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Dr Ranjith Kumar Yalamanchili
MS(Ortho)
Introduction :
• Neoplasia : It is defined as a mass of tissue
formed as result of abnormal, excessive,
uncoordinated, aut...
History :
• Gross in 1879 presented a paper on sarcoma of long bones
where he has clearly mentioned amputation as the
trea...
FEATURES BENIGN (DIFFERENTIATED) MALIGNANT (UNDIFFERENTIATED)
MACROSCOPIC FEATURES
Boundaries Encapsulated or well
circums...
Routes of Metastasis :
• Haematogenous Spread : Most common spread in
musculoskeletal tumours
• Lymphatic Spread – rare in...
Classification of Tumours
1. WHO Classification :
widely accepted and is based on histogenesis
and histological criteria.
...
Modified WHO Classification of BONE TUMOURS **
** www.springer.de.catalog/nsten/who
• 1. Bone forming tumours :
Benign :
...
2. Cartilage forming Tumours
• BENIGN :
• Chondroma
• Enchondroma
• Osteochondroma
• Chondroblastoma
• Chondromyxoid fibro...
3. Giant Cell Tumours :
– Osteoclastoma
– Malignant giant cell tumour
– Giant cell tumour in Pagets disease
– GCT occurrin...
4. Marrow tumours
•Ewing sarcoma
•Reticulosarcoma
•Lymphosarcoma
•Myeloma
5. Vascular Tumours
–Benign :
» Haemangioma
» Lymphangioma
» Glomus Tumour
–Intermediate or inderminate variant :
» Haeman...
6. Other Connective tissue tumours
• Benign :
» Benign fibrous histiocytoma
» Lipoma
• Intermediate :
» Desmoplastic fibro...
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)
•...
Classification based on origin of tumours
1. Primary Bone tumours : Derived from bone
2. Metastatic bone Tumours : Due to ...
Classification based on origin of tumours
1. Primary Bone tumours : Derived from bone
2. Metastatic bone Tumours : Due to ...
Classification based on site of Origin
1. Epiphyseal
Osteoclastoma, Chondroblastoma
2. Metaphyseal
Osteioid osteoma, Osteo...
General Concepts in Tumour Terminology
• True Capsule :
• Surrounds a benign lesion and is composed of
compressed normal c...
• Compartment :
It refers to bone or muscle of origin;
– For Muscle, compartment is that within its Fascia.
– For Bone :
•...
• Skip Metastasis :
• A skip metastasis, is defined as a tumor nodule that is
located within the same bone as the main tum...
Satellite lesion
Tumour nodule
within reactive zone.
Intra Osseous Skip Mets :
Embolization of tumour
cells within the mar...
GRADING and STAGING of TUMOURS
• To determine prognosis and choice of treatment.
GRADING :
• It is defined as macroscopic ...
Enneking`s Grading of Tumours
• G0 Histologically benign
(well differentiated and low cell to matrix ratio)
• G1 Low grade...
STAGING OF TUMOURS :
• STAGING is defined as extent of spread of tumour.
– It is determined by clinical examination, Inves...
ENNEKING`s STAGING OF BENIGN
TUMOURS
1. Latent—low biological activity; well marginated;
often incidental findings (i.e., ...
• GTM classification described by Enneking is
adopted by Musculoskeletal Tumour society
and is based on surgical grading (...
American joint committee on cancer system bone
sarcoma classification (AJCC Classification)
The AJCC system for bone sarco...
TNM STAGING (Union International Cancer
centre Geneva Staging System)
• T – Primary Tumour – T0 to T4
– In Situ lesion T0 ...
CLINICAL PRESENTATION :
• Pain :
– Initially may be activity related, but in case of malignancy
there could be progressive...
• In case of soft tissue sarcomas patients may come
with mass rather than pain but in some exceptions
like nerve sheath tu...
INVESTIGATIONS
Serological investigations :
• 1. Complete Blood Picture :
– Haemoglobin : to rule out anaemia that may be ...
 3. Serum alkaline phosphatase (ALP)
 Biological marker of tumour activity.
 Increases significantly when tumour and me...
 4. Antisarcoma Antibodies :
 Monoclonal antibodies can be detected by
immunohistochemical assays.
 Antibodies binding ...
Flow Cytometry
• A sample of cells are treated with special antibodies
that stick to the cells only if certain substances ...
INVESTIGATIONS: RADIOGRAPHS
• Phemister's Law = the most common site of
infection & tumours is the fastest growing site
of...
Radiographic Evaluation
• Five important parameters in evaluating a
tumour on a X RAY are
1. Anatomic site
2. Borders
3. B...
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
• Ad...
Characteristic Locations
• Simple bone cyst
Proximal humerus
• Chondroblastoma
Epiphyses
• Giant Cell tumor
Epiphyses
• Ad...
• Simple bone cyst
Proximal humerus
• Chondroblastoma
Epiphyses
• Giant Cell tumor
Epiphyses
• Adamantinoma
Tibia
• Chordo...
• Simple bone cyst
Proximal humerus
• Chondroblastoma
Epiphyses
• Giant Cell tumor
Epiphyses
• Adamantinoma
Tibia
• Chordo...
• Simple bone cyst
Proximal humerus
• Chondroblastoma
Epiphyses
• Giant Cell tumor
Epiphyses
• Adamantinoma
Tibia
• Chordo...
• Simple bone cyst
Proximal humerus
• Chondroblastoma
Epiphyses
• Giant Cell tumor
Epiphyses
• Adamantinoma
Tibia
• Chordo...
B. Borders
• The border reflects the growth rate and the response of the
adjacent normal bone to the tumor.
• Most tumors ...
C. Bone destruction
• Bone destruction is the hallmark of a bone
tumor.
• Three patterns of bone destruction are
described...
Geographic Bone Destruction
Complete destruction
of bone from
boundary to normal
bone
• Non-ossifying fibroma
• Chondromyx...
Moth-eaten Bone Destruction
• Areas of destruction with
ragged borders
• Implies more rapid growth
• Probably a malignancy...
Permeative Bone Destruction
• Ill-defined lesion with multiple “worm-holes”
• Spreads through marrow space
• Wide transiti...
Examples:
• Lymphoma, leukemia
• Ewing’s Sarcoma
• Myeloma
• Osteomyelitis
• Neuroblastoma
Permeative Bone Destruction
Leu...
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 ...
• Calcification and ossification may appear in
the same lesion.
• Neither type of matrix formation is diagnostic
of malign...
Tumor Matrix
• Osteoblastic
– Fluffy, cotton-
like or cloud-
like densities
– Osteosarcoma
Cartilaginous :
– Comma-shaped,
punctate, annular,
popcorn-like
as Enchondroma,
chondrosarcoma,
chondromyxoid
fibroma
Tumo...
Expansile Lesions of Bone
Multiple myeloma
Mets
Brown tumor
Enchondroma
Aneurysmal bone cyst
Fibrous dysplasia
Multiple myeloma
Mets
Brown tumor
Enchondroma
Aneurysmal bone cyst
Fibrous dysplasia
Expansile Lesions of Bone
Renal cell ...
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.
• An...
Solid Periosteal Reactions
Chronic osteomyelitis
• Single solid layer or multiple
closely apposed and fused
layers of new ...
Types of Solid Periosteal Reaction
• 1. SOLID BUTRESS
• Seen in Aneurysmal bone cyst, chondromyxoid fibroma
• 2. Solid smo...
Interupted Periosteal Reactions
• Commonly seen in
Aggressive/malignant
tumours.
– onion-peel ( lamellated)
– Ewings sarco...
– Sunburst TYPE OF periosteal
reaction :
Fine lines of increased
density representing newly
formed specules of
bone radia...
Codman’s triangle :
– When the tumour breaks
through the cortex and
destroys the newly formed
lamellated bone, the remnant...
Periosteal Reactions
Solid onion-peelSunburst Codman’s
triangle
Less malignant More malignant
Radiographic Features in a Benign vs.
Malignant
Clues by density of lesions
Sclerotic Cortical Lesions
• Osteoid osteoma
• Brodie’s abscess
• Osteoid osteoma
• Brodie’s abscess
Sclerotic Cortical Lesions
Mnemonic for Luscent bone lesions =
FOGMACHINES
Fibrous Dysplasia
Osteoblastoma
Giant Cell Tumour
Metastasis/ Myeloma
Aneu...
• Metastatic lesions
– Lung
– Renal
– Thyroid
• Multiple myeloma
• Primary bone tumor
Lytic Lesions in Adults
• Metastatic lesions
– Lung
– Renal
– Thyroid
• Multiple myeloma
• Primary bone tumor
Chondrosarcoma
Lytic Lesions in Adul...
• Lymphoma
Blastic Lesions in Children
• Metastatic disease
• Breast –female
• Prostate –male
• Lymphoma
• Paget’s disease
• Fluorosis
Prostatic Ca.
Blastic Lesi...
• Metastatic disease
• Breast –female
• Prostate –male
• Lymphoma
• Paget’s disease
• Fluorosis
Blastic Lesions in Adults
• Metastatic disease
• Breast –female
• Prostate –male
• Lymphoma
• Paget’s disease
• Fluorosis
Blastic Lesions in Adults
Sled runner tracks in
Olliers disease
Mafuccis syndrome – olliers +
multiple angiomas
Jail house pattern seen in
Haemangioma on X Rays
thickened, vertically
oriented trabeculae
Polka dot pattern is
seen in cr...
• It delineates intra and extra
osseous extent of tumour.
• It can reliably distinguish
between infection and tumor.
• CT ...
• Best imaging
– to localise the nidus of an osteiod osteoma,
– to detect a thin rim of reactive bone around an aneurysmal...
• It has better contrast
discrimination than any other
modality.
• Helps in detecting skip lesions
• Assesses the tumor re...
• It is the investigation of choice in local staging of
musculoskeletal tumours.
• On MRI, it is not possible to accuratel...
• It uses radioactive glucose to locate cancer by observing high
glycolysis rates in a malignant tissue metabolism.
• This...
Most reliable means of determining vascular anatomy.
• Reactive zone is best seen on early arterial phase,
while the intri...
Angiography demonstrating
vascularity of a tumour
Embolization of a vascular
lesion, performed at least 6
hours
prior to s...
• Technetium (99mTC) bone scans are used.
• It is an indicator for mineral turnover.
• Whenever there is altered local met...
Bone scan showing HOT
SPOTS over proximal
humerus and ribs
It detects the presence of
skeletal metastasis and
delineates i...
• Bone scinitigraphy tends to show larger area of
extension of medullary involvement of tumour
as the radio active agent a...
• Not routinely used in diagnosis of sarcoma; as it
better differentiates only bony cystic lesions.
• However Ultrasound i...
 Used for definitive diagnosis.
• Principles of biopsy:
 Opted only after all other investigations are performed.
 A bi...
– Core biopsy is preferred if limb spraying is an option as it entails
less contamination than open biopsy.
– A small inci...
 Approach for open biopsy is made through region of definitive
surgical excision. If a drain is used, it should exit in l...
• If hole must be made in bone during biopsy, defect should be
round or oval to minimize stress concentration, which
other...
Examples of poorly performed biopsies
Biopsy resulted in irregular defect in bone, which led to pathological
fracture
Examples of poorly performed
biopsies
Transverse incisions
should not be used
Needle biopsy track
contaminated patellar
te...
Drain site was not placed in line with
incision
Needle track placed posteriorly,
location that would be extremely
difficul...
• Biopsy should be done only after clinical, laboratory,
and radiographic examinations are complete.
• Completion of the e...
Criteria for prophylactic fixation of
metastatic tumours of long bones
• Surgical Fixation should not be proceeded until
p...
Harington's criteria
of prophylactic fixation
• > 50% destruction of diaphyseal cortices
• > 50-75% destruction of metaphy...
Mirel`s criteria of fixation
General principles &Technique for En Bloc
Tumour excision
Tourniquet
– For fear of embolic expression, exsanguinations of
...
Advantages of Tourniquet
• Prevention of tumour embolisation
• Ease of distinguishing neoplastic tissue from
normal tissue...
Incision
• A long vertical incision well above and below the
visible limits of the tumour.
• Incision should include the b...
Wound contamination
• Incidence of recurrence is approximately doubled in
case of a wound contamination due to the maligna...
• Curettage resection and restoration of function by
limb salvage procedures or amputation is primary
form of surgical cor...
• An intralesional margin is one in which the plane of surgical
dissection is within the tumor.
• This type of procedure i...
• As musculoskeletal tumors grow, they
compress the surrounding tissues and appear to
become encapsulated.
• This surround...
CURETTAGE
• Cortical window with rounded margins is made
• When possible, the window is sized larger than
the tumour so th...
Curettage
• Tumour margin should be treated with
cryotherapy, PMMA cementage or phenol –
alcohol cauterisation, argon beam...
• A marginal margin is achieved when the closest plane of dissection
passes through the pseudocapsule.
• This type of marg...
• Despite an increased risk of local recurrence, a marginal
resection may be preferable if the alternative is a more
mutil...
• Intracompartmental
• Wide margins are achieved when the plane of dissection is in
normal tissue.
• Although no specific ...
4] Radical :-
Extracompatmental:
Radical margins are achieved when all the compartments that
contain entire tumor and str...
For bone tumors, this involves removing the entire
bone and the compartments of any involved muscles.
 Radical operation...
ENNEKING`s Classification of Surgical
Procedures for Bone Tumors
Margin Local (Mode of
resection)
LIMB SALVAGE
OPTIONS
Amp...
Enneking`s
Classification of
Resection of
tumours.
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, fu...
Advantages of limb salvage proceedures :
• long term survival rates of patients have improved from
approximately 20% to 70...
Disadvantages of limb salvage proceedures:
 Limb salvage is more extensive procedure with greater
risk of infection, wou...
Guidelines:
 No major neurovascular tumour involvement.
 Wide resection of the affected bone with normal
muscle cuff in...
1. Major neurovascular involvement.
2. Pathological fractures spread tumour cells through fracture
hematoma increasing ris...
1. Resection of the tumour
To avoid local recurrence.
2. Skeletal reconstruction
Technique of reconstruction is independen...
• Reconstruction of bone defect may be done by
1] Osteoarticular allograft reconstruction
2] Allograft-prosthesis composit...
• Possibility to replace ligaments, tendons & intraarticular structures
• Osteoarticular allografts may have a role as a t...
2.Allograft-prosthesis composite reconstruction:
Main indication for an allograft-
prosthesis composite is an inadequate
l...
Intraoperative photograph after wide
resection of tumour.
Humeral allograft is prepared to accept
stem of tumor prosthesis...
Prosthesis is
cemented into
allograft.
Postoperative
radiograph.
• It also provide long-term function for
some patients.
• Endoprosthetic reconstruction provides the
advantage of predicta...
• Polyethylene wear is still a limiting factor for
articulating surfaces, but the inserts are easily
replaceable in most p...
4. Resection Arthrodesis – Ennekings shirney
5. ROTATIONPLASTY :
Winkelmann classified ROTATIONPLASTY
into five groups, as follows:
1. Group AI
– Lesion in distal femur.
– The distal femu...
• The distalmost femur, knee joint, and proximal tibia
are resected. After rotation of 180 degrees, the distal
tibia is jo...
• The upper femur and hip joint are
resected, and the leg is rotated
180 degrees. The distal femur is
joined to the pelvis...
• The upper femur, hip joint,
and lower hemipelvis are
resected, and the leg is rotated
180 degrees.
The remaining femur
i...
• The entire femur is resected.
• The tibia is attached to the pelvis using an
endoprosthesis.
5. Group BIII—Lesion in the...
• "TuRN-up PLAsTy" of the tibia refers to the replacement of a femur
by substituting the iniverted tibia and fibula. This ...
Two types of prosthesis are used:
• 1. modular
• 2. custom made
7. PROSTHETIC ARTHROPLASTY
Custom made
prosthesis to
accomodate large
proximal femoral
defects after resection
• A tailor- made metallic prosthesis for a particular patient with
specific measurements is called custom prosthesis.
• De...
• For pediatric patients, future limb-length inequality must be
considered.
• For patients who are near skeletal maturity,...
• The surgical technique, postoperative course, rehabilitation,
function, and complications for implantation of this devic...
Electromagnetic expansion of
Repiphysis expandable
prosthesis.
• Amputation provides definitive surgical treatment when limb
sparing is not a prudent one.
• Common amputations in malign...
Ennekings Classification of
Amputations
• Levels of
Above knee
amputation :
• Adjuvant chemotherapy:
– To treat presumed micrometastasis
• Neo adjuvant[induction]
– Before surgical resection of the ...
• Here the chemotherapy is intituted after the primary
tumour has been controlled by alternative treatment
such as surgery...
• Chemotherapy that is given before local
resection is considered neoadjuvant
chemotherapy.
• The most common reason for n...
Neoadjuvant chemotherapy
Advantages Disadvantages
• Early institution of systemic
therapy against
micrometastases
• Less c...
Adjuvant chemotherapy
Advantages Disadvantages
• Removal of bulk tumour
decreases tumour burden
and increases growth rate
...
GRADE EFFECT
I Little or No response of tumour identified
II Areas of necrosis and tumour cells seen.
III Scattered foci o...
GRADE EFFECT
I No viable tumour cells
II Single tumour cells or clusters <0.5cm
III Viable tumour cells <10%
IV Viable tum...
• With Megavoltage radiotherapy tumor cell can be destroyed.
• High voltages are administered in short sessions.
• Radiati...
Diagnosis
Neoadjuvant chemotherapy + Radiation
Resection/ surgery Repeat Chemo + Radiation
Adjuvant + irradiation +chemo H...
Gene therapy in sarcomas :
• Targeting Osteocalcin promoter.
• Osteocalcin is produced both in Osteoblastic[100%]and fibro...
Internal or Sealed source chemotherapy.
• Brachy means close/short distance.
• Source is placed near to the target tissue ...
• A portion of tumour is implanted in to a sarcoma
survivor and removed after 14 days.
• Sensitized lymphocytes from survi...
Extracorporeal Irradiation
• En-bloc resection, extracorporeal irradiation, and
re-implantation is done in limb salvage fo...
Extracorporeal Irradiation
• Meanwhile, the operative site is prepared for re-
implantation and marginal biopsies taken.
•...
Cementoplasty
• Patients with osteolytic tumours (metastasis, multiple
myeloma, lymphoma) located to the vertebral body,
a...
LASER ABLATION for Benign Tumours
• Because of the small size of the ablation zone produced, laser
is mostly used for smal...
Direct intra arterial chemotherapy
• Usually in chemotherapy is given intra
venously, but direct intra arterial
administra...
ISOLATED LIMB
PERFUSION METHOD
Effective way of delivering
chemotherapy to the affected
limb alone reducing systemic side
...
Oncologist BoneTumours
Diagnosis
Treatment
Radiologist
Cytopathologist
Surgeon
Histopathologist
Molecular
Pathologist
Gene...
References :
• Campbell’s Operative Orthopaedics – 11th & 12th edition
• Samuel Turek Text Book of Orthopaedics: 2nd editi...
Buzzwords: in Bone Tumours
• Ground glass fibrous dysplasia
• salt & pepper appearance Hyperparathyroidism,Paraganglionoma...
Classification of tumours and general principles of management of tumours
Classification of tumours and general principles of management of tumours
Classification of tumours and general principles of management of tumours
Classification of tumours and general principles of management of tumours
Classification of tumours and general principles of management of tumours
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Classification of tumours and general principles of management of tumours

Brief revision regarding orthopaedic bone tumours principles, classification and Management priniciples

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Classification of tumours and general principles of management of tumours

  1. 1. Dr Ranjith Kumar Yalamanchili MS(Ortho)
  2. 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. 3. History : • Gross in 1879 presented a paper on sarcoma of long bones where he has clearly mentioned amputation as the treatment of choice. • Codman in 1926 provided statistical proof of prognosis of musculoskeletal tumours • Blood Good in 1928 recommended resection and bone transplantation in order to restore function. He is considered as father of limb sparing surgery. • In 1930 Radiotherapy came in to light for treatment of metastasis and primary musculoskeletal tumours • Coley in 1936 attempted chemotherapy using streptococcus pyogenes which he called as TOXIN THERAPY. • Moore and Bohlman in 1943 introduced Ëndoprosthesis in treating GCT of femur.
  4. 4. 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 Malignant Tumours
  5. 5. 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 Cerebro Spinal Fluid : – malignant tumours of ependyma and leptomeninges rarely spread through CSF to vertebrae.
  6. 6. 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.
  7. 7. Modified WHO Classification of BONE TUMOURS ** ** www.springer.de.catalog/nsten/who • 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
  8. 8. 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
  9. 9. 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 6- non osteogenic fibroma 7- fibrous dysplasia 8- brown tumor of hyperparathyroidism .
  10. 10. 4. Marrow tumours •Ewing sarcoma •Reticulosarcoma •Lymphosarcoma •Myeloma
  11. 11. 5. Vascular Tumours –Benign : » Haemangioma » Lymphangioma » Glomus Tumour –Intermediate or inderminate variant : » Haemangio endothelioma » Hemangio pericytoma –Malignant : » Angiosarcoma » Malignant haemangio pericytoma
  12. 12. 6. Other Connective tissue tumours • Benign : » Benign fibrous histiocytoma » Lipoma • Intermediate : » Desmoplastic fibroma • Malignant : » Fibrosarcoma » Liposarcoma » Malignant mesenchymoma » Malignant fibrous histiocytoma » Leiomyosarcoma » Undifferentiated sarcoma
  13. 13. 7. Other Tumours • Chordoma • Adamantinoma • Neurilemmoma • Neurofibroma 8. Unclassified Tumours
  14. 14. 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 • Gaint cell granuloma
  15. 15. Classification based on origin of tumours 1. Primary Bone tumours : Derived from bone 2. Metastatic bone Tumours : Due to Mets from : –Breast Lytic + Blastic lesions -Kidney Lytic –Prostate Blastic - Adrenal Lytic –Thyroid Lytic - Intenstine Lytic - Lung, Liver 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.
  16. 16. Classification based on origin of tumours 1. Primary Bone tumours : Derived from bone 2. Metastatic bone Tumours : Due to Mets from : B –Breast Lytic + Blastic lesions K -Kidney Lytic P –Prostate Blastic A- Adrenal Lytic T –Thyroid Lytic I- Intenstine Lytic L- Lung, Liver Lytic University - Urinary Bladder, Uterine Cervix Lytic lesions • {Mnemonic : B.K.PATIL University} 3.
  17. 17. 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,
  18. 18. 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.
  19. 19. • 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.
  20. 20. • 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.
  21. 21. Satellite lesion Tumour nodule within reactive zone. Intra Osseous Skip Mets : Embolization of tumour cells within the marrow sinusoids. Transarticular Skip Mets : Occur via periarticular venous anastamosis – Very poor prognosis
  22. 22. 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 : Well differentiated; <25% Anaplastic cells – GRADE II : Moderately Differentiated; 25-50% Anaplastic cells – GRADE III : Moderately differentiated; 50-75% Anaplastic cells – GRADE IV: Poorly differentiated; >75% Anaplastic cells
  23. 23. 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
  24. 24. 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 )
  25. 25. ENNEKING`s STAGING OF BENIGN TUMOURS 1. Latent—low biological activity; well marginated; often incidental findings (i.e., nonossifying fibroma) 2. Active—symptomatic; limited bone destruction; 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)
  26. 26. • 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
  27. 27. 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.
  28. 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 to M2 – No Metastasis M0 to distant metastasis M2
  29. 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. 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. 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 with prostatic acid phosphatase levels in a case of blastic lesions of x ray is the diganostic of Mets secondary to Prostate Carcinoma
  32. 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. 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. 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. 35. INVESTIGATIONS: RADIOGRAPHS • Phemister's Law = the most common site of infection & tumours is the fastest growing site of the long bone • To see 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. 36. Radiographic Evaluation • Five important parameters in evaluating a tumour on a X RAY are 1. Anatomic site 2. Borders 3. Bone destruction 4. New Matrix ( Bone) formation 5. Periosteal reaction
  37. 37. A. Anatomic Sites – X ray • Anatomic site Specific anatomic sites of the bone give rise to specific groups of lesions.
  38. 38. Characteristic Locations • Simple bone cyst Proximal humerus • Chondroblastoma Epiphyses • Giant Cell tumor Epiphyses • Adamantinoma Tibia • Chordoma Sacrum, clivus • Osteoblastoma Spine, posterior
  39. 39. Characteristic Locations • Simple bone cyst Proximal humerus • Chondroblastoma Epiphyses • Giant Cell tumor Epiphyses • Adamantinoma Tibia • Chordoma Sacrum, clivus • Osteoblastoma Spine, posterior
  40. 40. • Simple bone cyst Proximal humerus • Chondroblastoma Epiphyses • Giant Cell tumor Epiphyses • Adamantinoma Tibia • Chordoma Sacrum, clivus • Osteoblastoma Spine, posterior Characteristic Locations
  41. 41. • Simple bone cyst Proximal humerus • Chondroblastoma Epiphyses • Giant Cell tumor Epiphyses • Adamantinoma Tibia • Chordoma Sacrum, clivus • Osteoblastoma Spine, posterior Characteristic Locations
  42. 42. • Simple bone cyst Proximal humerus • Chondroblastoma Epiphyses • Giant Cell tumor Epiphyses • Adamantinoma Tibia • Chordoma Sacrum, Pelvis • Osteoblastoma Spine, posterior Characteristic Locations
  43. 43. • Simple bone cyst Proximal humerus • Chondroblastoma Epiphyses • Giant Cell tumor Epiphyses • Adamantinoma Tibia • Chordoma Sacrum, clivus • Osteoblastoma Spine - posterior elements Characteristic Locations
  44. 44. 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
  45. 45. 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.
  46. 46. Geographic Bone Destruction Complete destruction of bone from boundary to normal bone • Non-ossifying fibroma • Chondromyxoid fibroma • Eosinophilic granuloma Non-ossifying fibroma
  47. 47. 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
  48. 48. Permeative Bone Destruction • Ill-defined lesion with multiple “worm-holes” • Spreads through marrow space • Wide transition zone • Implies an aggressive malignancy • Round-cell lesions
  49. 49. Examples: • Lymphoma, leukemia • Ewing’s Sarcoma • Myeloma • Osteomyelitis • Neuroblastoma Permeative Bone Destruction Leukemia
  50. 50. Patterns of Bone Destruction Geographic Moth-eaten Permeative Less malignant More malignant
  51. 51. 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."
  52. 52. • Calcification and ossification may appear in the same lesion. • Neither type of matrix formation is diagnostic of malignancy.
  53. 53. Tumor Matrix • Osteoblastic – Fluffy, cotton- like or cloud- like densities – Osteosarcoma
  54. 54. Cartilaginous : – Comma-shaped, punctate, annular, popcorn-like as Enchondroma, chondrosarcoma, chondromyxoid fibroma Tumor Matrix Chondrosarcoma
  55. 55. Expansile Lesions of Bone Multiple myeloma Mets Brown tumor Enchondroma Aneurysmal bone cyst Fibrous dysplasia
  56. 56. Multiple myeloma Mets Brown tumor Enchondroma Aneurysmal bone cyst Fibrous dysplasia Expansile Lesions of Bone Renal cell carcinoma
  57. 57. Multiple myeloma Mets Brown tumor Enchondroma Aneurysmal bone cyst Fibrous dysplasia Expansile Lesions of Bone
  58. 58. Multiple myeloma Mets Brown tumor Enchondroma Aneurysmal bone cyst Fibrous dysplasia Expansile Lesions of Bone
  59. 59. 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.
  60. 60. 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.
  61. 61. Types of Solid Periosteal Reaction • 1. SOLID BUTRESS • 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.
  62. 62. Interupted Periosteal Reactions • Commonly seen in Aggressive/malignant tumours. – onion-peel ( lamellated) – Ewings sarcoma – Gouchers disease – Sunburst – Codman’s triangle Ewing sarcoma
  63. 63. – 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
  64. 64. 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
  65. 65. Periosteal Reactions Solid onion-peelSunburst Codman’s triangle Less malignant More malignant
  66. 66. Radiographic Features in a Benign vs. Malignant
  67. 67. Clues by density of lesions Sclerotic Cortical Lesions • Osteoid osteoma • Brodie’s abscess
  68. 68. • Osteoid osteoma • Brodie’s abscess Sclerotic Cortical Lesions
  69. 69. Mnemonic for Luscent bone lesions = FOGMACHINES Fibrous Dysplasia Osteoblastoma Giant Cell Tumour Metastasis/ Myeloma Aneurysmal Bone Cyst Chondroblastoma/ Chondromyxoid Fibroma Hyperparathyroidism (brown tumour)/ Haemangioma Infection Non-ossifying Fibroma Eosinophilic Granuloma/ Enchondroma Simple Bone Cyst
  70. 70. • Metastatic lesions – Lung – Renal – Thyroid • Multiple myeloma • Primary bone tumor Lytic Lesions in Adults
  71. 71. • Metastatic lesions – Lung – Renal – Thyroid • Multiple myeloma • Primary bone tumor Chondrosarcoma Lytic Lesions in Adults
  72. 72. • Lymphoma Blastic Lesions in Children
  73. 73. • Metastatic disease • Breast –female • Prostate –male • Lymphoma • Paget’s disease • Fluorosis Prostatic Ca. Blastic Lesions in Adults
  74. 74. • Metastatic disease • Breast –female • Prostate –male • Lymphoma • Paget’s disease • Fluorosis Blastic Lesions in Adults
  75. 75. • Metastatic disease • Breast –female • Prostate –male • Lymphoma • Paget’s disease • Fluorosis Blastic Lesions in Adults
  76. 76. Sled runner tracks in Olliers disease Mafuccis syndrome – olliers + multiple angiomas
  77. 77. Jail house pattern seen in Haemangioma on X Rays thickened, vertically oriented trabeculae Polka dot pattern is seen in cross sections of CT scan.
  78. 78. • 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 • To differentiate solid and cystic lesions. • Most sensitive investigation to detect Pulmonary mets. CT scan:-
  79. 79. • 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. • To differentiate 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.
  80. 80. • 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:-
  81. 81. • 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.
  82. 82. • It uses radioactive glucose to locate cancer by observing high glycolysis rates in a malignant tissue metabolism. • This glucose contains a radioactive atom that is absorbed by the cancerous cells and then detected by a special receptacle. • 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
  83. 83. 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 :
  84. 84. 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.
  85. 85. • 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 :
  86. 86. 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.
  87. 87. • 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.
  88. 88. • 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:
  89. 89.  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
  90. 90. – 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.
  91. 91.  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.
  92. 92. • 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%.
  93. 93. Examples of poorly performed biopsies Biopsy resulted in irregular defect in bone, which led to pathological fracture
  94. 94. Examples of poorly performed biopsies Transverse incisions should not be used Needle biopsy track contaminated patellar tendon Multiple needle tracks contaminate quadriceps tendon
  95. 95. 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
  96. 96. • 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.
  97. 97. Criteria for prophylactic fixation of metastatic tumours of long bones • Surgical Fixation should not be proceeded until primary neoplasm of bone has been ruled out with biopsy. • Goals of fixation – maximize ability for immediate mobilization and weight- bearing – protect the entire bone in setting of systemic or metastatic disease • Type of fixation depends on location of lesion and type of disease – eg. femur • cephalomedullary nailing for peritrochanteric lesions • hemiarthroplasty for femoral neck and head lesions
  98. 98. Harington's criteria of prophylactic fixation • > 50% destruction of diaphyseal cortices • > 50-75% destruction of metaphysis (> 2.5 cm) • Permeative destruction of the subtrochanteric femoral region • Persistent pain following irradiation
  99. 99. Mirel`s criteria of fixation
  100. 100. General principles &Technique for En Bloc Tumour excision Tourniquet – For fear of embolic expression, exsanguinations of the extremity is not adviced, instead the limb is raised for 10 minutes prior to applying the tourniquet.
  101. 101. Advantages of Tourniquet • Prevention of tumour embolisation • Ease of distinguishing neoplastic tissue from normal tissue • Hemostasis of neovasculature • Quicker surgery • More precise resection • No enormous blood transfusion
  102. 102. Incision • A long vertical incision well above and below the visible limits of the tumour. • Incision should include the biopsy scar and drain exit tract in an elliptical fashion. • The plane should be developed through the normal tissues keeping a certain amount of normal tissue surrounding the tumour. • The main vessel and nerve involvement in tumour mass should be judged and cleared well away from the tumour. If their seperation causes one to enter the reactive zone, this does not become an ideal resection
  103. 103. Wound contamination • Incidence of recurrence is approximately doubled in case of a wound contamination due to the malignant tissue. • If contaminated the two options for a surgeon are : – To give up the procedure and consider amputation and – To meticuously wash the wound to the same fashion as is done for a compound fracture and then proceed with a planned resection.
  104. 104. • 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
  105. 105. • 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. 1) Intralesional Resection :
  106. 106. • As musculoskeletal tumors grow, they compress the surrounding tissues and appear to become encapsulated. • This surrounding reactive tissue often is referred to as the pseudocapsule. • Intralesional resection is through the psuedocapsule of the tumor directly in to the lesion. Macroscopic tumour is left behind. Curettage is intralesional proceedure.
  107. 107. 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.
  108. 108. Curettage • 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.
  109. 109. • 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 :
  110. 110. • Despite an increased risk of local recurrence, a marginal resection may be preferable if the alternative is a more mutilating procedure. • Improvements in preoperative radiation therapy and neoadjuvant chemotherapy have made marginal resections an acceptable alternative to amputation in some selective circumstances. • In Marginal resection, dissection passes through the pseudo capsule & reactive zone. Entire structure of origin of tumour is not removed. A margin of atleast 5-7 cm above and below limit of increased bone activity of bone scan is removed.
  111. 111. • 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 :
  112. 112. 4] Radical :- Extracompatmental: 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.
  113. 113. For bone tumors, this involves removing the entire bone and the compartments of any involved muscles.  Radical operations were previously the procedures of choice for most high-grade neoplasms; However, with improvements in imaging studies, radical procedures now are rarely performed because equivalent oncological results usually can be obtained with wide margins .
  114. 114. 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
  115. 115. Enneking`s Classification of Resection of tumours.
  116. 116. 1] Limb Salvage Proceedures 2] Amputations SURGICAL OPTIONS:
  117. 117. • 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
  118. 118. Advantages of limb salvage proceedures : • long term survival rates of patients have improved from approximately 20% to 70%. • The function of the salvaged limb is better than that of the amputation but not normal function. Limb salvage procedures vs Amputation
  119. 119. Disadvantages of limb salvage proceedures:  Limb salvage is more extensive procedure with greater risk of infection, wound dehiscence, flap necrosis, blood loss & DVT.  More chances to undergo multiple future operations for the treatment of complications.  After initial salvage upto 33% of long term survivors may ultimately require an amputation. Limb salvage procedures vs Amputation
  120. 120. Guidelines:  No major neurovascular tumour involvement.  Wide resection of the affected bone with normal muscle cuff in all directions.  Enbloc removal of all previous biopsy sites & all potentially contaminated tissues.  Resection of bone 3-4 cms beyond abnormal uptake as determined by CT /MRI /BONE SCAN.  Resection of the adjacent joint & capsule  Adequate motor reconstruction &soft tissue coverage. Limb salvage procedures
  121. 121. 1. Major neurovascular involvement. 2. Pathological fractures spread tumour cells through fracture hematoma increasing risk of recurrence. 3. Inappropriate biopsy sites contaminate normal tissue planes and jeopardizes local tumour control. 4. Infection flares with metallic implants and thereby jeopardises effect of chemotherapy.. 5. Skeletal immaturity : Predicted limb length discrepency should not be >6-8cm. In such cases expandable prosthesis be used. Upper limb reconstruction is independent of skeletal maturity. 6. Extensive muscle involvement. Contraindications: Limb salvage procedures
  122. 122. 1. Resection of the tumour To avoid local recurrence. 2. Skeletal reconstruction Technique of reconstruction is independent of resection. 3. Soft tissue & muscle transfers. To cover and close resection site and to restore motor power. Distal tissue transfers not used for possibility of contamination. Stages of Limb salvage procedures
  123. 123. • Reconstruction of bone defect may be done by 1] Osteoarticular allograft reconstruction 2] Allograft-prosthesis composite reconstruction 3] Endoprosthetic reconstruction. 4] Allograft arthrodesis 5} Rotationplasty 6} Turnoplasty Surgical reconstructive options :
  124. 124. • Possibility to replace ligaments, tendons & intraarticular structures • Osteoarticular allografts may have a role as a temporary measure to preserve an adjacent physis in an immature patient, when the alternatives include amputation or sacrifice of both physes. • A proximal tibial osteoarticular allograft could be used in an immature patient in an attempt to preserve the distal femoral physis until skeletal maturity. • This could be converted later to an endoprosthetic reconstruction when it becomes necessary • Complications :-non-union at graft –host junction, fatigue fracture ,articular collapse, degenerative joint disease 1. Osteoarticular allograft reconstruction:
  125. 125. 2.Allograft-prosthesis composite reconstruction: Main indication for an allograft- prosthesis composite is an inadequate length of remaining host bone to secure the stem of an endoprosthesis. Tumor prosthesis is used for reconstruction with allograft for fixation to the remaining host bone. Case scenario : osteosarcoma of `proximal humerus. X ray shows tumor extending down to distal diaphysis.
  126. 126. Intraoperative photograph after wide resection of tumour. Humeral allograft is prepared to accept stem of tumor prosthesis Allograft is fixed to bone with medial and lateral plates.
  127. 127. Prosthesis is cemented into allograft. Postoperative radiograph.
  128. 128. • It also provide long-term function for some patients. • Endoprosthetic reconstruction provides the advantage of predictable immediate stability that allows for quicker rehabilitation with immediate full weight bearing. • Most endoprostheses are modular, allowing for incremental limb lengthening as an immature patient grows. 3.Endoprosthetic Reconstruction:
  129. 129. • Polyethylene wear is still a limiting factor for articulating surfaces, but the inserts are easily replaceable in most prostheses. • Fatigue fracture can occur at the rotating hinge. • Fatigue fracture at the base of the intramedullary stem where it attaches to the body of the prosthesis is more problematic. In this location, extraction of the remaining stem can be extremely difficult.
  130. 130. 4. Resection Arthrodesis – Ennekings shirney
  131. 131. 5. ROTATIONPLASTY :
  132. 132. Winkelmann classified ROTATIONPLASTY into five groups, as follows: 1. Group AI – Lesion in distal femur. – The distal femur, knee joint, and proximal tibia are resected; the lower leg is rotated 180 degrees; and the tibia is joined to the remaining femur. Rotationplasty :
  133. 133. • The distalmost femur, knee joint, and proximal tibia are resected. After rotation of 180 degrees, the distal tibia is joined to the distal femur. 2. Group AII—Lesion in the proximal tibia.
  134. 134. • The upper femur and hip joint are resected, and the leg is rotated 180 degrees. The distal femur is joined to the pelvis so that the knee functions as the hip, and the ankle functions as the knee. 3. Group BI—Lesion in the proximal femur sparing the hip joint and gluteal muscles
  135. 135. • The upper femur, hip joint, and lower hemipelvis are resected, and the leg is rotated 180 degrees. The remaining femur is joined to the remnant of the ilium so that the knee functions as a hinged hip joint and the ankle functions as the knee. 4. Group BII—Lesion in the proximal femur with involvement of hip joint and contiguous soft tissue
  136. 136. • The entire femur is resected. • The tibia is attached to the pelvis using an endoprosthesis. 5. Group BIII—Lesion in the mid Femur
  137. 137. • "TuRN-up PLAsTy" of the tibia refers to the replacement of a femur by substituting the iniverted tibia and fibula. This procedure has been utilized to avoid total ablation of a lower extremity and to provide a thigh stump for a prosthesis. • A metallic hip prosthesis is used at turned up end of the tibia. • The first description of the operation was by Sauerbruch in 1922. His first case was a girl of 13 years with an ununited fracture of the femur with chronic osteomyelitis and considerable loss of substance of the shaft in which he did a resection of the femur distal to the trochanters and attached the distal tibia to it. 6. Turn-up-plasty *** : *** Anals of Orthopaedic Surgery, Total Resection of Femur with "Turn-up Plasty" of Tibia and Prosthetic Replacement of Hip Joint; HENRY S. WIEDER, JR., M.D., JESSE T
  138. 138. Two types of prosthesis are used: • 1. modular • 2. custom made 7. PROSTHETIC ARTHROPLASTY
  139. 139. Custom made prosthesis to accomodate large proximal femoral defects after resection
  140. 140. • A tailor- made metallic prosthesis for a particular patient with specific measurements is called custom prosthesis. • Designed using 3D computer modeling & CAD – CAM technologies.( Computer Assisted Designs and computer assisted manufacturing ) Custom mega prosthesis
  141. 141. • For pediatric patients, future limb-length inequality must be considered. • For patients who are near skeletal maturity, the reconstructed limb can be lengthened 1 cm at the initial procedure. • Also, epiphysiodesis of the contralateral limb can be done at the appropriate age to preserve limb-length equality (or to minimize inequality). • For younger patients, however, other options should be considered. • Although amputation and rotationplasty were previously considered the only reasonable treatments for very young patients with bone sarcomas, use of expandable prostheses currently is gaining support. Paediatric Consideration:
  142. 142. • The surgical technique, postoperative course, rehabilitation, function, and complications for implantation of this device is similar to that of other endoprostheses . • The device is unique, however, in that it uses energy stored in a compressed spring to allow for future expansion of the prosthesis as the child grows. Repiphysis Expandable Prosthesis
  143. 143. Electromagnetic expansion of Repiphysis expandable prosthesis.
  144. 144. • 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 :
  145. 145. Ennekings Classification of Amputations
  146. 146. • Levels of Above knee amputation :
  147. 147. • Adjuvant chemotherapy: – To treat 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
  148. 148. • Here the chemotherapy is intituted after the primary tumour has been controlled by alternative treatment such as surgery or radiotherapy. • The rationale is the microscopic metastatic disease can be eradicated. • Majority of the regimen used is High Dose Methotrexate. Adjuvant chemotherapy
  149. 149. • Chemotherapy that is given before local resection is considered neoadjuvant chemotherapy. • The most common reason for neoadjuvant therapy is to reduce the size of the tumor so as to facilitate more effective surgery. Neoadjuvant therapy
  150. 150. Neoadjuvant chemotherapy Advantages Disadvantages • Early institution of systemic therapy against micrometastases • Less chance of drug resistant clones • Reduces tumour size sparing limb salvage chances • Less chance of spread of viable tumour during surgery • Delays definitive control of bulk disease and chances for systemic dissemination. • Risk of local tumour progression with loss of limb sparing option • Psychological effect of retaining tumour
  151. 151. Adjuvant chemotherapy Advantages Disadvantages • Removal of bulk tumour decreases tumour burden and increases growth rate of residual disease making s-phase specific agents more active. • Decreased probability of selecting drug resistant clone in primary tumour. • Dealy of systemic therapy for micrometastases. • Possible spread of tumour by surgical manipulation. • No preoperative in vivo assay of cytotoxic response.
  152. 152. GRADE EFFECT I Little or No response of tumour identified II Areas of necrosis and tumour cells seen. III Scattered foci of tumour cells seen. IV No Tumour cells seen. HUVO`s Histologic grading of effect of preoperative chemotherapy on primary Bone tumour
  153. 153. GRADE EFFECT I No viable tumour cells II Single tumour cells or clusters <0.5cm III Viable tumour cells <10% IV Viable tumour cells 10-50% V Viable tumour cells >50% VI No response Salzer-Kuntschik `s Histologic grading of effect of preoperative chemotherapy on primary Bone tumour
  154. 154. • 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
  155. 155. Diagnosis Neoadjuvant chemotherapy + Radiation Resection/ surgery Repeat Chemo + Radiation Adjuvant + irradiation +chemo Histological Grading Sequence of treatment:
  156. 156. 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
  157. 157. Internal or Sealed source chemotherapy. • Brachy means close/short distance. • Source is placed near to the target tissue through multiple catheters placed over tumour bed. • High dose can be delivered to the target tissue..with minimal side effects. Brachy therapy:
  158. 158. • A portion of tumour is implanted in to a sarcoma survivor and removed after 14 days. • Sensitized lymphocytes from survivors are infused in to patient. These cells selectively kill the cancer cells. Immunotherapy
  159. 159. Extracorporeal Irradiation • En-bloc resection, extracorporeal irradiation, and re-implantation is done in limb salvage for bony malignancies. • After en-bloc resection, the segment is wrapped in a wet sterile drape to minimise air gaps, placed in two sealed sterile plastic bags and wrapped in another sterile drape. • This is delivered for radiotherapy.
  160. 160. Extracorporeal Irradiation • Meanwhile, the operative site is prepared for re- implantation and marginal biopsies taken. • On return the specimen is removed from the inner plastic bag and soaked in iodine solution. • It is cleared of unnecessary soft tissue leaving important muscle insertions for re-attachment and then re-implanted
  161. 161. Cementoplasty • Patients with osteolytic tumours (metastasis, multiple myeloma, lymphoma) located to the vertebral body, acetabulum and condyles and causing local pain, disability and with a risk of compression fracture are excellent indications. • Used in cases of palliative care to stabilise the lytic lesion. • For vertebroplasty, a 10-G and 15-G beveled needle (for thoracic/lumbar and cervical level, respectively) should be used. • Cement injection should be done under real-time imaging.
  162. 162. LASER ABLATION for Benign Tumours • Because of the small size of the ablation zone produced, laser is mostly used for small tumours or in case of Radio frequency contraindications (metallic implants). • Osteoid osteomas are the best indication. • The laser fibre (400 to 600 µm) is inserted always coaxially into the tumour under CT-guidance through a spinal needle and a laser beam directed to ablate the tumour.
  163. 163. Direct intra arterial chemotherapy • Usually in chemotherapy is given intra venously, but direct intra arterial administration there by localising the drug more pertaining to the affected tumour gives better results.
  164. 164. ISOLATED LIMB PERFUSION METHOD Effective way of delivering chemotherapy to the affected limb alone reducing systemic side effects. With a steinmann pin over iliac bone to anchor Eschmark torniquet in order to occlude proximal blood vessels. Cannulation of vessels and delivering the drug by ensuing warmth and perfusion of the limb.
  165. 165. Oncologist BoneTumours Diagnosis Treatment Radiologist Cytopathologist Surgeon Histopathologist Molecular Pathologist Geneticist psychiatrist Nursing And Support staff Audit MUSCULOSKELETAL TUMOURS are Evolving in to a multi disciplinary approach
  166. 166. References : • Campbell’s Operative Orthopaedics – 11th & 12th edition • Samuel Turek Text Book of Orthopaedics: 2nd edition • Bone and Cancer: Felix Bronner • Adam Greenspan- Differential diagnosis in orthopaedic oncology, 2nd edition • Mercers Orthopaedic Surgery Vol II 9th edition and 11th edition • WHO Manual 2001 reprint for Classification of Musculoskeletal Tumours • Wheeless`Textbook of Orthopaedics. • www.uptodate.com/musculoskeletal tumours • Anals of Orthopaedic Surgery, Total Resection of Femur with "Turn-up Plasty" of Tibia and Prosthetic Replacement of Hip Joint; HENRY S. WIEDER, JR., M.D., JESSE T • Musculoskeletal cancer surgeries – treatment of sarcomas and allied diseases – Martin M. Malawer • American Academy of Orthopaedic Surgeons – Text book of Orthopaedic knowledge update 8 series. THANK YOU
  167. 167. Buzzwords: in Bone Tumours • Ground glass fibrous dysplasia • salt & pepper appearance Hyperparathyroidism,Paraganglionoma • punched-out lesion eosinophilic granuloma, multiple myeloma • soap bubble Giant cell tumour • Popcorn balls cartilage tumours • Sled runner tracks Ollier's & Mafucci's • Sunburst spiculation osteosarcoma • Pseudo Rosette Ewings sarcoma • Chicken wire calcifications Ewings sarcoma, Chondroblastoma • Onion Peel appearance Ewings sarcoma, Gouchers disease • Codmans Triangle Osteosarcoma, Ewings sarcoma, Chronic osteomyelitis • Patchy / Mottled calcification Chondrosarcoma • Polka Dot Pattern CT finding in Haemangioma ( Cross section, trabeculae) • Jail House appearance X ray finding in a haemangioma (thickened, vertically oriented trabeculae • Double density Sign Osteioid Osteoma – Bone scan feature

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