastatic bone Tumours : Due to Mets from :
–Breast
-Kidney
–Prostate
- Adrenal
–Thyroid
- Intenstine
- Lung, Liver
Lytic + Blastic lesions
Lytic
Blastic
Lytic
Lytic
Lytic
Lytic
- Urinary Bladder, Uterine Cervix Lytic lesions
3. Tumour Like Lesions : Non neoplastic Conditions that resemble
tumours.
Eg : Solitary Bone cyst, Aneurysmal Bone cyst, Fibrous Dysplasia, Brown`s
tumour.
Classification based on site of Origin
1. Epiphyseal
Osteoclastoma, Chondroblastoma
2. Metaphyseal
Osteioid osteoma, Osteochondroma,
Osteoblastoma, Bone cysts, Osteogenic Sarcoma
3. Diaphyseal
Ewing`s sarcoma, Multiple myeloma
General Concepts in Tumour Terminology
• True Capsule :
• Surrounds a benign lesion and is composed of
compressed normal cells and mature fibrous tissue.
• Pseudocapsule :
• Compressed tumour cells.
• Fibrovascular zone of reactive tissue with an
inflamamtory component that interdigitates with normal
tissue and contains satellite lesions.
• Compartment :
It refers to bone or muscle of origin;
– For Muscle, compartment is that within its Fascia.
– For Bone :
• Intracompartmental implies Bone tumour within the
cortex
• Extracompartmental implies a bone tumour that
destroys the cortex and spreads in to the surrouding
tissue.
• Skip Metastasis :
• A skip metastasis, is defined as a tumor nodule that is
located within the same bone as the main tumor or on
the opposing side of joint but not in continuity with it.
• High grade sarcomas have the ability to break through
the pseudo capsule and metastasize within the same
compartment.
– MRI Scan better identifies them.
Intra Osseous Skip Mets :
Embolization of tumour
cells within the marrow
sinusoids.
Transarticular Skip Mets :
Occur via periarticular
venous anastamosis – Very
poor prognosis
Satellite lesion
Tumour nodule
within reactive zone.
GRADING and STAGING of TUMOURS
• To determine prognosis and choice of treatment.
GRADING :
• It is defined as macroscopic and microscopic degree of
differentiation of tumour
• BORDER`s GRADING :
– GRADE I :
– GRADE II :
– GRADE III :
– GRADE IV:
Well differentiated; <25% Anaplastic cells
Moderately Differentiated; 25-50% Anaplasticcells
Moderately differentiated; 50-75% Anaplastic cells
Poorly differentiated; >75% Anaplasticcells
Enneking`s Grading of Tumours
• G0 Histologically benign
(well differentiated and low cell to matrix ratio)
• G1 Low grade malignant
– (few mitoses, moderate differentiation and local spread
only); Have low risk of metastases
• G2 High grade malignancy
– (frequent mitoses, poorly differentiated); High risk of
metastases
STAGING OF TUMOURS
• STAGING is defined as extent of spread of tumour.
– It is determined by clinical examination, Investigations and
pathological studies.
– Common staging systems are
1. ENNEKING `S STAGING SYSTEM
2. AJCC SYSTEM
3. TNM STAGING ( Union International Cancer centre
Geneva Staging System )
ENNEKING`s STAGING OF BENIGN
TUMOURS
1. Latent—low biological activity; well marginated;
remains static or heals spontaneously;
often incidental findings (i.e., nonossifying
fibroma)
2. Active—symptomatic; limited bone destruction;
progressive growth but limited by natural barriers;
may present with pathological fracture (i.e., aneurysmal
bone cyst)
3. Aggressive—aggressive; bone destruction/soft tissue
extension; do not respect natural barriers (i.e., giant cell
tumor)
• GTM classification described by Enneking is adopted
by Musculoskeletal Tumour society and is based on
surgical grading (G), location (T), lymphnode
involvement & metastasis (M)
Enneking`s staging of malignant tumours
STAGE GRADE SITE
I A Low – G1 Intracompartmental T1
I B Low – G1 Extracompartmental T2
II A High G2 Intracompartmental T1
II B High G2 Extracompartmental T2
III A Any grade with regional or distal
Metastases
Intracompartmental T1
III B Any grade with regional or distal
Metastases
Extracompartmental T2
American joint committee on cancer system bone
sarcoma classification (AJCC Classification)
The AJCC system for bone sarcomas is based on tumor grade, size, and presence and
location of metastases.
TNM STAGING (Union International Cancer
centre Geneva Staging System)
• T – Primary Tumour – T0 to T4
– In Situ lesion T0 to largest and most extensive T4 primary
tumour
• N – Nodal involvement – N0 to N3
– No lymph nodes involvement N0 to wide spread nodal
involvement N3
• M – Metastasis – M0 toM2
– No Metastasis M0 to distant metastasis M2
CLINICAL PRESENTATION
• Pain :
– Initially may be activity related, but in case of malignancy
there could be progressive pain at rest and at night.
– In benign tumours, pain may be activity related when it is
large enough to compress surrounding soft tissue or when
it weakens bone.
– A benign Osteioid osteoma may cause night pain
initially that classically gets relieved with Aspirin.
• In case of soft tissue sarcomas patients may come with
mass rather than pain but in some exceptions like nerve
sheath tumours, they have pain and neurological
conditions.
• Age : It is the most important denominator because
most musculoskeletal tumours occur within specific age
ranges.
• Sex : Very few tumours show sex prediliction.
• Eg GCT is commoner in females.
• Family History : may be present in tumours like
exostosis/ von recklenghausen`s disease.
INVESTIGATIONS
Serological investigations :
• 1. Complete Blood Picture :
– Haemoglobin : to rule out anaemia that may be due
to replacement of bone marrow by neoplastic
process.
– ESR : raised in mets, Ewing`s sarcoma, lymphoma,
leukemias
• 2. Increased Prostate Specific Antigens (PSA)
with Prostatic Acid Phosphatase levels in a case
of blastic lesions of x ray is the diganostic of Mets
secondary to Prostate Carcinoma .
 3. Serum alkaline phosphatase (ALP)
 Biological marker of tumour activity.
 Increases significantly when tumour and metastasis are
highly osteogenic.
 ALP levels decline after Surgical removal of primary
tumour and elevates if metastasis aggravates.
 Good prognostic tool.
• Increased in following conditions: -
– Osteoblastic bone tumors (metastatic or osteogenic
sarcoma)
– 5-Nucleotidase and GGT ( Gamma glutamyl Trasferase ) are
elevated in liver pathology along with Alkaline
phosphatase, where as in bone pathologies only ALP is
increased.
 4. Antisarcoma Antibodies :
 Monoclonal antibodies can be detected by
immunohistochemical assays.
 Antibodies binding to sarcoma cell surface antigens
have specificity.
 5. Osteocalcin – A : Helpful in diagnosing heavily bone
producing types of tumours.
 6. Serum Calcium : Hypercalcemia is often due to Mets,
Myeloma, Hyperparathyroidism.
 7. Abnormal Serum protein electrophoresis along with
bence jones proteins in urine is classical of Multiple
myeloma
Flow Cytometry
• A sample of cells are treated with special antibodies that stick
to the cells only if certain substances are present on their
surfaces.
• The cells are then passed in front of a laser beam. If the cells
now have antibodies attached to them, the laser will cause
them to give off light, which can be measured and analyzed by
a computer.
• Flow cytometry can help determine type of those abnormal
cells and help in diagnosing a tumour early.
INVESTIGATIONS: RADIOGRAPHS
• Phemister's Law = the most common site of
infection & tumours is the fastest growing site
of the long bone
• Tosee a lucent lesion in bone, an estimated 30
to 50 % of the bone must first be lost
[Harris & Heaney, N Engl J Med 1969]
Plain X rays (SEVEN)
1. Where is the lesion? (What bone & what part
of the bone?)
2. Age & size of the lesion?
3. What is the lesion doing to bone?
4. What is the bone doing in response?
5. Is the lesion making matrix?
6. Is the cortex eroded?
7. Is a soft tissue mass evident?
A. Anatomic Sites – X ray
• Anatomic site Specific anatomic sites of the
bone give rise to specific groups of lesions.
Characteristic Locations
• Simple bone cyst
Proximal humerus
• Chondroblastoma
Epiphyses
• Giant Cell tumor
Epiphyses
• Adamantinoma
Tibia
• Chordoma
Sacrum, clivus
• Osteoblastoma
Spine, posterior
Characteristic Locations
• Simple bone cyst
Proximal humerus
• Chondroblastoma
Epiphyses
• Giant Cell tumor
Epiphyses
• Adamantinoma
Tibia
• Chordoma
Sacrum, clivus
• Osteoblastoma
Spine, posterior
• Simple bone cyst
Proximal humerus
• Chondroblastoma
Epiphyses
• Giant Cell tumor
Epiphyses
• Adamantinoma
Tibia
• Chordoma
Sacrum, clivus
• Osteoblastoma
Spine, posterior
Characteristic Locations
• Simple bone cyst
Proximal humerus
• Chondroblastoma
Epiphyses
• Giant Cell tumor
Epiphyses
• Adamantinoma
Tibia
• Chordoma
Sacrum, clivus
• Osteoblastoma
Spine, posterior
Characteristic Locations
• Simple bone cyst
Proximal humerus
• Chondroblastoma
Epiphyses
• Giant Cell tumor
Epiphyses
• Adamantinoma
Tibia
• Chordoma
Sacrum, Pelvis
• Osteoblastoma
Spine, posterior
Characteristic Locations
• Simple bone cyst
Proximal humerus
• Chondroblastoma
Epiphyses
• Giant Cell tumor
Epiphyses
• Adamantinoma
Tibia
• Chordoma
Sacrum, clivus
• Osteoblastoma
Spine - posterior
elements
Characteristic Locations
B. Borders
• The border reflects the growth rate and the response of the
adjacent normal bone to the tumor.
• Most tumors have a characteristic border
• Benign lesions (e.g., nonossifying fibromas and unicameral
bone cysts) have well-defined borders and a narrow
transition area that is often associated with a reactive
sclerosis.
• Aggressive or benign tumors (e.g., chondroblastoma and
GCTs) tend to have faint borders and wide zones of transition
with very little sclerosis, reflecting a faster-growing lesion.
• Poorly delineated or absent margins indicate an aggressive or
malignant lesion
ZONE OFTRANSITION
• To classify well-defined or ill-defined, look zone of
transition (between the lesion and the adjacent normal
bone)
• It is the most reliable indicator in determining benign or
malignant.
• It only applies in osteolytic lesions
Small zone of transition
•Sharp, well-defined
border (sign of slow
growth)
• Sclerotic border
(indicates poor biological
activity)
Wide zone of transition
• Feature of malignant bone tumors
• Ill-defined border with a broad zone of transition (sign of
aggressive growth)
• Two tumor like lesions(Infections & Eosinophilicgranuloma) may
mimic malignancy d/t their aggressive biologic behavior
LOCATION (Transverse Plane)
1. Centric
2. Eccentric
3. Cortical
4. Juxta-cortical
Centric
• SBC,eosinophilicgranuloma,fibrousdysplasia, ABC and
enchondroma
Eccentric
• Osteosarcoma, NOF, chondroblastoma, chondromyxoid fibroma,
GCT and osteoblastoma
Cortical
• Osteoid osteoma
Juxta-cortical
• Osteochondroma. The cortex must extend into the stalk of the
lesion. Parosteal osteosarcoma arises from the periosteum.
1. SBC: central diaphyseal
2. NOF: eccentric metaphyseal
3. SBC: central diaphyseal
4. Osteoid osteoma: cortical
5. Degenerative subchondral
cyst: epiphyseal
6. ABC: centric diaphyseal
AGE ATPRESENTATION
• Age is the most important clinical clue in differentiating
possible bone tumors
• Primary malignant bone tumours are rare before 5 years of
age
• 1st decade: Commonly disseminated bone lesions of
leukaemia and neuroblastoma
• 2nd decade: Commonly an osteosarcoma or Ewing’s sarcoma
• > 45 years of age: Metastases are the commonest
Specific tumors by age
Malignant bone tumors(red) and benign
tumors(blue)
C. Bone destruction
• Bone destruction is the hallmark of a bone
tumor.
• Three patterns of bone destruction are
described
1. Geographic,
2. Moth-eaten,
3. Permeative.
Patterns of Bone Destruction
(Lodwick Classification)
• For describing the margins of a lucent/lytic bone lesion
• Used in the description for aggressive and possibly malignant
Classification
• Type 1: Geographic
• Type 2: Moth-eaten
• Type 3: Permeative
A: Pattern of medullary destruction
B: Margination of lesions
Geographic Bone Destruction
Complete destruction of
bone from boundary to
normal bone
• Non-ossifying fibroma
• Chondromyxoid fibroma
• Eosinophilic granuloma
Non-ossifying fibroma
Moth-eaten Bone Destruction
• Areas of destruction with
ragged borders
• Implies more rapid growth
• Probably a malignancy
Examples:
• Myeloma
• Metastases
• Lymphoma
• Ewing’s sarcoma
Multiple Myeloma
Permeative Bone Destruction
• Ill-defined lesion with multiple
“worm-holes”
• Spreads through marrow space
• Wide transition zone
• Implies an aggressive
malignancy
• Round-cell lesions
Examples:
• Lymphoma, leukemia
• Ewing’s Sarcoma
• Myeloma
• Osteomyelitis
• Neuroblastoma Leukemia
Patterns of Bone Destruction
Geographic Moth-eaten Permeative
Less malignant More malignant
D. Matrix formation
• Calcification of the matrix, or new bone formation,
may produce an area of increased density within the
lesion.
• Calcification typically appears as flocculent or
stippled rings or clusters.
• The appearance of the new bone varies from dense
sclerosis that obliterates all evidence of normal
trabeculae to small, irregular, circumscribed masses
described as "wool" or "clouds."
• Calcification and ossification may appear in the same
lesion.
• Neither type of matrix formation is diagnostic of
malignancy.
• Calcifications or mineralization within a bone lesion
may be an important clue in the differential
diagnosis
• Two kinds of mineralization:
- Chondroid matrix in cartilaginous tumors
- Osteoid matrix in osseous tumors
Tumor bone formation, matrix calcification
Osseous mineralization
• Cloud-like and poorly defined, diffuse matrix
mineralization
• Trabecular ossification pattern in benign bone
forming lesions
• Cloud-like or ill-defined amorphous pattern in
osteosarcomas
• Sclerosis can also be reactive, e.g. in Ewing’s
sarcoma or lymphoma.
Cloud-like bone formation in osteosarcoma.
Notice the aggressive, interrupted periosteal
reaction (arrows
Trabecular ossification pattern in osteoid
osteoma.
Notice osteolytic nidus (arrow).
Chondroid mineralization
• Linear, curvilinear, ring-like, punctuate or nodular
• Often central
• Peripheral in benign lesions such
as a bone infarct or myositis
ossificans
• Calcifications in chondroid
tumors have many descriptions:
rings-and-arcs, popcorn, focal
stippled or flocculent.
Diffuse matrix
• Shows ground-glass density
• Punctate calcification may also be present
• Seen in benign fibrous tumours
Expansile Lesions of Bone
Multiple myeloma
Mets
Brown tumor
Enchondroma
Aneurysmal bone cyst
Fibrous dysplasia
Expansile Lesions of Bone
Multiple myeloma
Mets
Brown tumor
Enchondroma
Aneurysmal bone cyst
Fibrous dysplasia
Renal cell carcinoma
Multiple myeloma
Mets
Brown tumor
Enchondroma
Aneurysmal bone cyst
Fibrous dysplasia
Expansile Lesions of Bone
Multiple myeloma
Mets
Brown tumor
Enchondroma
Aneurysmal bone cyst
Fibrous dysplasia
Expansile Lesions of Bone
E. Periosteal reaction
• Periosteal reaction is indicative of malignancy
but not pathognomonic of a particular tumor.
• Any widening or irregularity of bone contour
may be regarded as periosteal activity.
Solid Periosteal Reactions
Chronic osteomyelitis
• Single solid layer or multiple
closely apposed and fused
layers of new bone attached
to the outer surface of
cortex resulting in cortical
thickening.
• It is uniterrupted or
continous.
Types of Solid Periosteal Reaction
• 1. SOLID BUTTRESS
• Seen in Aneurysmal bone cyst, chondromyxoid fibroma
• 2. Solid Smooth or Elleptical layer
• Seen in Osteoid osteoma and osteoblastoma
• 3. Undulating type :
• Seen in long standing varicosities, periosteitis, chronic
lymphaoedema.
• 4. Single Lamellar reaction :
• Seen in Osteomyelitis, Stress Fractures, Langerhans cell
histiocytosis.
Interupted Periosteal Reactions
• Commonly seen in
Aggressive/malignant
tumours.
– Onion-Peel ( lamellated)
– Ewings sarcoma
– Gouchers disease
– Sunburst
– Codman’s triangle
Ewing sarcoma
Sunburst type of periosteal reaction
Fine lines of increased
density representing newly
formed specules of bone
radiate laterally from and at
right angles to the surface of
the shaft giving a typical
sun ray appearance.
Osteo-sarcoma
Spiculated, vertical or ‘hair-on-
end’ periostealreaction
• Seen in the most aggressive
tumours
• E.g. Osteosarcoma or Ewing’s
sarcoma
• Most rapidly growing lesions may
not be associated with any
radiographically visible periosteal
reaction (as minerali- zation of
periosteum can take weeks)
A ‘hair-on-end’type vertical periosteal reaction
associated with Ewing’s sarcoma.
Note also the Codman’s triangle (arrow).
Codman’s triangle
– When the tumour breaks
through the cortex and
destroys the newly formed
lamellated bone, the remnants
of the latter on both ends of
the break through area may
remain as a triangular structure
known as codman triangle.
Also seen in Osteosarcoma,
Ewings sarcoma,
Chronic Osteomyelitis
Osteo-sarcoma
Ewing sarcoma of femur (AP & Lateral)
Mottled, osteolytic lesion (blue circle) with poorly marginated edges in the
diaphysis of the bone
Sunburst periosteal reaction (red circle) and
Lamellated periosteal reaction (whitearrows)
Periosteal Reactions
Solid onion-peelSunburst Codman’s
triangle
Less malignant More malignant
Wide zone of transition indicates:
Malignancy or Infection or Eosinophilic granuloma
CORTICAL DESTRUCTION
• Common finding
• Destruction of cortex by a lytic or sclerotic process
• Not very useful in distinguishing between benign and malignant
• Complete destruction seen in high-
grade malignant lesions and in locally
aggressive benign lesions like EG and osteomyelitis.
• Uniform cortical bone destruction in benign and low-grade
malignant lesions
Patterns of cortical disturbance
• It delineates intra and extra
osseous extent of tumour.
• It can reliably distinguish
between infection and tumor.
• CT scan identifies accurately area of cortical break through, soft
tissue extension, medullary spread and proximity of the tumour
to neurovascular bundle and evaluating integrity of cortex
• Todifferentiate solid and cystic lesions.
• Most sensitive investigation to detect Pulmonary mets.
CT scan:-
• Best imaging
– to localise the nidus of an osteiod osteoma,
– to detect a thin rim of reactive bone around an aneurysmal bone cyst,
– to evluate calcification in a suspected cartilagenous lesion and
– to evaluate endosteal cortical erosion in a suspected chodrosarcoma.
• Todifferentiate between the neoplastic mass and
inflammatory condition : Neoplastic masses displace soft tissue
fat planes where as they are obliterated in inflammatory
conditions.
• It cannot differentiate benign from malignant tumours
accurately.
• Except in detecting pulmonary mets, Contrast CT is better than
plain CT.
• It has better contrast
discrimination than any other
modality.
• Helps in detecting skip lesions
• Assesses the tumor relationship
with adjacent soft tissue, joints
and blood vessels.
• It can visualize bone marrow
content and demonstrate
intramedullary extension of
neoplasm.
MRI:-
• It is the investigation of choice in local staging of
musculoskeletal tumours.
• On MRI, it is not possible to accurately
differentiate benign from malignant tumours. But
if the following criteria are present, lesion can be
considered as a malignant one :
– 1. Mass with irregular Border
– 2. Non homogenous signal intensity with extra
compartmental extension
– 3. Peri tumoral edematous reaction.
– 4. Soft tissue mass situated deep to fascia and
measuring more than 5 cm in greatest diameter is likely
to be a sarcoma.
• It uses radioactive glucose to locate cancer by observing
high glycolysis rates in a malignant tissue metabolism.
• It has low specificity as the FDG ( Fluoro labelled deoxy
glucose) can also accumulate in benign aggressive and
inflammatory lesions.
• Also helpful in evaluating the tumour after chemotherapy.
• Micromets are better visulaised.
PET- Positron Emission
Tomography
Most reliable means of determining vascular anatomy.
• Reactive zone is best seen on early arterial phase,
while the intrinsic vascularity is best seen on late
venous phase as a tumour blush.
• Transcatheter embolisation is done as a definitive
treatment in some benign vascular tumours.
Angiography
Angiography demonstrating
vascularity of a tumour
Embolization of a vascular
lesion, performed at least 6
hours prior to surgery, is
expected to significantly
reduce intraoperative blood
loss.
• Technetium (99mTC) bone scans are used.
• It is an indicator for mineral turnover.
• Whenever there is altered local metabolism in remodeling
bone, increased vascularity or mineralization , the isotope
uptake is enhanced mainly in reactive zone surrounding the
lesions.
• Confirms epiphyseal spread of tumour.
• Helps in detecting multiple lesions like multiple
osteochondroma, enchondroma.
• Where as a MRI helps in detecting skip lesions
Nuclear Imaging -Bone scan Scinitigraphy
Bone scan showing HOT
SPOTS over proximal
humerus and ribs
It detects the presence
of skeletal metastasis
and delineates it from
primary else where in
the body.
• Bone scinitigraphy tends to show larger area of
extension of medullary involvement of tumour
as the radio active agent also localises the area
of hyperemia and edema adjacent to tumour.
• Nuclear imaging is advantageous only to
identifying whether skeletal involvement is
solitary or multiple.
• Not routinely used in diagnosis of sarcoma; as it
better differentiates only bony cystic lesions.
• However Ultrasound is used in guided
percutaneous biopsy.
• In patients treated with prosthetic implants, USG
is the modality that depicts early recurrence as
MRI produces blurred and artifact images due to
metallic implants.
Ultrasound
 Used for definitive diagnosis.
• Principles of biopsy:
 Opted only after all other investigations are performed.
 A biopsy can be done by FNAC, core needle biopsy, or an
open incisional procedure.
 FNAC may be 90% accurate at determining malignancy;
however, its accuracy at determining specific tumor type is
much lower.
– Trephine or core biopsy is recommended and often yields
an adequate sample for diagnosis.
– Complications are greater with incisional biopsy; but least
likely to be associated with a sampling error, and provides
the sample for additional diagnostic studies, such as
cytogenetics and flow cytometry.
Biopsy
– Core biopsy is preferred if limb spraying is an option as it entails
less contamination than open biopsy.
– A small incisional biopsy can be performed if core biopsy
specimen is inadequate.
– Performed under torniquet (possibly) - the limb may be elevated
before inflation but should not be exsanguinated by
compression bandage.
 Longitudinal incisions preferred as transverse excision are
extremely difficult or impossible to excise with the specimen.
 NV bundle not exposed.
 Dissection through muscle (not between) to prevent
contamination of tumour.
 Approach for open biopsy is made through region of definitive
surgical excision.
 If a drain is used, it should exit in line with the incision so that
the drain track also can be easily excised en bloc with the
tumor.
 Wound is closed tightly in layers.
 Meticulous haemostasis is arrested by use of bone wax/
Poly Methyl Metha Acrylate(PMMA) to plug the cortical
window.
 Always sample the tissues from periphery of lesion which
contains most viable tissue.
 Never biopsy a periosteal reaction / codmans traingle as it
contains a new reactive bone and could be false negative.
•If hole must be made in bone during biopsy, defect should be
round or oval to minimize stress concentration, which otherwise
could lead to pathological fracture.
•Torsional strength is not affected by length of defect. Always
attempt to keep defects less than 10% of bone diameter.
•When biopsy size is greater than 20% of bone diameter, torsional
strength decreases to 50%.
Examples of poorly performed biopsies
Biopsy resulted in irregular defect in bone, which led to pathological
fracture
Examples of poorly performed
biopsies
Transverseincisions
should not be used
Needle biopsy track
contaminated patellar
tendon
Multiple needle
tracks contaminate
quadriceps tendon
Drain site was not placed in line with
incision
Needle track placed posteriorly,
location that would be extremely
difficult to resect en bloc with
tumor if it had proved to be
sarcoma
• Biopsy should be done only after clinical, laboratory,
and radiographic examinations are complete.
• Completion of the evaluation before biopsy aids in
planning the placement of the biopsy incision, helps
provide more information leading to a more accurate
pathological diagnosis, and avoids artifacts on
imaging studies.
• If the results of the evaluation suggest that a primary
malignancy is in the differential diagnosis, Biopsy is
not done unless it is possible to operate the case in
the centre.
• Curettage resection and restoration of function by
limb salvage procedures or amputation is primary
form of surgical correction.
• Based on surgical plane of dissection in relation to
tumour, Enneking formulated following types of
resection.
1) Intralesional Resection
2) Marginal resection
3) Wide (Intracompartmental) resection
4) Radical (Extracompartmental) resection
SURGICAL OPTIONS
• An intralesional margin is one in which the plane of surgical
dissection is within the tumor.
• This type of procedure is often described as “debulking”
because it leaves behind gross residual tumor.
• This procedure may be appropriate for symptomatic benign
lesions when the only surgical alternative would be to
sacrifice important anatomical structures.
• This also may be appropriate as a palliative procedure in the
setting of metastatic disease.
• Intralesional resection is through the psuedocapsule of the
tumor directly in to the lesion. Macroscopic tumour is left
behind. Curettage is intralesional proceedure.
1) Intralesional Resection :
CURETTAGE
• Cortical window with rounded margins is made
• When possible, the window is sized larger than the tumour
so that the entire tumour is readily seen.
• The rounded margins reduce the risk of subsequent
fracture.
• Large curetts should be used to remove the lesional tissue.
• Tumour margin should be treated with cryotherapy, PMMA
cementage or phenol – alcohol cauterisation, argon beam
coagulation in case of aggressive tumours.
• If curettage weakens the bone, graft using allograft or
autograft with or with out internal fixation is indicated.
• A marginal margin is achieved when the closest plane of
dissection passes through the pseudocapsule.
• This type of margin usually is adequate to treat most benign
lesions and some low-grade malignancies.
• In high-grade malignancy, however, the pseudocapsule often
contains microscopic foci of disease, or “satellite” lesions.
• A marginal resection often leaves behind microscopic disease
that may lead to local recurrence if the remaining tumor cells
do not respond to adjuvant chemotherapy or radiation
therapy.
2) Marginal Resection
• Intracompartmental
• Wide margins are achieved when the plane of dissection is in
normal tissue.
• Although no specific distance is defined, the Resection
includes removal of entire tumour, + Reactive zone & cuff of
normal tissue.
• If the plane of dissection touches the pseudocapsule at any
point, the margin should be defined as being marginal and not
wide.
• Although sometimes impossible to achieve, wide margins are
the goal of most procedures for high-grade malignancies.
3) Wide Resection
4] Radical
Radical margins are achieved when all the compartments that
contain entire tumor and structure or origin of lesion are
removed en bloc.
The plane of dissection is beyond the limiting fascial & bone
borders.
For deep soft-tissue tumors, this involves removing the entire
compartment (or multiple compartments) of any involved
muscles.
However, with improvements in imaging studies, radical
procedures now are rarely performed because equivalent
oncological results usually can be obtained with wide margins
.
ENNEKING`s Classification of Surgical Procedures for Bone
Tumors
Margin Local (Mode of resection)
LIMB SALVAGE OPTIONS
Amputation(Mode of amputation)
Intralesional Curettage or debulking Debulking amputation
Marginal Marginal excision Marginal amputation
Wide Wide local excision Wide through bone, amputation
Radical Radical local resection Radical disarticulation
Enneking`s
Classification of
Resection of
tumours.
Ennekings Classification of
Amputations
1] Limb Salvage Proceedures
2] Amputations
SURGICAL OPTIONS
• It is designed to accomplish removal of a malignant tumour &
reconstruction of the limb with an acceptable oncologic,
functional & cosmetic result.
• It is sub amputative wide resection with preservation of the
limb & its function.
• Indications :
• Stage IA Stage IIA & Stage IIIA (All intracompartmental
tumours)with good response to pre-operative chemotherapy
• Skin should be uninvolved and free
• There should be feasibility of keeping a cuff of normal tissue
surrounding the tumour
• Upper extremity lesions are more suitable for limb sparing
surgery
 Tumours with good pre-operative chemotherapy response
LIMB SALVAGE PROCEDURES
Exclusion Criteria
Patients with bone tumors in whom limb salvage was not possible. The
criteria used to determine if the limb was worthy of salvage were as
follows:
1. The major neurovascular bundle is free of tumor.
2. Wide resection of the affected bone with a normal muscle cuff in all
directions is possible.
3. All previous biopsy sites and all potentially contaminated tissues can be
removed en bloc.
4. Bone can be resected 3 to 4 cm beyond abnormal uptake as determined
by bone scan
5. The adjacent joint and joint capsule can be resected.
6. Adequate motor reconstruction can be accomplished by
regional muscle transfers.
7. Adequate soft tissue coverage is possible to decrease the risk
of skin flap necrosis and secondary infection.
• Reconstruction of bone defect may be done by
1 Osteoarticular allograft reconstruction
2Allograft-prosthesis composite reconstruction
3] Endoprosthetic reconstruction.
4] Allograft arthrodesis
5} Rotationplasty
6} Turnoplasty
Surgical reconstructive options :
• Amputation provides definitive surgical treatment when limb sparing
is not a prudent one.
• Common amputations in malignant tumours:
– Proximal humerus : fore quarter amputation
– Distal femur : hip disarticulation
– Proximal tibia : mid thigh amputation
AMPUTATIONS
• Adjuvant chemotherapy:
– Totreat presumed micrometastasis
• Neo adjuvant[induction]
– Before surgical resection of the primary tumour
Advantages:
– It controls micro metastasis and improves survival rate.
– Chemotherapy makes limb salvage surgery easier.
– Decreases tumor size and vascularity.
– The response to Chemotherapy can be evaluated after
surgery.
Chemotherapy
• With Megavoltage radiotherapy tumor cell can be destroyed.
• High voltages are administered in short sessions.
• Radiation therapy should be started immediately after
diagnosis before surgery to prevent metastasis .
• Chemotherapy increases the susceptibility of tissues to
irradaition.
• Protect all normal tissue biopsy scars to prevent radiation
necrosis.
• Distribute the dose in accordance with distribution of tumor.
RADIOTHERAPY
Diagnosis
Neoadjuvant chemotherapy + Radiation
Resection/ surgery Repeat Chemo + Radiation
Adjuvant + irradiation +chemo Histological Grading
SEQUENCE OF TREATMENT
• Gene therapy in sarcomas :
• Targeting Osteocalcin promoter.
• Osteocalcin is produced both in Osteoblastic[100%]and fibroblastic[70%]
Osteosarcoma.
• LIQUID BRACHYTHERAPY :
– Injecting Intra arterial infusion of chemotherapeutic drugs with
brachytherapy
– It is in Phase 2 trials currently.
• Cryotherapy is used in curettage after resection of primary
tumour to prevent chances of recurrence.
• PMMA, Phenol, liquid nitorgen commonly used
cryoprecipitates.
RECENT ADVANCES
Bone tumours

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Bone tumours

  • 1. BONE TUMORS &ITS GENERAL PRINCIPLES OF MANAGEMENTDR.PRASHANTH KUMAR SEC.DNB RESIDENT HINDU RAO HOSPITAL DELHI
  • 2. Introduction : • Neoplasia : It is defined as a mass of tissue formed as result of abnormal, excessive, uncoordinated, autonomous and purposeless proliferation of cells. • Term Neoplasia includes both Benign and Malignant.
  • 3. FEATURES BENIGN (DIFFERENTIATED) MALIGNANT (UNDIFFERENTIATED) MACROSCOPIC FEATURES Boundaries Encapsulated or well circumscribed Poorly circumscribed and irregular Surrounding tissue Often compressed Usually invaded Secondary Changes Occur less often Occur more often MICROSCOPIC FEATURES Pattern Usually resemble tissue of origin No resemblance Nucleo – Cytoplasmic Ratio Normal Increased Pleomorphism Absent usually Often present Anisonucleosis Absent Generally present Hyperchromatism Absent Often present Growth Rate Usually Slow Rapid Metastasis Absent Frequently Present Contrasting Differences between Benign and MalignantTumours
  • 4. Routes of Metastasis : • Haematogenous Spread : Most common spread in musculoskeletal tumours • Lymphatic Spread : rare in musculoskeletal tumours. • Seen in Rhabdomyosarcoma, Synovial sarcoma, Malignant fibrous histiocytoma . • Direct Implantation : – Through surgeons scalpel, needles, sutures, FNAC, diagnostic or excision biopsy • Spread through CSF : – malignant tumours of ependyma and leptomeninges rarely spread through CSF to vertebrae.
  • 5. Classification of Tumours 1. WHO Classification : widely accepted and is based on histogenesis and histological criteria. 2. Classification based on origin of tumours 3. Classification based on site of lesions.
  • 6. Modified WHO Classification of BONE TUMOURS • 1. Bone forming tumours : Benign : Osteoma Osteoid Osteoma Osteoblastoma Intermediate Aggressive Osteoblastoma Malignant Osteosarcoma Conventional osteosarcoma Telangiectatic osteosarcoma Juxta cortical or Parosteal Osteosarcoma Periosteal Osteosarcoma
  • 7. 2. Cartilage forming Tumours • BENIGN : • Chondroma • Enchondroma • Osteochondroma • Chondroblastoma • Chondromyxoid fibroma • MALIGNANT : • Chondrosarcoma • Juxtacortical chondrosarcoma • Mesenchymal chondrosarcoma • Clear cell chondrosarcoma • De differentiated chondrosarcoma • Malignant chondroblastoma
  • 8. 3. Giant Cell Tumours : – Osteoclastoma – Malignant giant cell tumour – Giant cell tumour in Pagets disease – GCT occurring in non epithelial region – Giant cell variants : tumors which histologically show the osteoclastic giant cells 1 - aneurysmal bone cyst 2 osteoclastoma 3 chondroblastoma 4 unicameral bone cyst 5 chondromyxoid fibroma 6non osteogenic fibroma 7- fibrous dysplasia 8- brown tumor of hyperparathyroidism .
  • 9. 4. Marrow tumours •Ewing sarcoma •Reticulosarcoma •Lymphosarcoma •Myeloma
  • 10. 5. Vascular Tumours – Benign: » Haemangioma » Lymphangioma » GlomusTumour –Intermediate or inderminate variant : » Haemangioendothelioma » Hemangiopericytoma –Malignant : » Angiosarcoma » Malignant haemangiopericytoma
  • 11. 6. Other Connective tissue tumours • Benign : » Benign fibroushistiocytoma » Lipoma • Intermediate : » Desmoplasticfibroma • Malignant : » Fibrosarcoma » Liposarcoma » Malignantmesenchymoma » Malignant fibroushistiocytoma » Leiomyosarcoma » Undifferentiatedsarcoma
  • 12. 7. Other Tumours • Chordoma • Adamantinoma • Neurilemmoma • Neurofibroma 8. Unclassified Tumours
  • 13. 9. Tumour Like Lesions • Solitary Bone cysts • Aneurysmal bone cyst • Juxta articular bone cyst (Intra osseous ganglion) • Metaphilic fibrous defect ( non ossifying fibroma) • Eosinophilic granuloma • Fibrous dysplasia • Myositis ossificans • Brown Tumour or hyperparathyroidism • Intraosseous epidermoid cyst • Giant cell granuloma
  • 14. Classification based on origin of tumours 1. Primary Bone tumours : Derived from bone 2. Metastatic bone Tumours : Due to Mets from : –Breast -Kidney –Prostate - Adrenal –Thyroid - Intenstine - Lung, Liver Lytic + Blastic lesions Lytic Blastic Lytic Lytic Lytic Lytic - Urinary Bladder, Uterine Cervix Lytic lesions 3. Tumour Like Lesions : Non neoplastic Conditions that resemble tumours. Eg : Solitary Bone cyst, Aneurysmal Bone cyst, Fibrous Dysplasia, Brown`s tumour.
  • 15. Classification based on site of Origin 1. Epiphyseal Osteoclastoma, Chondroblastoma 2. Metaphyseal Osteioid osteoma, Osteochondroma, Osteoblastoma, Bone cysts, Osteogenic Sarcoma 3. Diaphyseal Ewing`s sarcoma, Multiple myeloma
  • 16.
  • 17. General Concepts in Tumour Terminology • True Capsule : • Surrounds a benign lesion and is composed of compressed normal cells and mature fibrous tissue. • Pseudocapsule : • Compressed tumour cells. • Fibrovascular zone of reactive tissue with an inflamamtory component that interdigitates with normal tissue and contains satellite lesions.
  • 18. • Compartment : It refers to bone or muscle of origin; – For Muscle, compartment is that within its Fascia. – For Bone : • Intracompartmental implies Bone tumour within the cortex • Extracompartmental implies a bone tumour that destroys the cortex and spreads in to the surrouding tissue.
  • 19. • Skip Metastasis : • A skip metastasis, is defined as a tumor nodule that is located within the same bone as the main tumor or on the opposing side of joint but not in continuity with it. • High grade sarcomas have the ability to break through the pseudo capsule and metastasize within the same compartment. – MRI Scan better identifies them.
  • 20. Intra Osseous Skip Mets : Embolization of tumour cells within the marrow sinusoids. Transarticular Skip Mets : Occur via periarticular venous anastamosis – Very poor prognosis Satellite lesion Tumour nodule within reactive zone.
  • 21. GRADING and STAGING of TUMOURS • To determine prognosis and choice of treatment. GRADING : • It is defined as macroscopic and microscopic degree of differentiation of tumour • BORDER`s GRADING : – GRADE I : – GRADE II : – GRADE III : – GRADE IV: Well differentiated; <25% Anaplastic cells Moderately Differentiated; 25-50% Anaplasticcells Moderately differentiated; 50-75% Anaplastic cells Poorly differentiated; >75% Anaplasticcells
  • 22. Enneking`s Grading of Tumours • G0 Histologically benign (well differentiated and low cell to matrix ratio) • G1 Low grade malignant – (few mitoses, moderate differentiation and local spread only); Have low risk of metastases • G2 High grade malignancy – (frequent mitoses, poorly differentiated); High risk of metastases
  • 23. STAGING OF TUMOURS • STAGING is defined as extent of spread of tumour. – It is determined by clinical examination, Investigations and pathological studies. – Common staging systems are 1. ENNEKING `S STAGING SYSTEM 2. AJCC SYSTEM 3. TNM STAGING ( Union International Cancer centre Geneva Staging System )
  • 24. ENNEKING`s STAGING OF BENIGN TUMOURS 1. Latent—low biological activity; well marginated; remains static or heals spontaneously; often incidental findings (i.e., nonossifying fibroma) 2. Active—symptomatic; limited bone destruction; progressive growth but limited by natural barriers; may present with pathological fracture (i.e., aneurysmal bone cyst) 3. Aggressive—aggressive; bone destruction/soft tissue extension; do not respect natural barriers (i.e., giant cell tumor)
  • 25. • GTM classification described by Enneking is adopted by Musculoskeletal Tumour society and is based on surgical grading (G), location (T), lymphnode involvement & metastasis (M) Enneking`s staging of malignant tumours STAGE GRADE SITE I A Low – G1 Intracompartmental T1 I B Low – G1 Extracompartmental T2 II A High G2 Intracompartmental T1 II B High G2 Extracompartmental T2 III A Any grade with regional or distal Metastases Intracompartmental T1 III B Any grade with regional or distal Metastases Extracompartmental T2
  • 26. American joint committee on cancer system bone sarcoma classification (AJCC Classification) The AJCC system for bone sarcomas is based on tumor grade, size, and presence and location of metastases.
  • 27.
  • 28. TNM STAGING (Union International Cancer centre Geneva Staging System) • T – Primary Tumour – T0 to T4 – In Situ lesion T0 to largest and most extensive T4 primary tumour • N – Nodal involvement – N0 to N3 – No lymph nodes involvement N0 to wide spread nodal involvement N3 • M – Metastasis – M0 toM2 – No Metastasis M0 to distant metastasis M2
  • 29. CLINICAL PRESENTATION • Pain : – Initially may be activity related, but in case of malignancy there could be progressive pain at rest and at night. – In benign tumours, pain may be activity related when it is large enough to compress surrounding soft tissue or when it weakens bone. – A benign Osteioid osteoma may cause night pain initially that classically gets relieved with Aspirin.
  • 30. • In case of soft tissue sarcomas patients may come with mass rather than pain but in some exceptions like nerve sheath tumours, they have pain and neurological conditions. • Age : It is the most important denominator because most musculoskeletal tumours occur within specific age ranges. • Sex : Very few tumours show sex prediliction. • Eg GCT is commoner in females. • Family History : may be present in tumours like exostosis/ von recklenghausen`s disease.
  • 31. INVESTIGATIONS Serological investigations : • 1. Complete Blood Picture : – Haemoglobin : to rule out anaemia that may be due to replacement of bone marrow by neoplastic process. – ESR : raised in mets, Ewing`s sarcoma, lymphoma, leukemias • 2. Increased Prostate Specific Antigens (PSA) with Prostatic Acid Phosphatase levels in a case of blastic lesions of x ray is the diganostic of Mets secondary to Prostate Carcinoma .
  • 32.  3. Serum alkaline phosphatase (ALP)  Biological marker of tumour activity.  Increases significantly when tumour and metastasis are highly osteogenic.  ALP levels decline after Surgical removal of primary tumour and elevates if metastasis aggravates.  Good prognostic tool. • Increased in following conditions: - – Osteoblastic bone tumors (metastatic or osteogenic sarcoma) – 5-Nucleotidase and GGT ( Gamma glutamyl Trasferase ) are elevated in liver pathology along with Alkaline phosphatase, where as in bone pathologies only ALP is increased.
  • 33.  4. Antisarcoma Antibodies :  Monoclonal antibodies can be detected by immunohistochemical assays.  Antibodies binding to sarcoma cell surface antigens have specificity.  5. Osteocalcin – A : Helpful in diagnosing heavily bone producing types of tumours.  6. Serum Calcium : Hypercalcemia is often due to Mets, Myeloma, Hyperparathyroidism.  7. Abnormal Serum protein electrophoresis along with bence jones proteins in urine is classical of Multiple myeloma
  • 34. Flow Cytometry • A sample of cells are treated with special antibodies that stick to the cells only if certain substances are present on their surfaces. • The cells are then passed in front of a laser beam. If the cells now have antibodies attached to them, the laser will cause them to give off light, which can be measured and analyzed by a computer. • Flow cytometry can help determine type of those abnormal cells and help in diagnosing a tumour early.
  • 35. INVESTIGATIONS: RADIOGRAPHS • Phemister's Law = the most common site of infection & tumours is the fastest growing site of the long bone • Tosee a lucent lesion in bone, an estimated 30 to 50 % of the bone must first be lost [Harris & Heaney, N Engl J Med 1969]
  • 36. Plain X rays (SEVEN) 1. Where is the lesion? (What bone & what part of the bone?) 2. Age & size of the lesion? 3. What is the lesion doing to bone? 4. What is the bone doing in response? 5. Is the lesion making matrix? 6. Is the cortex eroded? 7. Is a soft tissue mass evident?
  • 37. A. Anatomic Sites – X ray • Anatomic site Specific anatomic sites of the bone give rise to specific groups of lesions.
  • 38. Characteristic Locations • Simple bone cyst Proximal humerus • Chondroblastoma Epiphyses • Giant Cell tumor Epiphyses • Adamantinoma Tibia • Chordoma Sacrum, clivus • Osteoblastoma Spine, posterior
  • 39. Characteristic Locations • Simple bone cyst Proximal humerus • Chondroblastoma Epiphyses • Giant Cell tumor Epiphyses • Adamantinoma Tibia • Chordoma Sacrum, clivus • Osteoblastoma Spine, posterior
  • 40. • Simple bone cyst Proximal humerus • Chondroblastoma Epiphyses • Giant Cell tumor Epiphyses • Adamantinoma Tibia • Chordoma Sacrum, clivus • Osteoblastoma Spine, posterior Characteristic Locations
  • 41. • Simple bone cyst Proximal humerus • Chondroblastoma Epiphyses • Giant Cell tumor Epiphyses • Adamantinoma Tibia • Chordoma Sacrum, clivus • Osteoblastoma Spine, posterior Characteristic Locations
  • 42. • Simple bone cyst Proximal humerus • Chondroblastoma Epiphyses • Giant Cell tumor Epiphyses • Adamantinoma Tibia • Chordoma Sacrum, Pelvis • Osteoblastoma Spine, posterior Characteristic Locations
  • 43. • Simple bone cyst Proximal humerus • Chondroblastoma Epiphyses • Giant Cell tumor Epiphyses • Adamantinoma Tibia • Chordoma Sacrum, clivus • Osteoblastoma Spine - posterior elements Characteristic Locations
  • 44.
  • 45.
  • 46. B. Borders • The border reflects the growth rate and the response of the adjacent normal bone to the tumor. • Most tumors have a characteristic border • Benign lesions (e.g., nonossifying fibromas and unicameral bone cysts) have well-defined borders and a narrow transition area that is often associated with a reactive sclerosis. • Aggressive or benign tumors (e.g., chondroblastoma and GCTs) tend to have faint borders and wide zones of transition with very little sclerosis, reflecting a faster-growing lesion. • Poorly delineated or absent margins indicate an aggressive or malignant lesion
  • 47. ZONE OFTRANSITION • To classify well-defined or ill-defined, look zone of transition (between the lesion and the adjacent normal bone) • It is the most reliable indicator in determining benign or malignant. • It only applies in osteolytic lesions
  • 48. Small zone of transition •Sharp, well-defined border (sign of slow growth) • Sclerotic border (indicates poor biological activity)
  • 49. Wide zone of transition • Feature of malignant bone tumors • Ill-defined border with a broad zone of transition (sign of aggressive growth) • Two tumor like lesions(Infections & Eosinophilicgranuloma) may mimic malignancy d/t their aggressive biologic behavior
  • 50. LOCATION (Transverse Plane) 1. Centric 2. Eccentric 3. Cortical 4. Juxta-cortical
  • 51. Centric • SBC,eosinophilicgranuloma,fibrousdysplasia, ABC and enchondroma Eccentric • Osteosarcoma, NOF, chondroblastoma, chondromyxoid fibroma, GCT and osteoblastoma Cortical • Osteoid osteoma Juxta-cortical • Osteochondroma. The cortex must extend into the stalk of the lesion. Parosteal osteosarcoma arises from the periosteum.
  • 52. 1. SBC: central diaphyseal 2. NOF: eccentric metaphyseal 3. SBC: central diaphyseal 4. Osteoid osteoma: cortical 5. Degenerative subchondral cyst: epiphyseal 6. ABC: centric diaphyseal
  • 53. AGE ATPRESENTATION • Age is the most important clinical clue in differentiating possible bone tumors • Primary malignant bone tumours are rare before 5 years of age • 1st decade: Commonly disseminated bone lesions of leukaemia and neuroblastoma • 2nd decade: Commonly an osteosarcoma or Ewing’s sarcoma • > 45 years of age: Metastases are the commonest
  • 54. Specific tumors by age Malignant bone tumors(red) and benign tumors(blue)
  • 55.
  • 56. C. Bone destruction • Bone destruction is the hallmark of a bone tumor. • Three patterns of bone destruction are described 1. Geographic, 2. Moth-eaten, 3. Permeative.
  • 57. Patterns of Bone Destruction (Lodwick Classification) • For describing the margins of a lucent/lytic bone lesion • Used in the description for aggressive and possibly malignant Classification • Type 1: Geographic • Type 2: Moth-eaten • Type 3: Permeative
  • 58. A: Pattern of medullary destruction B: Margination of lesions
  • 59.
  • 60. Geographic Bone Destruction Complete destruction of bone from boundary to normal bone • Non-ossifying fibroma • Chondromyxoid fibroma • Eosinophilic granuloma Non-ossifying fibroma
  • 61. Moth-eaten Bone Destruction • Areas of destruction with ragged borders • Implies more rapid growth • Probably a malignancy Examples: • Myeloma • Metastases • Lymphoma • Ewing’s sarcoma Multiple Myeloma
  • 62. Permeative Bone Destruction • Ill-defined lesion with multiple “worm-holes” • Spreads through marrow space • Wide transition zone • Implies an aggressive malignancy • Round-cell lesions Examples: • Lymphoma, leukemia • Ewing’s Sarcoma • Myeloma • Osteomyelitis • Neuroblastoma Leukemia
  • 63. Patterns of Bone Destruction Geographic Moth-eaten Permeative Less malignant More malignant
  • 64. D. Matrix formation • Calcification of the matrix, or new bone formation, may produce an area of increased density within the lesion. • Calcification typically appears as flocculent or stippled rings or clusters. • The appearance of the new bone varies from dense sclerosis that obliterates all evidence of normal trabeculae to small, irregular, circumscribed masses described as "wool" or "clouds." • Calcification and ossification may appear in the same lesion. • Neither type of matrix formation is diagnostic of malignancy.
  • 65. • Calcifications or mineralization within a bone lesion may be an important clue in the differential diagnosis • Two kinds of mineralization: - Chondroid matrix in cartilaginous tumors - Osteoid matrix in osseous tumors
  • 66.
  • 67. Tumor bone formation, matrix calcification
  • 68. Osseous mineralization • Cloud-like and poorly defined, diffuse matrix mineralization • Trabecular ossification pattern in benign bone forming lesions • Cloud-like or ill-defined amorphous pattern in osteosarcomas • Sclerosis can also be reactive, e.g. in Ewing’s sarcoma or lymphoma.
  • 69. Cloud-like bone formation in osteosarcoma. Notice the aggressive, interrupted periosteal reaction (arrows Trabecular ossification pattern in osteoid osteoma. Notice osteolytic nidus (arrow).
  • 70. Chondroid mineralization • Linear, curvilinear, ring-like, punctuate or nodular • Often central • Peripheral in benign lesions such as a bone infarct or myositis ossificans • Calcifications in chondroid tumors have many descriptions: rings-and-arcs, popcorn, focal stippled or flocculent.
  • 71.
  • 72. Diffuse matrix • Shows ground-glass density • Punctate calcification may also be present • Seen in benign fibrous tumours
  • 73.
  • 74. Expansile Lesions of Bone Multiple myeloma Mets Brown tumor Enchondroma Aneurysmal bone cyst Fibrous dysplasia
  • 75. Expansile Lesions of Bone Multiple myeloma Mets Brown tumor Enchondroma Aneurysmal bone cyst Fibrous dysplasia Renal cell carcinoma
  • 76. Multiple myeloma Mets Brown tumor Enchondroma Aneurysmal bone cyst Fibrous dysplasia Expansile Lesions of Bone
  • 77. Multiple myeloma Mets Brown tumor Enchondroma Aneurysmal bone cyst Fibrous dysplasia Expansile Lesions of Bone
  • 78. E. Periosteal reaction • Periosteal reaction is indicative of malignancy but not pathognomonic of a particular tumor. • Any widening or irregularity of bone contour may be regarded as periosteal activity.
  • 79. Solid Periosteal Reactions Chronic osteomyelitis • Single solid layer or multiple closely apposed and fused layers of new bone attached to the outer surface of cortex resulting in cortical thickening. • It is uniterrupted or continous.
  • 80. Types of Solid Periosteal Reaction • 1. SOLID BUTTRESS • Seen in Aneurysmal bone cyst, chondromyxoid fibroma • 2. Solid Smooth or Elleptical layer • Seen in Osteoid osteoma and osteoblastoma • 3. Undulating type : • Seen in long standing varicosities, periosteitis, chronic lymphaoedema. • 4. Single Lamellar reaction : • Seen in Osteomyelitis, Stress Fractures, Langerhans cell histiocytosis.
  • 81.
  • 82.
  • 83. Interupted Periosteal Reactions • Commonly seen in Aggressive/malignant tumours. – Onion-Peel ( lamellated) – Ewings sarcoma – Gouchers disease – Sunburst – Codman’s triangle Ewing sarcoma
  • 84. Sunburst type of periosteal reaction Fine lines of increased density representing newly formed specules of bone radiate laterally from and at right angles to the surface of the shaft giving a typical sun ray appearance. Osteo-sarcoma
  • 85. Spiculated, vertical or ‘hair-on- end’ periostealreaction • Seen in the most aggressive tumours • E.g. Osteosarcoma or Ewing’s sarcoma • Most rapidly growing lesions may not be associated with any radiographically visible periosteal reaction (as minerali- zation of periosteum can take weeks) A ‘hair-on-end’type vertical periosteal reaction associated with Ewing’s sarcoma. Note also the Codman’s triangle (arrow).
  • 86. Codman’s triangle – When the tumour breaks through the cortex and destroys the newly formed lamellated bone, the remnants of the latter on both ends of the break through area may remain as a triangular structure known as codman triangle. Also seen in Osteosarcoma, Ewings sarcoma, Chronic Osteomyelitis Osteo-sarcoma
  • 87. Ewing sarcoma of femur (AP & Lateral) Mottled, osteolytic lesion (blue circle) with poorly marginated edges in the diaphysis of the bone Sunburst periosteal reaction (red circle) and Lamellated periosteal reaction (whitearrows)
  • 88. Periosteal Reactions Solid onion-peelSunburst Codman’s triangle Less malignant More malignant
  • 89. Wide zone of transition indicates: Malignancy or Infection or Eosinophilic granuloma
  • 90. CORTICAL DESTRUCTION • Common finding • Destruction of cortex by a lytic or sclerotic process • Not very useful in distinguishing between benign and malignant • Complete destruction seen in high- grade malignant lesions and in locally aggressive benign lesions like EG and osteomyelitis. • Uniform cortical bone destruction in benign and low-grade malignant lesions
  • 91. Patterns of cortical disturbance
  • 92. • It delineates intra and extra osseous extent of tumour. • It can reliably distinguish between infection and tumor. • CT scan identifies accurately area of cortical break through, soft tissue extension, medullary spread and proximity of the tumour to neurovascular bundle and evaluating integrity of cortex • Todifferentiate solid and cystic lesions. • Most sensitive investigation to detect Pulmonary mets. CT scan:-
  • 93. • Best imaging – to localise the nidus of an osteiod osteoma, – to detect a thin rim of reactive bone around an aneurysmal bone cyst, – to evluate calcification in a suspected cartilagenous lesion and – to evaluate endosteal cortical erosion in a suspected chodrosarcoma. • Todifferentiate between the neoplastic mass and inflammatory condition : Neoplastic masses displace soft tissue fat planes where as they are obliterated in inflammatory conditions. • It cannot differentiate benign from malignant tumours accurately. • Except in detecting pulmonary mets, Contrast CT is better than plain CT.
  • 94. • It has better contrast discrimination than any other modality. • Helps in detecting skip lesions • Assesses the tumor relationship with adjacent soft tissue, joints and blood vessels. • It can visualize bone marrow content and demonstrate intramedullary extension of neoplasm. MRI:-
  • 95. • It is the investigation of choice in local staging of musculoskeletal tumours. • On MRI, it is not possible to accurately differentiate benign from malignant tumours. But if the following criteria are present, lesion can be considered as a malignant one : – 1. Mass with irregular Border – 2. Non homogenous signal intensity with extra compartmental extension – 3. Peri tumoral edematous reaction. – 4. Soft tissue mass situated deep to fascia and measuring more than 5 cm in greatest diameter is likely to be a sarcoma.
  • 96. • It uses radioactive glucose to locate cancer by observing high glycolysis rates in a malignant tissue metabolism. • It has low specificity as the FDG ( Fluoro labelled deoxy glucose) can also accumulate in benign aggressive and inflammatory lesions. • Also helpful in evaluating the tumour after chemotherapy. • Micromets are better visulaised. PET- Positron Emission Tomography
  • 97. Most reliable means of determining vascular anatomy. • Reactive zone is best seen on early arterial phase, while the intrinsic vascularity is best seen on late venous phase as a tumour blush. • Transcatheter embolisation is done as a definitive treatment in some benign vascular tumours. Angiography
  • 98. Angiography demonstrating vascularity of a tumour Embolization of a vascular lesion, performed at least 6 hours prior to surgery, is expected to significantly reduce intraoperative blood loss.
  • 99. • Technetium (99mTC) bone scans are used. • It is an indicator for mineral turnover. • Whenever there is altered local metabolism in remodeling bone, increased vascularity or mineralization , the isotope uptake is enhanced mainly in reactive zone surrounding the lesions. • Confirms epiphyseal spread of tumour. • Helps in detecting multiple lesions like multiple osteochondroma, enchondroma. • Where as a MRI helps in detecting skip lesions Nuclear Imaging -Bone scan Scinitigraphy
  • 100. Bone scan showing HOT SPOTS over proximal humerus and ribs It detects the presence of skeletal metastasis and delineates it from primary else where in the body.
  • 101. • Bone scinitigraphy tends to show larger area of extension of medullary involvement of tumour as the radio active agent also localises the area of hyperemia and edema adjacent to tumour. • Nuclear imaging is advantageous only to identifying whether skeletal involvement is solitary or multiple.
  • 102. • Not routinely used in diagnosis of sarcoma; as it better differentiates only bony cystic lesions. • However Ultrasound is used in guided percutaneous biopsy. • In patients treated with prosthetic implants, USG is the modality that depicts early recurrence as MRI produces blurred and artifact images due to metallic implants. Ultrasound
  • 103.  Used for definitive diagnosis. • Principles of biopsy:  Opted only after all other investigations are performed.  A biopsy can be done by FNAC, core needle biopsy, or an open incisional procedure.  FNAC may be 90% accurate at determining malignancy; however, its accuracy at determining specific tumor type is much lower. – Trephine or core biopsy is recommended and often yields an adequate sample for diagnosis. – Complications are greater with incisional biopsy; but least likely to be associated with a sampling error, and provides the sample for additional diagnostic studies, such as cytogenetics and flow cytometry. Biopsy
  • 104. – Core biopsy is preferred if limb spraying is an option as it entails less contamination than open biopsy. – A small incisional biopsy can be performed if core biopsy specimen is inadequate. – Performed under torniquet (possibly) - the limb may be elevated before inflation but should not be exsanguinated by compression bandage.  Longitudinal incisions preferred as transverse excision are extremely difficult or impossible to excise with the specimen.  NV bundle not exposed.  Dissection through muscle (not between) to prevent contamination of tumour.
  • 105.  Approach for open biopsy is made through region of definitive surgical excision.  If a drain is used, it should exit in line with the incision so that the drain track also can be easily excised en bloc with the tumor.  Wound is closed tightly in layers.  Meticulous haemostasis is arrested by use of bone wax/ Poly Methyl Metha Acrylate(PMMA) to plug the cortical window.  Always sample the tissues from periphery of lesion which contains most viable tissue.  Never biopsy a periosteal reaction / codmans traingle as it contains a new reactive bone and could be false negative.
  • 106. •If hole must be made in bone during biopsy, defect should be round or oval to minimize stress concentration, which otherwise could lead to pathological fracture. •Torsional strength is not affected by length of defect. Always attempt to keep defects less than 10% of bone diameter. •When biopsy size is greater than 20% of bone diameter, torsional strength decreases to 50%.
  • 107. Examples of poorly performed biopsies Biopsy resulted in irregular defect in bone, which led to pathological fracture
  • 108. Examples of poorly performed biopsies Transverseincisions should not be used Needle biopsy track contaminated patellar tendon Multiple needle tracks contaminate quadriceps tendon
  • 109. Drain site was not placed in line with incision Needle track placed posteriorly, location that would be extremely difficult to resect en bloc with tumor if it had proved to be sarcoma
  • 110. • Biopsy should be done only after clinical, laboratory, and radiographic examinations are complete. • Completion of the evaluation before biopsy aids in planning the placement of the biopsy incision, helps provide more information leading to a more accurate pathological diagnosis, and avoids artifacts on imaging studies. • If the results of the evaluation suggest that a primary malignancy is in the differential diagnosis, Biopsy is not done unless it is possible to operate the case in the centre.
  • 111. • Curettage resection and restoration of function by limb salvage procedures or amputation is primary form of surgical correction. • Based on surgical plane of dissection in relation to tumour, Enneking formulated following types of resection. 1) Intralesional Resection 2) Marginal resection 3) Wide (Intracompartmental) resection 4) Radical (Extracompartmental) resection SURGICAL OPTIONS
  • 112. • An intralesional margin is one in which the plane of surgical dissection is within the tumor. • This type of procedure is often described as “debulking” because it leaves behind gross residual tumor. • This procedure may be appropriate for symptomatic benign lesions when the only surgical alternative would be to sacrifice important anatomical structures. • This also may be appropriate as a palliative procedure in the setting of metastatic disease. • Intralesional resection is through the psuedocapsule of the tumor directly in to the lesion. Macroscopic tumour is left behind. Curettage is intralesional proceedure. 1) Intralesional Resection :
  • 113. CURETTAGE • Cortical window with rounded margins is made • When possible, the window is sized larger than the tumour so that the entire tumour is readily seen. • The rounded margins reduce the risk of subsequent fracture. • Large curetts should be used to remove the lesional tissue. • Tumour margin should be treated with cryotherapy, PMMA cementage or phenol – alcohol cauterisation, argon beam coagulation in case of aggressive tumours. • If curettage weakens the bone, graft using allograft or autograft with or with out internal fixation is indicated.
  • 114. • A marginal margin is achieved when the closest plane of dissection passes through the pseudocapsule. • This type of margin usually is adequate to treat most benign lesions and some low-grade malignancies. • In high-grade malignancy, however, the pseudocapsule often contains microscopic foci of disease, or “satellite” lesions. • A marginal resection often leaves behind microscopic disease that may lead to local recurrence if the remaining tumor cells do not respond to adjuvant chemotherapy or radiation therapy. 2) Marginal Resection
  • 115. • Intracompartmental • Wide margins are achieved when the plane of dissection is in normal tissue. • Although no specific distance is defined, the Resection includes removal of entire tumour, + Reactive zone & cuff of normal tissue. • If the plane of dissection touches the pseudocapsule at any point, the margin should be defined as being marginal and not wide. • Although sometimes impossible to achieve, wide margins are the goal of most procedures for high-grade malignancies. 3) Wide Resection
  • 116. 4] Radical Radical margins are achieved when all the compartments that contain entire tumor and structure or origin of lesion are removed en bloc. The plane of dissection is beyond the limiting fascial & bone borders. For deep soft-tissue tumors, this involves removing the entire compartment (or multiple compartments) of any involved muscles. However, with improvements in imaging studies, radical procedures now are rarely performed because equivalent oncological results usually can be obtained with wide margins .
  • 117. ENNEKING`s Classification of Surgical Procedures for Bone Tumors Margin Local (Mode of resection) LIMB SALVAGE OPTIONS Amputation(Mode of amputation) Intralesional Curettage or debulking Debulking amputation Marginal Marginal excision Marginal amputation Wide Wide local excision Wide through bone, amputation Radical Radical local resection Radical disarticulation
  • 120. 1] Limb Salvage Proceedures 2] Amputations SURGICAL OPTIONS
  • 121. • It is designed to accomplish removal of a malignant tumour & reconstruction of the limb with an acceptable oncologic, functional & cosmetic result. • It is sub amputative wide resection with preservation of the limb & its function. • Indications : • Stage IA Stage IIA & Stage IIIA (All intracompartmental tumours)with good response to pre-operative chemotherapy • Skin should be uninvolved and free • There should be feasibility of keeping a cuff of normal tissue surrounding the tumour • Upper extremity lesions are more suitable for limb sparing surgery  Tumours with good pre-operative chemotherapy response LIMB SALVAGE PROCEDURES
  • 122. Exclusion Criteria Patients with bone tumors in whom limb salvage was not possible. The criteria used to determine if the limb was worthy of salvage were as follows: 1. The major neurovascular bundle is free of tumor. 2. Wide resection of the affected bone with a normal muscle cuff in all directions is possible. 3. All previous biopsy sites and all potentially contaminated tissues can be removed en bloc. 4. Bone can be resected 3 to 4 cm beyond abnormal uptake as determined by bone scan
  • 123. 5. The adjacent joint and joint capsule can be resected. 6. Adequate motor reconstruction can be accomplished by regional muscle transfers. 7. Adequate soft tissue coverage is possible to decrease the risk of skin flap necrosis and secondary infection.
  • 124. • Reconstruction of bone defect may be done by 1 Osteoarticular allograft reconstruction 2Allograft-prosthesis composite reconstruction 3] Endoprosthetic reconstruction. 4] Allograft arthrodesis 5} Rotationplasty 6} Turnoplasty Surgical reconstructive options :
  • 125. • Amputation provides definitive surgical treatment when limb sparing is not a prudent one. • Common amputations in malignant tumours: – Proximal humerus : fore quarter amputation – Distal femur : hip disarticulation – Proximal tibia : mid thigh amputation AMPUTATIONS
  • 126. • Adjuvant chemotherapy: – Totreat presumed micrometastasis • Neo adjuvant[induction] – Before surgical resection of the primary tumour Advantages: – It controls micro metastasis and improves survival rate. – Chemotherapy makes limb salvage surgery easier. – Decreases tumor size and vascularity. – The response to Chemotherapy can be evaluated after surgery. Chemotherapy
  • 127. • With Megavoltage radiotherapy tumor cell can be destroyed. • High voltages are administered in short sessions. • Radiation therapy should be started immediately after diagnosis before surgery to prevent metastasis . • Chemotherapy increases the susceptibility of tissues to irradaition. • Protect all normal tissue biopsy scars to prevent radiation necrosis. • Distribute the dose in accordance with distribution of tumor. RADIOTHERAPY
  • 128. Diagnosis Neoadjuvant chemotherapy + Radiation Resection/ surgery Repeat Chemo + Radiation Adjuvant + irradiation +chemo Histological Grading SEQUENCE OF TREATMENT
  • 129. • Gene therapy in sarcomas : • Targeting Osteocalcin promoter. • Osteocalcin is produced both in Osteoblastic[100%]and fibroblastic[70%] Osteosarcoma. • LIQUID BRACHYTHERAPY : – Injecting Intra arterial infusion of chemotherapeutic drugs with brachytherapy – It is in Phase 2 trials currently. • Cryotherapy is used in curettage after resection of primary tumour to prevent chances of recurrence. • PMMA, Phenol, liquid nitorgen commonly used cryoprecipitates. RECENT ADVANCES