LONG BONE TUMOURS ORTHOPAEDICS ,PPTX....

BONE TUMOURS
Dr. Bipul Borthakur
Professor And Head
Department of orthopaedics
Assam Medical College
Dibrugarh, Assam

INTRODUCTION
• Comprise a spectrum of musculo-skeletal disorder ranging from
latent, benign lesion to destructive, malignant neoplasm.
• Mostly classified according to the tissue type they arise from.
• Diverse in their gross & morphologic feature.
• Critical to diagnose correctly, stage accurately & treat
appropriately.
• Management requires a team effort comprising of orthopedic
surgeon, radiologist, pathologist, medical & radiation oncologist and
plastic surgeon.

Approach to Musculo-skeletal neoplasm
•Adequate history & physical examination are first
step. HISTORY-
Age
Sex ( GCT- F>M)
Family history ( multiple hereditary exostosis –AD, NF-
AD)
Pain - most common.
- deep seated, dull-aching
-initially activity related, later progressive pain at
rest & at night.
- NSAIDs/ strong analgesics- during flare

- sudden increase in pain ( malignancy/ hg’e/ pathological #)
- radicular pain ( impingement of neurovascular structure)
Swelling- better examined than explained.
- duration/progression/presence of pain & tenderness
• Pain usually precedes swelling by months but pain appearing with
swelling indicates pathological # in an often benign lesion.
Functio-lesia/ loss of function - uncommon presentation.

PHYSICAL EXAMINATION
• Evaluation of patient’s general health
• Mass- location/shape/consistency/mobility/tenderness/
local temp/extent
• Overlying skin changes (eg. hyperpigmented patches in
FD)
• Shiny adherent skin tethered to the underlying mass with
dilated supf. Veins suggests aggressive pathology.
• Atrophy of surrounding musculature (neurodeficit /?adequacy
of circulation)
• Draining lymph nodes.

RADIOLOGICAL EVALUATION
X-ray-
• Always the first investigation to order.
• Often , patient’s age & xray findings sufficient to arrive at a diagnosis.

Lesion margin
• Evaluating margin helps us to identify what tumour does to the bone.
• Reveals the ‘nature’ of the lesion in terms of ‘aggressiveness’.
• Slow growing lesion- relatively sharp, sclerotic, well-defined margin.
• Aggressive lesion- ill-defined margin.
• Lodwick classified radiographic lytic lesions into 3 categories-
Grade 1- “geographic” destruction + sharp/well-defined margin/
narrow zone of transition.
Grade 2- “moth-eaten” destruction.
Grade 3- “permeative” destruction.

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Cortical destruction
• High grade aggressive lesion - penetrates through cortex,
periosteum & its reactions, leaving nothing
behind.
• Intermediate grade lesion - develops focal cortical defect
resulting from ‘spillage’ of tumour cells.
• Low grade lesion - ‘expand’ the cortex from within with new
bone formation in an attempt to remodelling.
* Lesions can have various pattern of cortical destruction, in such cases,
highest grade of cortical destruction represents the “nature” of the
lesion.

Matrix
• Represents proliferative characteristics of bone
tumours-
 Chondroid matrix- appears
dot-like/popcorn- like/punctate/ stippled
calcification.
eg. Enchondroma, chondroblastoma,
chondrosarcoma.
 Osteoid matrix- cloud-like/wispy appearance
eg. Osteoid osteoma, osteosarcoma.
 Dystrophic matrix- degenerative fibrous
+ myxoid tissue
eg. Intraosseous lipoma, bone infarcts.

Bone reactions
• Explains what bone does to the tumour.
• Medullary or periosteal.
• Very aggressive lesion- has no medullary
reaction as they ‘eat up’ everything.
• Reactive shell ( thin/thick) around lesion in
medullary cavity-slow growing lesion.
• Periosteal reaction occurs as the lesion
approaches cortex stimulating the periosteum
to thicken & become cellular.
• Periosteal reaction- solid vs. interrupted

Other imaging examinations
CT SCAN-
• Most helpful in assessing ossification, calcification
& integrity of cortex.
• Best imaging study to localize the nidus of osteoid
osteoma, to detect the thin rim of reactive bone
in ABC.
• 3D reconstructions- surgical planning.
• CECT- differentiates cystic from vascular lesion in
soft tissue tumours.
• CT lung- to detect metastasis.

MRI-
• To determine size, extent, anatomic relationship
of bone & soft tissue tumours.
• To identify the extent of intramedullary &
extraosseous disease and relationship with
neurovascular structure.
• To plan “safe” surgical margins ( 1-2 cm for soft tissue ,
1.5-2 cm for bone beyond MRI demonstrated limit).
• Signal intensity is compared to adjacent soft tissues.
• CEMRI- evaluates relationship of tumour with adjacent
blood vessel & characterizes cystic lesion.

• Excellent modality for screening the
spine for occult metastasis,
myeloma, lymphoma.
• Tumour viability (T2) & response to
chemotherapy can be judged.
• Most MISUSED investigation for
diagnosis of benign bone
pathology.

TECHNETIUM BONE SCAN-
• Classically used to detect bony metastasis.
• Malignant neoplasm- increased uptake on bone scan.
• To determine metabolically active intramedullary extent
of tumour.
• To determine polyostotic nature of disease & 2◦
involvement of bone by 1◦ soft tissue pathology like
infection, neoplasia.
• Also used to assess response to chemotherapy.

ULTRASOUND - differentiates cystic from solid lesion.
ANGIOGRAPHY –
• Useful for large tumours that likely to
displace neurovascular bundles.
• Describes vascular supply of a tumour by
feeding vessel/ alternative vascularity.
• Planning of pre-operative embolization for highly
vascular tumours s( RCC, ABC)
• To rule out non-neoplastic conditions like
pseudoaneurysm, AV malformations.

POSITRON EMISSION TOMOGRAPHY –
• Being increasingly used to “stage”
the tumours, “plan” biopsy &
“assess” response to
chemotherapy.
• FDG labelled PET – in detection, staging &
management of sarcoma.
( FDG- analog of glucose that is trapped
in malignant cells in proportion to the
respective rate of glycolysis)

Laboratory tests
• Basic metabolic panel to evaluate overall health.
• CBC ( to r/o infection/anaemia)
• ESR ( infection, malignancy, small blue cell tumours)
• Serum protein electrophoresis ( MM)
• Serum calcium ( metastatic ds, MM, hyperparathyroidism)
• PSA ( prostate CA)
• ALP ( metabolic bone ds, metastasis, osteosarcoma, ewing sarcoma)

Biopsy
• Done only after clinical, laboratory & radiologic examinations are complete.
• Planned as carefully as the definitive procedure.
PRINCIPLES OF TAKING BIOPSY
1. Biopsy track should be removed en bloc with the tumour.
2. Should be performed by the surgeon who will carry out the definitive surgery.
3. If tourniquet used, limb is elevated before inflation but should not be
exsanguinated ( to prevent ‘squeezing’)
4. Transverse incision avoided ( difficult to excise)
5. Intracompartmental, shouldn’t be intercompartmental.
6. Major neurovascular structures should be avoided.

7. Soft tissue extension of a tumour should be sampled ( leading
edge contains most viable tumour cells).
8. If a hole is made in the bone - round/oval - to minimize stress
concentration & prevent pathological #. Hole should be
plugged with bone wax/ methacrylate.
9. Frozen section should be sent to pathology intra-operatively.
10. If tourniquet is used - should be deflated & hemostasis ensured
before closure.
11. If drain is used - should exit in line with the skin incision.
12. Wound should be closed tightly in layers.
13. Wide retention sutures should not be used.

STAGING
• Benign & malignant tumours of bone & soft tissues are staged according
to ENNEKING staging system.
STAGING OF BENIGN TUMOURS
STAGE 1-
LATENT-
• Intracapsular,
usually
asymptomatic,
incidental
finding.
• Well-defined
margin with

STAGE 2- ACTIVE-
• Also intracapsular but actively growing.
• Causes symptoms, leads to pathological #.
• Well-defined margin but may expand- thinning of cortex.
• T/t- extended curettage.
STAGE 3- AGGRESSIVE-
• Extracapsular.
• Do not respect natural anatomical barrier.
• Breaks through the reactive bone & through cortex.
• Metastasis may present in 1-5% cases.
• T/t- extended curettage/marginal resection/wide excision.
• Local recurrences are common.

STAGING OF MALIGNANT TUMOURS
• Based on histologic grade, local extent,
presence/absence of metastasis.
• Helps to guide surgical & adjuvant
treatment.
Compartment- natural anatomical barriers to tumour growth
like cortical bone, articular cartilage, fascial septa, joint
capsule.

TREATMENT
• Primary goal- to make the patient disease-free.
• It combines-
 Radiotherapy
Chemotherapy
Surgery - amputation vs. limb salvage
- margins
- curettage
- resection & reconstruction

RADIOTHERAPY
• Intracellular free radicals → DNA damage → cell death.
• Goal- to deliver the highest possible dose of radiation
to tumour cells while minimizing toxicity to normal
tissues.
• External beam radiotherapy vs. brachytherapy.
• Most radiation protocols- 150-200 cGy/day until target
dose achieved.
• Used for pre-operative treatment of soft tissue sarcoma
( reducing tumour volume/make resection easier).
• Reduces incidence of local recurrences of
malignant soft tissue tumours.
• Most primary bone malignancies- radioresistant
( exception- small blue cell tumours)

COMPICATIONS - ACUTE &
LATE
ACUTE
• MC- skin irritation ( initial
erythema →
desquamation)
• Gi upset
• Urinary frequency
• Fatigue
• Anorexia
• Extremity edema.
LATE
• Chronic edema
• Fibrosis
• Osteonecrosis
• Pathological #
• Radiation sarcoma ( in survivors
of childhood & adolescent
cancers)

CHEMOTHERAPY
• Well-defined role in the t/t of high grade malignancies (osteosarcoma/ ewing’s
/MFH/ rhabdomyosarcoma).
• Not useful for cartilaginous lesion & most low grade malignancies.
• Adjuvant vs. Neoadjuvant.
• Advantages of neoadjuvant chemotherapy –
Regression of 1◦ tumour, making surgery easier.
Decreases the spread of tumour cells.
Can effectively treat micrometastasis at earliest.
Allows time to plan surgery.
• Currently, most orthopaedic oncologists prefer neoadjuvant chemotherapy with
definitive surgery performed 3-4 weeks after the last dose , again restarted after
2 weeks postoperatively.

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PRINCIPLES OF SURGERY
AMPUTATION VS. LIMB SALVAGE
• Advances in diagnostic imaging, radiotherapy, chemotherapy,
surgical techniques of resection & reconstruction make limb
salvage easier.
• Attainment of ‘safe’ surgical margin regardless of whether
achieved by amputation or wide resection - important.
• Local recurrence- extremely poor prognosis.
• AMPUTATION
 technically demanding, requires flaps for closure to
obtain more functional residual limb.
 Complications- infection, wound dehiscence,
phantom limb, appositional bone growth requiring
revision sx.

LIMB SALVAGE –
 Requires more extensive surgical procedure.
 Associated with greater perioperative & long term morbidity.
 Early Complications - infection, wound dehiscence, flap necrosis,
blood loss, DVT
Long term complication - periprosthetic #
- prosthesis loosening/dislocation
- non-union of graft-host junction
- limb length discrepancy
 A patient with salvaged limb - more likely to need multiple
future operations for treatment of complications.
 1/3rd of long term survivors ultimately require amputation.

SURGICAL MARGIN
•Intralesional/ marginal/ wide/
radical. INTRALESIONAL –
plane of surgical dissection is within
the tumour.
‘Debulking’ as it leaves behind the gross
residual tumour.
Suitable for symptomatic benign lesion when
the only surgical alternative to sacrifice
important anatomic structure.
Appropriate as a palliative procedure in
metastatic ds.

MARGINAL –
 Plane of surgical dissection
passes through ‘pseudocapsule’.
 Usually adequate for most benign lesions &
low grade malignancies.
 Often leaves behind microscopic foci
of disease leading to local recurrence if
not addressed by adjuvant
chemotherapy/radiotherapy.
 Advent of preoperative radiotherapy &
neoadjuvant chemotherapy make marginal
resection an acceptable alternative to amputations.

WIDE –
Plane of dissection is in normal tissue.
‘Quality’ of margin is more important than quantity.
Goal for most high grade malignancies.
Sometimes, impossible to
achieve. RADICAL –
 Achieved when all the compartments containing tumour are
removed en bloc.
 Was surgery of choice previously for high grade neoplasms but
rarely performed nowadays bcz equal oncologic result obtained with
wide margins.

CURETTAGE
• Most benign tumours are treated adequately
with curettage.
•High rate of local recurrence as compared to
resection. SIMPLE CURETTAGE –
 large cortical window ( as large as the tumour)
Bulk of the lesion is ‘scooped out’ with large curettes.
Cavity is enlarged upto the normal host bone in
each directions with power burr.
The cavity & wound is copiously irrigated to remove
debris & tumour cells.

EXTENDED CURETTAGE –
 Includes the use of adjuvants eg. Liquid nitrogen,
phenol, methacrylate, thermal cautery to extend
the destruction of tumour cells.
Greatly reduced recurrence rate of aggressive
tumours.
Filling the bone cavity after curettage –
• Autologous bone graft/ allograft/ artificial bone graft
substitutes/bone cement.
• Harvested using a different set of instruments.
• Any recurrence- “expanding lucency” adjacent to
bone cement.

RESECTION & RECONSTRUCTION
• Currently, most musculoskeletal neoplasms are treated with resection &
reconstruction.
• Goal of resection- to achieve “wide” surgical margin.
• If wide margin is not possible due to anatomic constraint, ‘marginal’
resection
combined with adjuvant/neoadjuvant treatment is preferred over
amputations.
RECONSTRUCTION –
 Involves preserving a mobile joint.
 3 options – osteoarticular allograft reconstruction
- endoprosthetic reconstruction (MC)
- allograft-prosthesis composite reconstruction
 Oncologic reconstructions are always associated with higher complication rate
( infection, wound necrosis).

Osteoarticular allograft
• Advantages like ability to replace ligaments,
tendons, intra-articular structures.
• Complications- non-union at graft-host
junction, fatigue fracture, infection, articular
collapse, degenerative joint disease.
Allograft-prosthesis composites
• Provide a long term solution for
most patients.
• Indication- inadequate length of host bone
to secure the stem of endoprosthesis.
• Avoid complication of degenerative joint ds &
articular collapse.
• Complications- infection, fatigue fracture,
non-union at graft-host junction.

Endoprosthetic reconstruction –
• Provides immediate stability , allows quick
rehabilitation, immediate full weight bearing.
• ‘modular’- allows incremental limb
lengthening.
• Provides long term functions for some
patients.
• Complications- polyethylene wear at the
articulating surfaces, fatigue fracture at rotating
hinge/ at the base of intramedullary stem.

Classsification of bone tumours

OSTEOID OSTEOMA
• AGE- 2nd
-3rd
decade.
• DEMOGRAPHICS- M:F= 3:1
• SITE- femur, tibia, posterior elements of spine.
- diaphyseal/metaphyseal.
• PRESENTATION- pain worses at night ; frequently relieved by NSAIDs.
( tumour contains cyclooxygenase & prostaglandins)
• IMAGING- XRAY- small(<1.5 cm),central radiolucent nidus surrounded
by bony sclerosis.
-CT SCAN- best technique to identify nidus & confirm
diagnosis.
-MRI- demonstrates extensive surrounding edema.
-Tc Bone scan- increased uptake.
• HISTOLOGY- trabeculae surrounded by loose fibrovascular tissue.
• TREATMENT- NSAIDs
- radiofrequency ablation
- curettage ( burr down technique)
- en bloc resection
- MRgFUS ablation
• No malignant change documented.

CHONDROMA
• Benign lesion of hyaline cartilage.
• Enchondroma vs. periosteal/juxtacortical chondroma.
• AGE- adults
• DEMOGRAPHICS- M=F
• SITE- phalanges of hand (MC) & foot, proximal humerus, distal femur
• PRESENTATION- usually asymptomatic, almost incidental finding.
• IMAGING- XRAY- benign appearing lesion with irregular calcification
(stippled/punctate/popcorn)
- erosion & expansion of overlying cortex in small
bones
CT SCAN- to evaluate endosteal erosion
• HISTOLOGY- hyaline cartilage
- any degree of hypercellularity/atypia- ?
chondrosarcoma
• TREATMENT- observation with serial radiographs ( asymptomatic)
- extended curettage (if symptomatic)

OLLIER’S DISEASE
• Multiple enchondromatosis.
• Many cartilaginous tumours appear in the
tubular bones & flat bones.
• Caused by failure of normal
endochondral ossification.
• Epiphysis/metaphysis.
• Deformities- shortening d/t lack of epiphyseal
growth, metaphyseal broadening, bowing of
long bones.
• 25% pre-malignant.
MAFFUCCI SYNDROME
• Multiple enchondroma
• Hemangioma of overlying soft
tissue.
• Phleboliths.
• 100% pre-malignant.

OSTEOCHONDROMA/ EXOSTOSIS
• Developmental malformation rather than true neoplasm.
• Originates within the periosteum as a small cartilaginous nodule.
• Growth parallels with growth of the patient, ceases at maturity.
• AGE- 2nd-3rd decade.
• SEX- M>F
• SITE- metaphysis of long bone (distal femur, proximal tibia).
• TREVOR ds- intra-articular epiphyseal osteochondroma.
• Solitary (90%) vs. multiple hereditary exostosis (AD with incomplete
penetrance).
• PRESENTATION- asymptomatic/ pressure symptoms/pain due to #.
• Pedunculated (MC) vs. sessile.
• XRAY- cartilaginous cap ( few mm to 2 cm) not seen.
• CT/MRI confirms diagnosis.
• TREATMENT- observation ( asymptomatic)/ resection(symptomatic),
cartilage cap should be resected entirely.
• Malignant transformation is rare.

NON OSSIFYING FIBROMA/
FIBROUS CORTICAL DEFECT
• MC benign lesion of bone.
• AGE- 1st-2nd decade.
• SEX- M=F
• SITE- metaphysis of long bones ( distal femur > tibia > fibula)
• PRESENTATION- asymptomatic, usually incidental diagnosis.
• XRAY- well defined lobulated lesion located eccentrically in metaphysis
- multilobulated appearance with sclerotic margin.
• HISTOLOGY- spindle cells arranged in storiform pattern in a
collagenous matrix.
• TREATMENT- observation/ curettage/ pathological # treated non-
operatively.

FIBROUS DYSPLASIA
• Developmental anomaly- bone is replaced by fibrous tissue.
• Monostotic / polyostotic.
• AGE-1st -3rd decade.
• SEX- M=F
• SITE- femur > tibia.
• PRESENTATION- pain f/b deformity.
• XRAY- lucent area having ground glass appearance surrounded
by well defined sclerotic rim ( RING SIGN)
• HISTOLOGY- irregular woven bony spicules with a fibrous
stroma.
• TREATMENT- prophylactic intramedullary fixation of impending
#
- correction of deformity
- bisphosphonates for severe cases.

SIMPLE/ UNICAMERAL BONE
CYST
• Developmental/reactive lesion than a true tumour.
• AGE- 1st
-2nd
decade.
• SEX- M:F= 2:1
• SITE- proximal humerus> proximal femur (children)
- ilium, calcaneum (adults)
• Most active during skeletal growth, heals spontaneously at maturity.
• PRESENTATION- asymptomatic unless a pathological #
• XRAY- centrally located, purely radiolucent lesion
- concentrically expanding cortex
- no cortical destruction
- FALLEN LEAF SIGN (pathognomonic)
• HISTOLOGY- cyst filled with straw coloured fluid/ thin fibrovascular lining.
• TREATMENT- observation with serial x rays
- aspiration & injection ( steroid/ bone marrow aspirate/ bone graft substitutes)
- curettage with bone grafting & internal fixation.

ANEURYSMAL BONE CYST
• Locally destructive, blood-filled reactive lesion of bone.
• AGE- 1st-2nd decade.
• SEX- slight female preponderance.
• SITE- proximal humerus, distal femur, proximal tibia, post. elements of spine.
• PRESENTATION- pain (weeks-months)
• Rapid growth can occur & clinically mimics a malignancy.
• XRAY- eccentric expansile lytic lesion
- thin cortical shell
• MRI- intralesional septa / double density fluid level.
• HISTOLOGY- hemorrhagic cavernous space
- septae of fibroblast, histiocytes, macrophage, giant cells
• TREATMENT- extended curettage with bone grafting.
- pre-operative embolization to reduce blood loss.

BENIGN AGGRESSIVE BONE
LESIONS

GIANT CELL TUMOUR
• Benign but locally aggressive.
• AGE- 20-40 yrs.
• SEX- slight female preponderance.
• SITE- distal femur > proximal tibia > distal radius.
• Epiphyseal in origin, metaphyseal in skeletally immature patients.
• PRESENTATION- progressive pain ( activity related → rest pain)
- severe pain (pathological #)
• Pulmonary metastasis- 3% of patients
• XRAY - eccentrically located in epiphysis
- abuts subchondral bone
- purely lytic lesion with no matrix formation ( SOAP
BUBBLE )
- poorly defined zone of transition
- no rim of reactive bone
- may exhibit cortical destruction with soft tissue extension
- intra-articular extension is rare.

• MRI – to determine extent of lesion in bone & soft tissues.
• HISTOLOGY- multinucleate giant cells in the sea of mononuclear stromal
cells
- nuclei are identical
• TREATMENT - simple curettage ( recurrence rate >50%)
- extended curettage with bone grafting ( commonly done)
- en bloc wide resection ( stage 3 tumours /cases of local
recurrence/ tumours recalcitrant to
other method of t/t)
- bisphosphonates ( i.v zoledronate in unresectable
tumours)
- ? Denosumab ( insufficient data)
- resection of pulmonary metastasis.

OSTEOBLASTOMA
• Rare bone forming neoplasm.
• AGE- 10-30 yrs
• SEX- M:F= 3:1
• SITE- posterior elements of spine > long bones.
• PRESENTATION- pain (worse at night , relieved by NSAIDs)
- painful scoliosis, neurodeficit
• XRAY- bone forming neoplasm in posterior elements of spine
- variable radiographic appearance outside the spine
• HISTOLOGY- fibrovascular stroma with production of osteoid &
primitive woven bone.
• D/d- osteoid osteoma, low grade osteosarcoma.
• TREATMENT- extended curettage / resection with bone grafting
- might require spinal stabilization

CHONDROBLASTOMA
• AGE- 10-25 yrs
• SEX- M:F = 2:1
• SITE- epiphysis of long bones ( distal femur, proximal tibia)
• PRESENTATION- progressive pain
• XRAY - well circumscribed lesion in epiphysis/apophysis
- may cross open physis
- surrounding rim of reactive bone
- matrix calcification ( 30-50%)
• CT- detects subtle calcification
• MRI- demonstrates surrounding edema.
• Soft tissue extension is rare.
• HISTOLOGY - sheets of chondroblasts with a background of chondroid matrix
- “chicken wire “ calcification
- multinucleate giant cells
• TREATMENT- extended curettage with bone grafting.
• Malignant transformation- extremely rare.

OSTEOSARCOMA
• Most common non-hematologic primary bone malignancy.
• Characterized by production of OSTEOID by malignant cells.
• AGE- bimodal
- primary (10-20 yrs) – high grade
- secondary (>50 yrs)- paget’s/previous radiation therapy
• SEX- M>F (except parosteal osteosarcoma)
• ASSOCIATIONS- retinoblastoma
- Rothmund –Thomson syndrome
- Li Fraumeni syndrome
• SITE- areas of rapid growth (distal femur, proximal
tibia, proximal humerus)

• PRESENTATION -- for high grade tumour, ‘progressive pain’ ( initially
improved with conservative measures but later ‘NIGHT PAIN’ )
-- for low grade surface osteosarcoma, ‘painless mass’
• XRAY - aggressive lesion in the metaphysis of a long bone
- mixed lytic & blastic appearance
- cortical destruction
- soft tissue mass
- periosteal rxn ( CODMAN triangle/ SUNBURST appearance)
• MRI - to measure the extent of tumour in bone & soft tissue
- to determine the relationship of tumour with nearby anatomic structure
• BONE SCAN - increased uptake
- to look for skeletal metastasis
• CT – CT chest to look for pulmonary mets (MC)

PRIMARY OSTEOSARCOMA
1. Conventional osteosarcoma (MC)
2. Low grade intramedullary
3. Parosteal
4. Periosteal
5. High grade surface osteosarcoma
6. Telengiectatic
7. Small cell osteosarcoma
SECONDARY OSTEOSARCOMA
• > 50 yrs
• MC location- pelvis, skull, spine,
ribs, scapula
• Latency -10-15 yrs
1. Paget’s ds
2. Post radiation osteosarcoma

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• Distal
femur
* Proximal
tibia
•Proxima
l
humeru
s
Progressiv
e pain
- Mixed lytic and
blastir
appearance
-Cortical
destruction
•Osteoid
production from
malignant
spindle cells
Chemotherap
y and wide
resection
Rarely associated
with hereditary
form of
retinobfastoma,
Rothmund-
Thomson
syndrome, or Li-
Fraumeni
syndrome
- P
e iosteaT reaction •Marked
nuclear
(Codman triangle or
pleamorphism
Periosteal
osteosartam
a
2nd-
3rd
decade
s
Slight
female
predominanc
e
Diaphysis femur -
Pain and tibia -
Mass
hair-on-end) •Abundant
mitotic
- Soft tissue mass
figures
- Didphyseal
lesion
- Lesion siu in
Str
an
ds of osteoid
radiating
among
Wide
resection
Chemotherap
y
controversia
l
depression on sur- lobules of
cartilage
face of bone
- Irregular
ossification
blends into soft
tissue
Parosteal 3rd-4th Slight female Posterior
aspect
Painless mass Lobulated
ossified
• 5lighty atypical Wide
resection
osteosarcoma decade
s
predominance o1 distal
femur
mass on posterior Spindle celI”s aloné
surface of
distal demur
- Purely Iyti‹
Mayhave
bal-
Iooned/‹ysti
‹
• Slightly
atypical
trabeculae
- Blood-filled
spaces
separated by
thin septa
Telangiectati
‹
osteosarcom
a
2nd
decad
e
Slight male
predominanc
e
- DiStal femur
• Proximaltibia
Progressiv
e pain
Chemotherap
y and wide
reseuion
Secondary
osteosarcom
a
6th-
8th
decade
s
Male:female 2:1, ,AXiaIla.cation
Progressiv
e pain
Paget osteosarcoma (sites of
previous radiation
or
Paget
disease)
Male:femat
e 1:2,
radiation
osteosar
¢oma
appearance (similar •5pindte cells
in septa frankly
ma1ignant
Similar to
high•
Chemotherapy Poor
prognosis
grade conventional and side
to aneurysmal
bone cyst)
Mixed lytir and
blastic appearance
- Cortical
destruction
osteosarcama
resection
P
a
g
et oseosarcoma
more common
in patients with
advanced
polyostotic
disease
Radiation
osteosar- noma
usually occurs
IO-15. years after

• TREATMENT
 neoadjuvant chemotherapy → wide /
radical surgery (resection/ amputation) →
adjuvant chemotherapy
 Multiple agent chemotherapy – Methotrexate
+ Adriamycin/Doxorubicin + Cisplatin (MAP)
 Pulmonary metastasis are resected
after neoadjuvant chemotherapy.
Goal of chemotherapy- to treat
micrometastasis.
 5 year survival rate -70% (after proper
treatment)

• PROGNOSTIC FACTORS –
 Presence & location of metastasis at the time
of diagnosis (15%- detectable pulmonary mets
at diagnosis)
 Grade of the lesion ( low grade- rarely
metastasize)
 size of primary tumours
Skeletal location (proximal- worse outcome)

CHONDROSARCOMA
• 2nd MC non-hematologic primary bone malignancy.
• AGE - 40-60 yrs ( 1◦ cs)
- 25-45 yrs ( 2◦ cs)
• Secondary > primary
• SEX- M>F
• SITE- pelvis, proximal femur, proximal humerus.
• PRESENTATION - pain / palpable mass
- slow growing
- pathological #
• XRAY - punctate/ popcorn/ comma shaped
calcification
- cortical erosion/destruction
- periosteal reaction
- soft tissue extension
• CT – shows endosteal erosions

• HISTOLOGY- malignant cells in abundant cartilaginous matrix
- binucleate cells
- entrapment of bony trabeculae
- histological grades acc. to degree of hypercellularity
(dedifferentiated/ clear cell/
mesenchymal)
• TREATMENT- extended curettage ( low grade lesions)
- wide/radical resection/amputation ( high grade
lesions)
- radiotherapy- palliative role in surgically
inaccessible cases

EWING’S SARCOMA
• Third MC non-hematologic primary bone malignancy.
• AGE- 5-25 yrs
• SEX – M>F
• SITE- flat bones , metadiaphysis of long bones.
• PRESENTATION - pain ( insidious/mild/intermittent/responds to analgesic)
- fever
- erythema
- swelling
• LAB STUDIES – increased WBC, ESR, CRP, LDH
• XRAY -“permeative” bone destruction
- large soft tissue mass
- “onion skin’’ periosteal reaction
• MRI - to evaluate full extent of the lesion
- to evaluate the extent of soft tissue
mass
• CT chest ( lung- MC site of metastasis)

• HISTOLOGY - small round blue cells
• TREATMENT- “wide” resection for primary tumour
- adjuvant/neoadjuvant chemotherapy
to treat distant
metastasis
- radiation for large unresectable
tumour
+ to reduce local recurrence after marginal
resection.
• Relapse is always associated with poor prognosis.

ADAMANTINOMA
• Rare neoplasm, <1% of all primary bone malignancy
• AGE- 2nd-3rd decade
• SEX - male=female
• SITE - subcutaneous bones (aberrant nests of epithelial cells)
- tibial diaphysis (85%)
• PRESENTATION - pain/palpable mass/pathological #
• XRAY- multiple, sharply demarcated radiolucent lesion separated by
areas of dense, sclerotic bone
• HISTOLOGY – islands of epithelial cells in fibrous stroma
• TREATMENT- “wide” resection/amputation
• Both radio & chemoresistant
• Metastasis (30%)
• Prognosis depends on adequacy of surgical margin

MULTIPLE MYELOMA
• MC primary bone malignancy.
• AGE – 5th
-7th
decade
• SEX – M:F= 2:1
• SITE – spine > ribs, pelvis
• PRESENTATION - bone pain/pathological #
- systemic symptoms (anemia, wt loss, thrombocytopenia, neuropathy, hypercalcemia, renal failure)
• XRAY – multiple, “punched out”, sharply demarcated, purely lytic lesion without surrounding reactive sclerosis
• SERUM ELECTROPHORESIS- monoclonal gammopathy
• Raised ESR, urine for bence jones protein
• Biopsy to confirm the diagnosis
• HISTOLOGY- plasma cells ( round blue cell with ‘clock face’ nuclei with perinuclear ‘halo’)
• TREATMENT- chemotherapy + radiotherapy + bisphosphonates
- pathological # - internal fixation with bone grafting
• High risk of perioperative complications ( infection, renal failure, DVT)

METASTATIC CARCINOMA
• MC malignancy treated by orthopaedic surgeon.
• Newly discovered, aggressive appearing bone lesion in patients >40 yr
• SITE- spine > proximal femur, proximal humerus
• “Search” for the primary before taking biopsy-
- basic laboratory tests
- serum protein electrophoresis
- PSA
- CT chest, abdomen, pelvis
- bone scan
• CA Breast > prostate > lung >kidney > thyroid > Gi
• XRAY – blastic/lytic/mixed
• CT & MRI –helps in the confirmation of diagnosis
• HISTOLOGY- similar to the primary lesion
• TREATMENT – “multimodal” & depends on the primary lesion
- bisphosphonates prevents new metastasis & slowing the growth of existing lesion
- radiation ( except kidney cancer)
- fixation for impending/ actual pathological #

LONG BONE TUMOURS ORTHOPAEDICS ,PPTX....

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