2. Bone tumors
◾Condition of skeletal system that are neoplastic or could be
mistaken for neoplastic condition on basis of radiological or
pathological evidence.
◾Progenitor cells- bone tumors can arise from osteoblasts,
chondroblasts, periosteal cells, haematopoietic cells, lipocytes,
schwann cells, fibroblasts, osteoclasts, endothelial cells,
notochordal cells and epithelial cells
4. CHARACTERISTICS OF MALIGNANT TUMOR
◾Most commonly presents as painful bony mass
◾Extends to soft tissue through broken cortex
◾Rarely multifocal
◾Radiographically-
Poorly defined borders
Wide zone of transition
Moth eaten or permeative type of bone destruction
Interrupted type of periosteal reaction
5. APPROACH TO DIAGNOSIS OF MALIGNANT BONE TUMOR
◾ Requires multi-phased work up
◾ T
eam work is necessary.
◾ Goal- whether lesion is benign or malignant
◾ Steps-
History
Local examination
Laboratory test
Radiological test
Histopathological examination
6. HISTORY
◾ Age- some tumors are very age specific. Eg- Ewing sarcoma 10-20yrs;
osteosarcoma 15-25yrs and > 45yrs (bimodal); chondrosarcoma >45yrs;
multiple myeloma >50yrs.
◾ Non specific
◾ Dull aching painful lump
◾ Pathological fracture
◾ Sometimes as incidental finding
◾ H/o exposure to radiation and chemical carcinogens
◾ History of any malignancy anywhere in body or treatment history for any
malignancy at present or past.
7. ON EXAMINATION
◾Swelling- tenderness, location, shape, consistency, fixity to skin
and adjacent structure, mobility, skin over swelling , dilated or
engorged veins.
◾Joint range of movement limitation
◾Sign of inflammation may be present
◾Any other skin lesion anywhere else in the body
◾Regional lymph node
◾Systemic examination to diagnose primary tumor in case of
metastasis.
8. LABORATORY TEST
◾ Hemogram
◾ Erythrocyte sedimentation rate
◾ C- reactive protein
◾ Serum calcium
◾ Serum phosphorus
◾ Alkaline phosphatase
◾ Lactate dehydrogenase
◾ Parathormone
◾ Urinary Bence Jones protein
◾ Urinary 24hrs calcium
◾ Electrophoresis
◾ Bone marrow examination
9. HEMOGRAM AND ERYTHROCYTE SEDIMENTATION RATE
◾ Anaemia may be seen
◾ T
o rule out infection, myeloma and leukemia.
◾ ESR is raised in
Metastasis
Ewing’s sarcoma
Lymphoma
Leukemia
10. SERUM CALCIUM AND PHOSPHORUS
◾Hypercalcemia is most common metabolic complication of
metastatic bone disease.
◾Increased level than normal indicates
Metastasis,
Myeloma.
Hyperparathyroidism
11. ALKALINE PHOSPHATASE
◾Raised alkaline phosphatse indicates high turn over of the bone
◾Raised in osteoblastic lesion
Blastic metastasis from prostate and breast
Active Paget’s disease
Hyperparathyroidism
Growth spurt
12. PARATHORMONE
◾ Increased PTH indicates increased bone metabolism, increased calcium
uptake by bone which might be seen in bone tumors.
◾ increased PTH indicates
◾ PTH should be checked in all bone tumors patients as routine to rule out
Brown tumor as well
13. URINARY BENCE JONES PROTEIN
◾ A Bence Jones protein is a monoclonal globulin protein or immunoglobulin
light chain found in the urine.
◾ Urine containing B-J protein flocculates when heated to 50-60*. Flocculated
protein dissolves on further boiling to 90-100*, and reappears on cooling
below 60*.
◾ It is produced by neoplastic plasma cells and sometimes induced
by hypercalcemia from the calcium released as the bones.
◾ Bence Jones proteins are particularly diagnostic of multiple myeloma, lytic (or
"punched out") bone lesions, anemia, or large numbers of plasma cells in
the bone marrow of patients.
15. X-RAY
◾Gold standard for diagnosing any bone tumor.
◾Lesion is assessed for-
Location,
Size,
Cortical integrity,
Margination,
Periosteal reaction and
Soft tissue involvement
Pattern of bone destruction
21. WHY CT SCAN IN BONE TUMOR?
◾T
o determine intramedullary and extramedullary extension.
◾T
o know relation between tumor and surrounding structure.
◾T
o evaluate integrity of cortex.
◾T
o differentiate between solid and cystic lesion.
◾T
o distinguish between neoplastic and inflammatory mass.
◾T
o know epicenter of tumor
.
22. MRI
◾ Accurate in determining limits of disease
within and outside bone.
◾ Visualizes bone marrow content.
◾ Demonstrates intramedullary extension of
neoplasm.
◾ Identifies skip lesion.
◾ Capable of producing images in desired
plane.
◾ Can differentiate between benign and
malignant tumors.
23. FEATURES OF MALIGNANCY ON MRI-
◾ Mass with irregular
border.
◾ Nonhomogenous signal
intensity with extra
compartmental
extension.
◾ Peritumoural edematous
reaction
◾ In case of soft tissue
tumor
, situated deep to
fascia
>5cm
and measuring
in greatest
24. BONE SCAN
◾ Is an indicator of mineral turnover.
◾ Useful in locating tumors and tumor like lesion in
skeletal.
◾ Technitium 99 methylene diphosphate is injected
intravenously and after 3-4 hrs image is taken
using gamma camera.
◾ It is used to detect skeletal metastasis from a
primary elsewhere in the body.
◾ T
o detect multiple lesion.
◾ T
o localize small lesion.
◾ Cannot distinguish between benign from
malignant lesion.
25. METASTATIC BONE TUMORS
◾ Most common bone tumors are secondaries.
◾ Skeleton is third most common site of metastasis after lung and liver.
◾ Beside myeloma and lymphoma tumor which commonly metastasize to
bone are from breast, prostate, lung, kidney and thyroid.
◾ Breast and prostate carcinoma together accounts for 80% of metastatic
bone tumors.
◾ Bone pain is most feared clinical manifestation of metastatic bone disease.
◾ Other clinical manifestation- hypercalcemia, pathological fractures, spinal
cord or nerve root involvement, bone marrow infiltration.
26. METASTATIC BONE TUMORS
◾ Mirel’s scoring system is to quantify risk of pathological fracture based on various
criteria.
◾ Score ≥ 9/12- high risk of pathological fracture and in an indication of prophylactic
fixation.
Variable Score 1 Score 2 Score 3
Site Upper limb Lower limb Peritrochanter
Pain Mild Moderate Functional
Size < 1/3rd 1/3rd – 2/3rd > 2/3rd
Lesion Blastic Mixed Lytic
27.
28. TYPICAL RADIOLOGICAL FEATURES OF METASTATIS FROM
VARIOUS PRIMARY TUMOR
Commonly lytic Commonly blastic Commonly mixed
Kidney Breast Lung
Thyroid Prostate Breast
Adrenal Bladder Ovary
Uterus Bronchial carcinoid Testis
Lung Cervix
29. METASTATIC BONE TUMORS
◾ Specific investigation depending upon suspected malignancy-
PSA
Acid phosphatase
PAP smear
Thyroid profile
Renal function test
Electrolytes
CA 125
Specific tumor marker
30. PSA & ACID PHOSPHATASE
◾Lytic lesion in males, should suspect for prostate cancer
◾Prostate cancer accounts for 65-75% bony metastasis.
◾Prostate specific antigen is screening tool for diagnosing prostate
cancer.
◾So on X-ray, if we see blastic lesion, we must think of ruling out
prostate cancer since it is leading cause in males.
◾Level of PSA> 4ng/ml is not normal and should be further
evaluated.
◾Serum prostate specific acid phosphatase is also raised
31. ELECTROPHORESIS
◾Immunoelectrophoresis (IEP) is a technology used to detect the
presence of monoclonal immunoglobulin proteins and to
evaluate for immune system disorders.
◾It is an important tool in the detection of malignant lymphoma,
myeloma, and lymphoproliferative disorders.
32. TUMOR MARKER
Tumor marker Disease
Prostate specific antigen Prostate cancer
Prostate acid phosphatase Prostate cancer
CA 15-3 Breast cancer
Calcitonin Medullary carcinoma of thyroid
Chorioembryonic antigen & CA 19-9 Colon and pancreatic cancer
CA - 125 Ovarian cancer
Immunoglobulins Multiple myeloma
33. METASTATIC BONE TUMORS
◾ Radiological investigation in case of metastatic bone tumor
Chest X-ray
CT chest, abdomen and pelvis
Mammogram
Thyroid scan
PET scan
34. MAMMOGRAM
◾Blastic lesion on X-ray of
female patient should raise a
suspect of breast cancer.
◾Mostly patient gives history of
known
breast
recent treatment or
diagnosed case of
cancer with bone pain.
Normal Benign cyst
Breast cancer
35. THYROID SCAN
◾ The radioactive iodine uptake
test, or RAIU test, is a type of scan
used in the diagnosis
of thyroid problems,
particularly hyperthyroidism and
sometimes to rule out thyroid
cancer.
◾131I sodium iodide is used to see
the uptake.
◾ Cold spot in scan indicates cancer
36. PET SCAN
◾ Useful in staging, planning of biopsy,
evaluating the response to
chemotherapy.
◾ It observes metabolism of
musculoskeletal lesions based on
observation of high glycolysis rates of
malignant tumor.
◾ It produces biological images based on
detection of gamma rays that are
emitted by 2- fluro 2 deoxy glucose
(FDG).
◾ It detect primary and metastatic tumors.
◾ It is more sensitive than CT and MRI.
37. HISTOPATHOLOGICAL EXAMINATION
◾ Biopsy is most important to determine histological diagnosis and hence plan the treatment.
◾ Golden rule-
Biopsy should be done when all radiological investigation have been completed.
All suspected malignant tumor and aggressive benign tumor should be biopsied prior to
treatment.
Biopsy should be done by the surgeon who is doing the definitive management.
Exception- where imaging guidance biopsy is needed, there it is done by interventional
radiologist.
Joint should never be violated during biopsy.
Biopsy should be done through recommended site.
Best material for biopsy from the periphery of the tumor. Lytic area provide most
representative tissue.
Biopsy site should be small as feasible with longitudinal incision avoiding major
38. HISTOPATHOLOGICAL EXAMINATION
◾ Types of biopsy-
Needle biopsy
Incisional biopsy
Excisional biopsy
◾ Advantage of open biopsy-
Adequate tissue obtained
Less sampling error
Both cytological and tumor pattern are visible.
◾ Disadvantage of open biopsy-
Bleeding, infection and tissue contamination is more
39. NEWER MODALITIES TO DIAGNOSE
◾ Immunohistochemistry
◾ Molecular cytogenetics
◾ Flow cytometric analysis of nuclear DNA
40. ENNIKING CLASSIFICATION OF MALIGNANT TUMORS
Stage Grade Site Metastasis
1A Low (G1) Intracompartment
l
None
1B Low (G1) Extracompartment
al
None
IIA High (G2) Intracompartment
l
None
IIB High (G2) Extracompartment
al
None
III Grade is
differentiat
Ahis
nto
ylogical grade; G1-
ed, G3- poorly differentiate
w
A
ell
ny
differentiated, G2- m
d, G4- undifferentiated
o
R
d
e
e
g
ra
it
o
el
n
y
al or distant
metastasis
41. MANAGEMENT OF MALIGNANT BONE TUMOR
◾Goal of treatment-
In case of primary malignant bone tumor- to make patient disease free.
In case of secondary malignant bone tumor- to minimize pain to preserve
function and to diagnose the site of primary tumor.
◾Optimal treatment often require combination of radiotherapy,
chemotherapy and surgery.
42. RADIATION THERAPY
◾ Radiation causes cell death by inducing formation of intracellular free radicals that
subsequently causes DNA damage.
◾ Most malignant bone tumors are radio-resistant, except small blue cell tumors
including multiple myeloma, Ewing’s sarcoma and lymphoma.
◾ Therapy reduces the incidence of local recurrence of malignant tumors treated with
marginal resection.
◾ Pre operative use decreases the tumor volume, facilitating the tumor resection.
◾ Complications include: skin irritation, gastro-intestinal upset, urinary frequency,
fatigue, anorexia, fibrosis, osteonecrosis and pathological fracture.
◾ Ewing’s sarcoma is highly radiosensitive tumor, so it was initially treated by
radiotherapy alone. But now it has been reserved for patient who have close
margin of excision or poor response to chemotherapy.
43.
44. CHEMOTHERAPY
◾ With use of modern chemotherapy protocol, 5-year survival rate of malignant bone
tumors have drastically increased.
◾ Adjuvant chemotherapy- chemotherapy administered postoperatively to treat
presumed micro metastasis.
◾ Neoadjuvant chemotherapy- chemotherapy administered before surgical resection
to regress size of the tumor , making limb salvage operation easier, tumor
resectable and also decreases spread of tumor.
◾ Neoadjuvant chemotherapy followed by surgical resection allow histologic
evaluation of effectiveness of treatment. (most valuable prognostic indicator)
◾ T
o combat diversity in resistance, most chemotherapy regime involve combinations
of toxic drugs.
◾ Combinations of the drugs are more effective than single agents.
45.
46. SURGERY
◾Advances in diagnostic images, chemotherapy, radiotherapy and
surgical technique for resection and reconstruction, makes limb
salvage to be reasonable option over amputation.
◾Simon described 4 issues to be considered whenever
contemplating limb salvage over amputation.
1. Would survival be affected by treatment choice?
2. How do short and long term morbidity compare?
3. How would function of salvaged limb compare with that of prosthesis?
4. Are there any psychosocial consequences?
47. SURGERY
◾Decision about what type of surgery to be done depends upon-
Diagnosis
Site of tumor
Extent of tumor
Response of tumor to neoadjuvant chemotherapy
Patient's age, background and financial status
◾Goal of limb salvage- complete eradication of surgery, while
maintaining acceptable function, durability, and cosmesis of limb
48. LIMB SALVAGE VS AMPUTATION
◾ Limb salvage is more extensive surgical procedure.
◾ Long term morbidity.
◾ Risk of infection, wound dehiscence, flap necrosis, blood loss and DVT for which further
surgery may be required.
◾ Ultimately may require amputation.
◾ Small increases in rate of local recurrence.
◾ Durability of reconstruction is variable
◾ Function is better than amputation. (high functional score)
◾ Patient with amputation are more active and least worried about limb injury.
◾ Amputation are technically demanding, often requiring nonstandard flap for closure..
◾ Complication in case amputation include- infection, wound dehiscence, chronically painful
limb, phantom limb and revision surgery for oppositional bone over growth.
49. SURGICAL RESECTION AND RECONSTRUCTION
Margin Surgical procedure Result
Intralesional Piecemeal debulking or
curettage
Leave microscopic
disease
Marginal Shell out en bloc through
pseudocapsule or
reactive zone
May leave either satellite
or skip lesion
Wide Intracompartmental en
bloc with cuff of normal
tissue
May occasionally leave
skip lesion
Radical Extracompartmental en
bloc entire compartment
50.
51.
52. DIFFERENT MODALITIES OF LIMB SALVAGE
◾ Allograft
◾ Endoprosthesis replacement
◾ Autograft
◾ Bone lengthening
◾ Rotationplasty
◾ Arthrodesis
53. RECONSTRUCTION OF BONE DEFECT
◾Most reconstruction are involve preserving a mobile joint, for
which three options are available-
Osteoarticular allograft reconstruction
Endoprosthetic reconstruction
Allograft prosthetis composite reconstruction
◾Allograft arthrodesis still have role in some circumstances
54. OSTEOARTICULAR ALLOGRAFT RECONSTRUCTION
◾ Advantage and disadvantage of this
reconstruction have been reported by
many author.
◾ May have temporary measure to
preserve an adjacent physis in an
immature patient where alternative
include amputation or sacrifice of both
physis.
◾ This could be converted to
endoprosthetic reconstruction when it
becomes necessary.
55. ALLOGRAFT PROSTHETIS COMPOSITE RECONSTRUCTION
◾ Long term solution for some patient.
◾ It avoid complications of degenerative joint disease and articular collapse.
◾ The most important indication is an inadequate length of remaining host
bone to secure the stem of endoprosthesis.
◾ We still use tumor prosthesis for reconstruction with allograft for fixation to
remaining host bone.
◾ Reported complications are- fatigue fracture, infection and non union at
graft host junction
56. Ten-year-old girl with
osteosarcoma of humerus.
A
le
,
ft
Ant
h
e
u
ro
m
p
e
o
r
s
u
t
s
erior
sh
r
o
ad
w
i
s
ogra
t
p
u
h
mor
f
extending down to distal
diaphysis.
a
B
f
,
ter
I
w
n
i
t
d
ra
e
o
r
p
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e
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r
e
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t
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u
o
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to
o
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r.
raph
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to
, Hu
a
m
cc
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r
p
a
t
l all
s
o
t
g
e
r
m
aft i
o
s
f
pre
t
p
u
a
m
re
o
d
r
prosthesis.
b
D
o
,
n
A
e
llog
w
r
i
a
th
ft is
m
f
e
ix
d
e
ia
d
l
to
an
r
d
em
l
a
a
i
t
n
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in
ra
g
l
plates.
E
al
,
lo
P
g
r
r
o
af
s
t
t
.
hesis is cemented into
F, Postoperative radiograph
57. ENDOPROSTHETIC RECONSTRUCTION
◾ It provide advantage of predictable
immediate full weight bearing.
◾ Most endoprosthesis are modular, allowing
for incremental limb lengthening as an
immature patient grows.
◾ Complication- polyethylene wear and
fatigue fracture
◾ Initial fixation with cement provides
immediate stability for quick rehabilitation.
58. ENDOPROSTHETIC RECONSTRUCTION
◾ An intramedullary stem is fixed with
cement, and the shoulder region of the
prosthesis is constructed with a porous
coating with the goal of promoting late
extramedullary cortical
purpose to serve as a
bridging with
purse-string to
protect the cement-bone interface from
particulate debris generated at the
articulating surface and to provide
additional structural support protecting the
junction of the base of the stem with the
body of the prosthesis.
Extramedullary
cortical bridging
develops at the host
bone-prosthesis
interface.
59. A, Anteroposterior radiograph of
distal femur of 7-year-old girl with
telangiectatic osteosarcoma.
B, Coronal MR image.
C, Intraoperative photograph of
resected specimen and custom
Repiphysis prosthesis.
D, Intraoperative photograph after
placement of prosthesis.
E, Anteroposterior radiograph.
60. AUTOGRAFT
◾ Involve taking bone from patient themselves to fill the defect created by
tumor resection.
◾ Vascularized graft with good blood supply is the ideal graft. Eg- fibula.
◾ In upper limb, bone graft is extremely useful.
◾ In lower limb reconstruction should be combined with other form of bone
graft.
◾ Patient own tumor bone is now being used as a bone graft after sterilization.
This technique is often combined with vascularized graft to produce long
term good outcome.
◾ Advantage of using tumor bone is that bone fits perfectly and unite rapidly
although bone is dead.
61. BONE LENGTHENING
◾ Uses principles of either epiphyseal distraction
or distraction osteogenesis.
◾ After resection bone is either
◾ Stabilized using external fixator and
transported to fill the defect
◾ Or acute shortening carried out initially
followed by lengthening procedure later.
◾ Advantage- patient will have vascularized new
bone that replaces the old one.
◾ Disadvantage- takes very long time,
chemotherapy further retards the growth of
bone.
62. ROTATIONPLASTY
◾Principle- excising diseased or damaged
part of limb, then joining the remaining
part together after rotating them through
180*.
◾Most common site- for tumor of distal
femur
◾At the end ankle becomes the knee and
foot becomes useful attachment for below
knee prosthesis.
◾Advantage-
useful functioning limb with prosthesis
63. MANAGEMENT OF METASTATIC BONE TUMOR
◾ Pain management is the prime principle in management of metastatic
bone tumor.
◾ According to Mirel’s scoring system we treat pathological fracture
prophylactically to preserve function of limb.
◾ Multidisciplinary approach to control bone pain-
Drugs therapy- analgesics and bisphosphonates.
Physical therapy- splint
Radiotherapy and use of radiopharmaceuticals.
Anaesthetia methods- blocks
Neurosurgical methods- hypophysectomy
Behavioural approaches- relaxation techniques