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Primary & Metastatic

Malignant Bone Tumours


Bone Producing Tumor

Benign
Osteoid osteoma
Osteoblastoma
Malignant
Osteosacroma
Osteoid osteoma
A small, active, discrete, painful, benign bone lesion
Predominant group : < 30 years old male
Site : any bone except skull
Only occur in bone form by endochondral
ossification
Common site:femur and tibia (50%)
Spine posterior elements
Run a self limiting course
Pain subside as lesion matures lasting 18-30 months
Lesion healed by 3-7 years
But usually required surgery for pain relief
Osteoid osteoma
Pathology
Nidus usually < 1 cm
diameter
nidus
central nodule of woven
bone and osteoid with
osteoblastic rimming
reactive zone
area of thickened bone
and fibrovascular tissue
Clinical features
Pain worst at night
Palpable superficial tender swelling
If lesion in spine backache ,
scoliosis (10% of cases)
Investigation
X-ray
CT diagnosis
Bone scan hot spot
X-rays
Plain X ray
This is belong to a
immatured skeletal
with solitary cortical
lesion
within proximal
diaphysis of fibula.
It is a geograohic lytic
lesion surrounded by
sclerotic bone and
cortical thickening.
It has bone producing
characterize matrix
With no soft tissue
Management
NSAID
Typically relieve with aspirin
May take 3-4 years for symptoms to
resolve
Prophylatic internal fixator may be
needed
10-15% recurrence rate with
percutaneous radiofrequency
ablation
Surgery (Definitive treatment)
Intraoperative localisation with
CT
Then excised nidus or destroyed with
percutaneous radiofrequency coagulation.
Percutaneous inserion of biopsy needle under
CT scan guide
Tissue biopsy to prove needle is properly
Osteoblastoma
Similar to osteoid osteoma but bigger
>1cm
Cell origin : osteoblast
Age and gender : 10-20 years M>F
Bone affected: spine ,jaw, limb
Investigation
X-ray
Radioisotope reveal hot area
X ray
Plain X ray
This is belong to a
matured skeletal with
solitary cortical lesion
within distal femoral
metaphyseal.
It is a well demarcated
osteolytic lesion which
contain small flecks of
ossification and sclerotic
rim.
Nidus > 2cm
It has bone producing
characterize matrix
Management
Not respond to aspirin
Curettage or marginal excision
Bone grafting

X-ray of rush
Radiographic differential for
osteoblastoma
osteosarcoma
Aneurysmal bone cyst (ABC)
osteomyelitis
osteoid osteoma
Osteoid Osteoma Osteoblastoma

Incidence 10% of benign tumors 3%of benign tumors


>2 cm (average, 3.5 - 4.0
Size < 2 cm(typically <1.5cm)
cm)
Site > 50 % in long bone > 35% in posterior
diaphysis elements of the spine

Location Proximal femur > tibia vetebral column >


diaphysis > spine proximal humerus > hip

Natural History Self-limited Progressive


Benign appearance.
Benign appearance. No
Localized growth, with
growth potential.Central
aggressive potential.
Histology nidus composed of more
Central lesion less
organized osteoid and
organized, with greater
lined by osteoblasts.
vascularity.
Symptoms Nocturnal pain, relieved Dull ache, not relieved by
by NSAIDS NSAIDS. >50% of spine
Osteosacroma 1

most common primary


sarcoma of bone
Types Paraosteal

Classic form intramedullary


osteosacroma 2
Surface osteosacroma
Telangnictatic
Secondary :
paget
post radiation
periosteal
Incidence
bimodal distribution of occurrence
majority occur in thesecond decade of life
second peak in occurrence is in elderly patients (50-70yo)
withPaget's disease
Cell of origin : osteoblast
M:F 2:1
Site of bone: long bone metaphyses (intramedullary
type)
Common bone: distal femur, proximal tibia, proximal end
of humerus
Mets:
10-20% of patients present with pulmonary metastases(obtain
CT of chest) at first visit
lung is most common site of metastasis
bone is second most common site
Clinical feature
Pain (first symptom) worst at night
and gradual increase intensity
Tender
Swelling and inflamed
Stretched shiny andLate sign
mobile skin
Investigation
ESR
Serum alkaline phosphotase
Modality Purpose
Plain X ray Sun burst appearance,
Codmans triangle
Large soft tissue mass
Cortical breach common
Variable combination of
bone destruction & bone
formation
chest CT scan At first visit to evaluate
pulmonary mets
MRI To determine
soft tissue involvement
neurovascular
involvement
presence of skip lesions
Bone scan To detect
radiographically occult
Enneking staging
for staging bone sarcomas
Plain X ray
This is belong to a
matured skeletal with
solitary lesion centered
within distal femoral
metaphyseal.
It is a sclerotic lesion
There are cortical
erosion and "sunburst"
periosteal reaction
around the distal
femur.
It has bone producing
characterize matrix
with soft tissue
involvement.
Management
Multiagent neoadjuvant chemotherapy
8-12 weeks
Surgery
Wide resection or amputation
Reconstruct with bone graft or
endoprotheses
Surgical resection of pulmonary metastases
Prerequisite : no extra-pulmonary mets and good
local disease control
Pathological specimen is examined and
assess response to chemo
Continue chemo 6-12 months
Wide resection

Intra-compartment and outside of reactive zone


Amputation indication
pathologic fracture
encasing neurovascular bundle
enlarging during preop chemo AND adjacent to
neurovascular bundle
Limb salvage
Requires ability to achive safe, tumor free
margins
Preserve function
Preserve nerve
Preserve or reconstruct vessels
Preserve sufficient muscle for functional motor and
soft tissue coverage
megaprosthesis used to
reconstruct the defect
after the resection.
A rotating hinge distal femoral
megaprosthesis was used to
reconstruct the resected bone
after neoadjuvant
Reference
http://www.orthopaedicsone.com/dis
play/PORT/Intramedullary,+High-
Grade+Osteosarcoma
Cartilage Producing
Bone
Tumour
(Benign)
Osteochondroma
cartilage-capped exostosis
one of the commonest tumours of bone
(45%)
*developmental lesion
commonest sites are the fast-growing ends
of long bones and the crest of the ilium
50% are distal femur, proximal tibia, ribs
teenager or young adult
occasionally, there is pain due to an
overlying bursa or impingement on soft
tissues
in long bones
growth leaves the bump stranded further
down the metaphysis
it may go on growing but at the end of
the normal growth period for that bone, it
will stops enlarging

**any further enlargement after the


end of the growth period is suggestive
of malignant transformation
X-ray
well-defined exostosis emerging from
the metaphysis
its base co-extensive with the parent
bone
looks smaller than it feels
There is a mushroom-shaped, pedunculated bony
excrescence arising from the anteromedial aspect of the
distal femoral metaphysis
Large cartilage-capped exostosis Hyaline cartilage cap with
(calcified) bony excrescence grows
Sessile. Pedunculated or
Treatment
Excised if it is symptomatic !!
Extracapsular marginal excision
if recently become bigger or painful then
operation is urgent. Why?
Any suspicious features must further managed
by imaging and staging before biopsy

If the benign tumour is known to be enlarging


after end of growth period. How would you
treat ?
Complication
1% for maglinant transformation in
solitary lesion
6% for maglinant transformation in
multiple lesions
features suggestive of malignant change
are:
enlargement of the cartilage cap in
successive examinations
a bulky cartilage cap (more than 1 cm in
thickness)
irregularly scattered flecks of calcification
within the cartilage cap
Chondroma
(Enchondroma)
Asymptomatic
discovered incidentally on x-ray OR after a
pathological fracture
seen at any age (mostly in young people)
occur in any bone preformed in cartilage
(tubular bones of the hands and feet)
Originating within the medullary cavity
lesions may be solitary or multiple and part
of a generalized dysplasia
X-ray
well-defined
centrally placed radiolucent area at the
junction of metaphysis and diaphysis
sometimes the bone is slightly
expanded

in mature lesions, pathognomonic


features when:
flecks or wisps of calcification within the
lucent area
Enchondroma of middle phalanx
-Geographic lesion
-Stippled calcifications in lesion
-Phalanx is expanded
-Significant endosteal scalloping
Popcorn strippled calcification Curettage and bone grafting
and rings
Treatment
Not always necessary
If appears to be enlarging OR presents
as a pathological fracture
curettage and bone grafting
Fairly high recurrence rate
Chondromas in expendable sites are
better removed en bloc
Complication
Small but significant risk of malignant
changes:
< 2% with solitary lesions
30% with multiple lesions (Olliers disease)
100% with haemangiomas (Maffuccis
syndrome)

signs of malignant transformation in


patients over 30 years are:
onset of pain
enlargement of the lesion
cortical erosion
Dyschondroplasia (Olliers
disease)
Rare
Easily recognized
Defective transformation of physeal cartilage
columns into bone

Unilateral
Affected limb is short
Bent if growth plate is asymmetrically affected
Fingers and toes contain multiple
enchondromata
X-ray
Radiolucent streaking
Extending from physes into
metaphysis (appearance of
incomplete ossified cartilage
trapped in bone)
Multiple enchondromata in hand
and feet
Bent femur, slow Multiple chondromas
growth
Maffucis Disease
Development of multiple enchondromas
and soft tissue haemangiomas
Skin and viscera
Appear during childhood
Boys = Girls
Strong tendency of malignant change
(bone lesions or soft tissues)
Should be monitored throughout the life
Cartilage Producing
Bone
Tumour
(Malignant )
Chondrosarcoma
Peak incidence 30-60 years old
Male : Female 2 : 1
Slow growing
Various forms
Location in bone
Develop without precedent (primary) or by
malignant change in pre-existing benign lesion
(secondary)
Predominant cell type in tumour
Majority fall into:
Central tumour
Peripheral tumour
Juxtacortical tumour
Clear-cell tumour
Sites
Pelvis 30%
Femur 20%
Femoral head 10%
Ribs 10%

Clinical features
Deep, dull, achy pain
Pain at night
Nerve dysfunction
Limitation of joint movement
Pathologic #
Central Chondrosarcoma
Tumour develops in medullary cavity
Most common at proximal end of the
femur or innominate bone of pelvis

X-ray
Expended, radiolucent area in bone
Flecks of increased density
Aggressive lesions may take on a globular
appearance with scalloping or destruction of
cortex
Pale glistening cartilage tissue found in
medullary cavity
Haemorrhagic tissues
Peripheral
Chondrosarcoma
Arises in cartilage cap of an exostosis
(osteochondroma)
*Exostoses of pelvis and scapula more
susceptible than others to malignant
change

X-ray
Bony exostosis
Often surmounted by clouds of patchy
calcification in the otherwise unseen
lobulated cartilage cap
Fluffy calcification and poorly outlined
typical cartilaginous calcifications
round with a clear center
a part of the lesion is made of high-signal lobules, but the lesion is
heterogeneous.
The dedifferentiated part takes up strongly contrast medium after
injection
Juxtacortical (periosteal) chondrosarcoma
Lesion appears as an excrescence on the
surface of one of the tubular bone
Arises from the outermost layer of cortex
deep to periosteum

X-ray
Comprise features of both chondrosarcoma
and periosteal osteosarcoma
Flecks of calcification
Sunray streaks
New-bone formation at margin of stripped
periosteum
Anteroposterior
radiograph
revealing a well
formed sclerotic
periosteal
reaction in
metaphysis of
the distal femur
Axial CT revealing

tumour contained
calcified densities
characteristic of the
external bone
surface

associated with a
thickened cortex
Axial T1-weighted MRI showing a sharply
delineated mass with low to intermediate
signal intensity in the bone surface of the
metaphyseal region

Axial T2-weighted MRI with a bright signal


and associated lobulated structure with
hypo-intense septa.

Gadolinium-enhanced T1-weighted
MRI with peripheral and septal
enhancements. No intramedullary
extension or edema was identified.
Clear-cell chondrosarcoma
There is doubt as to whether this is really a
chondrosarcoma
Resembles an aggressive chondroblastoma
(typical location in the head of femur rather
than metaphysis)
Slow growing, eventually metastasise

X-ray
Usually osteolytic, expansile lesion
May be focally calcified
Often a sharp interface between tumor and
surrounding bone
Overlying cortex is usually thin, but intact
Pathology
High-grade tumours are more cellular
There may be obvious abnormal features
of the cells
Plumpness
Hyperchromasia
Mitoses
Treatment
Present the ideal case for wide excision
and prosthetic replacement
Amputation is preferable, provided the
lesion can be removed without exposing
the tumour OR causing unacceptable loss
of function
Removal of the affected part of the bone
and some healthy tissue around it
Replaced with the prosthesis or bone graft
Tumours tend to metastasise late
therefore attempt wide local excision
initially
Resistant to chemotherapy and
Low-grade chondrosarcoma in an arm or leg, curettage
with cryotherapy is an option.

If the tumor is high-grade, limb-sparing surgery will be


done if possible.

Sometimes amputation is needed to completely remove


the cancer.

If the chondrosarcoma has spread to the lung and there


are only a few metastases, they may be removed
surgically.

Chondrosarcomas in the skull are hard to treat.


Complete surgical removal is difficult, and may cause
serious side effects.

Sometimes the patient is treated withradiation therapy.


Giant Cell Tumor
Benign
Usually solitary
Nearly located at very end
of long bone (metaphyseal/epiphyseal)
- Distal femur
- Proximal tibia
- Proximal humerus
- Distal radius
- Spine- anterior vetebral body
Most common tumour occur in distal phalanx
Locally aggressive
Can undergo malignant transformation ( 5-10 % )
Rarely Metastasises
Incidence
10 % of benign bone lesions
Not seen until after growth plate
closes : age 20-40 years old
More common in Female
Clinical features
Young adult
Persistant intraosseous pain at the
end of long bone
with movement of nearby joint
increases with activity and relieved on
rest
progressively increases
Swelling - reactive knee effusion
Pathological fracture 10-15%
Pathology
Chocolate brown, soft spongy & friable with cystic
cavities filled with blood
Comes away in pieces quite easily when curetted but
it is difficult to completely remove from surrounding
bone
Aggressive lesions have poorly defined floor and
appear to extend into the surrounding bone
Histology

Mulinucleated giant cell which is round to oval, polygonal,


or spindle shaped
Campanacci Grading
System
Grade 1
-Lesion confined within bone
well-defined margin
- an intact cortex.
Grade 2
- Lesion expanding cortex
- relatively well-defined margin but no radiopaque rim
- cortex is thinned and moderately expanded.
Grade 3
- Breach of cortex: 1- involvement of joint
2- Distant Metastasis
- indistinct borders
- cortical destruction.
Ennekings Staging
Stage 1
Benign latent GCT
No local aggressive activity
Stage 2
Benign active GCT
Imaging Studies: alteration of cortical bone structure
Stage 3
Locally aggressive GCT
Imaging studies: lytic lesion surrounding medullary &
cortical bone
May be indication of tumor penetration through cortex
into soft tissue
Investigations
Xray
Well defined radiolucent (Lytic lesion) in
meaphysis/epiphysis extending up to joint
surface
Eccenteric
Junction with normal bone poorly defined with
or without marginal sclerosis
Thin cortex & sometimes ballooned
Soap bubble appearance- scalloping &
ridges in wall (septation)
Pathognomonic feature - Extends up to
subchondral bone plate
distal femur reveals an expansile lytic metaphyseal-
epiphyseal lesion.
Distal radius reveals an aggressive lesion characterized by
extensive local bony destruction, cortical breakthrough and
significant soft-tissue expansion.
Distal tibia demonstrates extension of the lesion to
the articular surface.
Investigations
CT, MRI
- Reveal extent of tumor

Arthroscopy
- establish whether articular surface
has been broached

Biopsy
Differential Diagnosis
Aneurysmal bone cysts (ABC)
Hyperparathyroidism (Brown Tumor)
Fibrous cortical defect(FCD)
Treatment
Extended curettage
Is a technique of intralesional excision where the margins of
the excision are extended by a surgical, chemical, or thermal
adjuvant.
Adjuvant options include phenol, liquid nitrogen, high-speed
burr, cementation with polymethylmethacrylate, and argon-
beam coagulation. The technique described here uses a
combination of high-speed burr, argon-beam coagulation, and
cementation
decreased recurrence rates and significantly less surgical
morbidity
For well-confined, slow growing lesions with benign histology
85%-90% success rate of local control

distal femur with


Extended curettage of giant cell polymethylmethacrylate and
tumor by Argon beam laser Steinman pins inserted into the
Treatment
Excision
- For aggressive tumors
- Followed by bone grafting or prosthetic
replacement

Radiation therapy and embolization


- generally are reserved for cases in which
surgical treatment is not feasible.
Mega Prosthesis (knee replacement)
if involves articulation surface of the
knee

proximal femoral GCT


distal femoral GCTproximal tibial GCT
Custo-mega-
prosthesis used
for proximal
Ewing Sarcoma
Is arised from endothelial cells in the bone
marrow.
10-20 years of age

M>F

Usually in tubular bone ( tibia, fibula,


clavicle)
Clinical Features
Pain

- Throbbing

- Localized

- Intermittent

- Worse at night

Swelling (fusiform, tender diaphyseal)

Others:

- Fever

- Sweating

- Leucocytosis

- Anaemia mimics acute osteomyelitis


Investigation

Imaging

1. X-ray:

-. Area of bone destruction (mid-diaphysis)

-. New bone formation ( extend along the shaft, sometimes

appear as fusiform layer of bone around the lesion onion-

peel effect )

-. Tumour extend into surrounding soft tissue, with radiating

streaks of ossification and reactive periosteal bone at

proximal and distal margin.

-. ( sunray appearance and codmans triangles)


demostrate mottled, osteolytic lesion (blue circle) with poorly
marginated edges in the diaphysis of the bone. There is sunburst
periosteal reaction (red circle) and lamellated periosteal reaction (white
arrows).
2. CT, MRI
- Large extraosseous component

3. Radioisotope scan

- Multiple areas of activity in skeleton


Pathology
1. Macro
-. Tumour is lobulated, large, grey ( like brain), red
(like redcurrant jelly when there is hemorrhage)
2. Micro
- Sheets of small dark polyhedral cells, no
regular arrangement and no ground
substance
Diagnosis
- Must always exclude bone infection

- On biopsy, must recognize malignant round-cell


tumour (*distinct from osteosarcoma)
- Other round cell tumour:
Prognosis
-Poor

Treatment

-neoadjuvant + wide excision + adjuvant chemotherapy


Chemotherapy(more effective than radiotherapy): 5-year survival rate of
about 50%.

Option:

Preoperative neoadjuvant chemotheraoy+wide excision( if the tumour is

at the favourable site)

radiotherapy+ local excision(if it is less accesible)

Then a further course of chemotherapy for one year.

- Postop radiotherapy maybe added if the resected specimen is found not


to have a sufficiently wide margin of normal tissue.
Bone cysts
Unicameral (Simple) Bone Cyst
Also known as solitary cyst
Benign lesion which occurs during growth
Cysts may be active or latent
Active cysts are located near growth plate, but
they move further away as child grows &
become inactive (latent)
Aetiology :
-unknown
-venous obstruction leading to a transudate of
fluid
Incidence
20 % of benign bone lesions
Age 5-15 years not found in adult
M:F 2:1
Proximal humerus (63%)
proximal femur (15%)
Maybe found in unusual site (eg: calcanum,
pelvis) in patient >17 yrs

Clinical
Asymptomatic
Usually discovered after a pathological fracture
or as an incidental finding of xray
Radiographic features
Well defined, central osteolytic area
with thin sclerotic margin
Metaphysis in young & move towards
diaphysis with growth
Symmetric expansion with thinning of
cortex and bone expansion
Falling leaves (pathological fracture
with fallen cortical fragment in base of
empty cyst is pathognomonic)
Treatment
Non operative
Immobolise alone proximal humerus
lesion with pathologic fracture
Aspiration / methylprednisolone acetate
injection active cysts in the proximal
humerus

.
Operative
Curretage and bone grafting +/-
internal fixation based on tumour
location- symptomatic latent cyst that
have not responded to steroid
injections.

Contraindication: active lesion as


communication with physis may lead to
growth arrest
Aneurysmal Bone Cyst
Benign and non-neoplastic reactive bone lesion filled
with multiple blood filled cavities
May arises spontaneously after degeneration or
trauma or vascular disturbances in some other lesion
Can be locally destructive to normal bone and may
extend to soft tissue
Epidemiology- 75 % of patients are <20 y/o
Location :
- 25 % in spine
-20 % in long bones (distal femur, proximal t ibia)
-Usually in metaphysis
-Posterior elements of pelvis
May complain of pain and cause a visible or palpable
swelling of bone
Radiographs:
-Expansile, eccentric and lytic lesion
with bone septae (bubbly appearance)
-Usually in metaphyseal
-Classic cases have thin rim of
periosteal new bone surrounding lesion
-No matrix mineralization
-Occasional sites include vertebrae and
the flat bones
Histology
-Cavernous space
-Blood-filled spaces
without endothelial
lining

Non-operative fracture management- ABC for


adult fracture

Operative
-Aggressive curretage and bone grafting
symptomatic
ABC without acute fracture
METASTATIC CANCERS
OF BONE
EPIDEMIOLOGY
Approximately 1.2 million patients present with
cancer each year in the United States. Of these,
50% have metastases to bone.

In contrast, 2,700 patients (0.2%) per year


develop primary bone sarcoma.

Bone is the most common site for secondary


cancer especially in patient >50 yo

10% cases no primary tumor is found


Common Sources of Common sites for bone
metastasis metastases
Breast (MOST Vertebrae
COMMON) proximal half of
prostate femur
Kidney Pelvis
Lung humerus
Thyroid
Bladder
GIT
Pathophysiology
Spread through
blood stream (mostly)
occasionally direct spreading (eg.
visceral tumours to adjacent pelvis or
ribs)
Pathophysiology
Metastases are usually osteolytic, and
pathological fractures are common.

Bone resorption (osteolytic) is due either to


direct action of tumour cells, or
tumour-derived factors (stimulate
osteoclastic activity)

Osteoblastic lesions (uncommon); usually occur


in prostatic carcinoma.
Clinical Features
Patient aged 5070 years with any destructive
bone lesion, the differential diagnosis must
include metastasis.
Sudden appearance of pain (most common / might
be the only symptoms) eg. Backache, thigh pain
Sudden collapse of vertebrae / fracture
Symptoms of hypercalcaemia
Anorexia / nausea
Thirst
Polyuria
abdominal pain
general weakness
depression.
Clinical Features
In children < 6 yo, metastatic lesions
are most commonly from adrenal
neuroblastoma.
The child presents with bone pain
and fever; examination reveals the
abdominal mass.
Investigations
X-rays
Osteolytic / moth-eaten
rarified areas in the medulla
marked bone destruction
pathological fracture
Osteoblastic deposits prostatic
carcinoma
pelvis may show a mottled increase in
density (must be distinguished from
Pagets disease or lymphoma)
Typical x-ray appearance of osteolytic bone
metastases.
Typical x-ray appearance of osteoblastic bone metastases
(prostate CA)
Investigations
Bone scans with 99mTc-MDP
most sensitive for detecting silent
metastatic
increased activity areas are selected for
x-ray
Investigations
MRI
Check cord compression in the case of vertebral
fracture
Investigations

CT Scan

An example of metastatic breast cancer, in this case illustrated on CT


and bone scan. The CT clearly depicts multiple sclerotic lesions in
thoracic vertebrae and the sacrum, which show corresponding
Investigations
PET Scans of Bone Metastases
may show these lesions even better than CT
scans and better than MRI
show the response to radiation and
chemotherapy
helpful for hard to see areas like the ribs and
scapula

PET's may be best for osteolytic lesions and


bone scans best for osteoblastic metastases.
PET SCAN

PET scan showing bone metastasis in lower thoracic vertebrae


PET SCAN
PET SCAN

The lesion is seen better in PET scan than MRI


PET SCAN
Other Investigations
ESR may
Hb
Serum alkaline phosphatase
Serum acid phosphatase in prostate
CA
Tumor markers (eg. Breast CA CEA)
Treatment Objectives

Decrease pain
Restore function (eg. weightbearing)
Maintain/restore mobility
Limit surgical procedures
Minimize hospital stay
Treatment
Control of pain and metastatic
activity
Treatment of fractures
Prophylactic fixation
Spinal stabilization

In majority of cases, especially those with multiple


secondaries, treatment is entirely symptomatic.
Thus, elaborate witch-hunts to discover the source of an
occult primary tumour are avoided.
Control of pain and metastatic
activity
Most patients require analgesics, but the more
powerful narcotics should be reserved for the
terminally ill.

Unless specifically contraindicated,


radiotherapy is useful.

hormone therapy (mostly for prostate and


breast CA) :
Stilboestrol - prostatic CA
androgenic drugs or oestrogens breast CA
oophorectomy combined with adrenalectomy
or by hypophyseal ablation - Disseminated
Control of pain and metastatic
activity
Hypercalcaemia management :
adequate hydration
reducing calcium intake
administering bisphosphonates
(to prevent complications : renal acidosis,
nephrocalcinosis, unconsciousness and coma)
Treatment of fractures
Shaft fractures should always be treated by
internal fixation and (if necessary) packing with
methylmethacrylate cement.
Relieve pain and make nursing easier

Fractures usually unite satisfactorily.

Postoperative irradiation is essential to


prevent further extension of the metastatic
lesion.
Prophylactic fixation
Large deposits that threaten to result in
fracture should be treated by internal
fixation while the bone is still intact.

As a rule of thumb, where 50% destruction


of a single cortex of a long bone (in any
radiological view) inevitable
pathological fracture
Prophylactic fixation

Preoperative radionuclide scan show presence


of other lesions in that bone more extensive fixation
and postoperative radiotherapy.
Spinal stabilization
Metastatic spinal disease is 40 times more common than
all primary tumours of the spine together (Galasko et al.,
2000).

40-70% of all malignant tumours have a spinal


metastasis
mostly in the thoracic spine
mainly in the vertebral body

The aims of intervention


decrease pain
preserve the ability to walk
maintain urinary and faecal continence
Prolong survival
Spinal stabilization
spine is stable well-fitting brace
Spine is unstable posterior spinal fusion followed by
radiotherapy
Preoperative assessment for the need of cord decompression
(urgent cord decompression if overt signs and symptoms!!):
CT
MRI
Myelography

BUT, if the patient is in a terminal stage, it may be more


humane to give radiotherapy, with or without
corticosteroids and narcotics, to control oedema and
pain.
Other treatments
debulking of the tumour or removal of a
solitary metastasis by vertebrectomy and
reconstruction.
better functional outcome than radiotherapy

Patients remain ambulatory and continent for


longer and the 5-year survival rate is around 18%.

Radiotherapy alone is reserved for


soft tissue compression
inoperable cases
Preoperative radiotherapy has been shown to the postop
infection rate
Prognosis

Numbers of Bauers
Survivorship at 1 year
positive criteria
45 50%
23 25%
1 or None Majority survived for < 6
months
Multipl
e
Myelo
ma
(March as Myeloma Action
Month)Burgundy ribbon by
International Myeloma
Introduction
Is a malignant B cell lymphoproliferative disorder of marrow which the plasma
cell predominating.

Multiple myeloma is the second most common blood cancer.

Multiple myeloma causes cancer cells to accumulate in the bone marrow, where
they crowd out healthy blood cells. Rather than produce helpful antibodies, the
cancer cells produce abnormal proteins that can cause kidney problems.

The effects on bone marrow cell proliferation and increased osteoclastic activity
Osteoporosis
Myelomatosis: appearance of discrete lytic lesions throughout skeleton

Plasmacytoma a particularly large colony of plasma cells may form what


appears to be a solitary tumor

At operation the affected bone is soft and crumbly


Etiology
1. Unknown

2. Radiation exposure
Large dose exposure with long latent peroid

3. Epidemiologic factors
Occupational exposure to petroleum product has higher
incidence ; wood workers & leather workers

4. Genetic abnormalities
Tranlocations t (11;14),t( 4;14)
Deletion of 13q

5. Oncogenes
overexpression of myc and ras growth promoting oncogenes

Mutation in p53 and RB growth supressing antioncogenes


Pathophysiology
Neoplastic proliferation of plasma cells
involving more than 10% of the bone marrow

B-cell maturation is associated with a programmed


rearrangement of DNA sequences in the process of encoding
the structure of mature immunoglobulins.

Leading to overproduction of monoclonal IgG, IgA, and/or


light chains.

Subsequent overabundance of monoclonal paraprotein (M


protein).
Histology

Bone marrow
Bone marrow biopsy
aspirate
demonstrating sheets
demonstrating
of malignant plasma
plasma cells of
cells in multiple
multiple myeloma.
myeloma.
Note the blue
cytoplasm,
eccentric nucleus,
Morphologic Features

A. Osseous (Bone Marrow) Lesions


. most commonly affected bones are those with
red marrow i.e skull, spine, ribs and pelvis
. The lesions begin in the medullary cavity, erode
the cancellous bone and ultimately cause
destruction of the bony cortex.
. Radiographically these lesions appears as
punch out, rounded, 1-2 cm sized defects in the
affected bone.
. Grossly , the normal bone marrow is replaced
by soft, gelatinous, reddish- grey tumors.
Skull bone
X-ray
X ray features of multiple myeloma
B. Extra osseous Lesions
Late in the course of disease
1. Blood atypical plasma cells present in the
blood of 50% of patient resulted in
neutropenia, anemia and thrompocytopenia.
2. Myeloma kidney renal involvement due to
filtration of light chain proteins (Bence Jones
proteins) which are precipitated in the distal
convoluted tubule.
3. Myeloma Neuropathy infiltration of nerves
trunks by tumors cells resulting
polyneuropathy.
4. Systemic amyloidosis Primary generalised
amyloidosis (AL amyloid)
5. Liver & Spleen involvement hepatomegaly
and spleenomegaly
Clinical features
Typically aged 45 65 years and presentation is usually insidious (male
> female)

Osteolytic bone lesions Weakness, Backache, bone pain, pathological


fracture, and spinal cord or root compression due to vertebral collapse

Hypercalcemia thirst, polyuria, abdominal pain

Anemia, neutropenia, thrombocytopenia (marrow infiltration by plasma


cells)

Serum hyperviscosity-> sludging in the capillaries

Renal impairment due to light chain deposition (bence-jones protein)


Imaging
Xray
Osteoporosis
(myeloma is one of the commonest cause of
osteoporosis and vertebral compression fracture in
men over the age of 45 years)
Multiple punched-out defects in the skull, pelvis and
proximal femur , a crushed vertebra, or a solitary lytic
tumor in a large bone metaphysis

MRI is useful in detecting thoracic and lumbar spine


lesions, paraspinal involvement, and early cord
compression.
Radiograph of the skull demonstrating a
typical lytic lesion in multiple myeloma.
Investigations
Screening test serum and urine electrophoresis
(monoclonal protein band)
FBC Pancytopenia with marked rouleaux
formation
High ESR up to 100mm/hr
Raised urea and creatinine
Hypercalcemia
Urinanalysis - Bence-jones protein (Free or light
chains) and hyperurecemia
Sternal marrow puncture may show
plasmacytosis, with typical myelomacells
Bone marrow aspirate or biopsy
Electrophoresis of Plasma Proteins

Normal

M band/spike

In Myeloma patient
Peripheral Blood picture of Multiple Myeloma
Bone marrow picture
Staging
Management
Incurable disease, its chronic
relapsing and remitting.

Treatment aims:
Controlling disease
Prolonging survival
Maximizing quality of life
Management
Pain control

Correction of fluid balance

Management of limb fracture


Internal fixation and packing of cavities
with methyl methacrylate cement
Perioperative antibiotic prophylaxis
Management
Bisphosphonates
Inhibit bone resorption
Inhibit proliferation of MM cells
Prevent bone pain
Prevent hypercalcemia
Prevent pathological fractures
Management
Management of spinal fracture
Bracing
Internal fixation
Decompression of cord

Chemotherapy
Melphalan with prednisolone
Melphalan with prednisolone and thalidomide
Melphalan with prednisolone and bortezomib
Stem cell transplantation
Induction followed by high-dose therapy with autologous stem cell
transplantation (ASCT) is the standard treatment.

Induction therapy :
Bortezomib-dexamethasone
The addition of a third agent (eg, thalidomide, doxorubicin, lenalidomide or
cyclophosphamide) has shown higher response rates.

Peripheral blood progenitor cells are the preferred source of stem cells,
rather than bone marrow.

Allogeneic stem cell transplantation should only be carried out in the


context of a clinical trial and only in patients with good response before
transplant.
Prognosis
Poor, with median survival rate of
only 2-5 years
Spinal Metastases
Epidemiology
3rd most common site for
metastasize, following lung and the
liver.
Vertebral body is the
most common site
2/3 of malignant tumour
develops metastasis
(Chong-Suh Lee & Chul-Hee Jung, 2012)
Frequently in breast (23.3%), lung
(21.2%), prostate (9.3%), thyroid
(7.3%), renal cell cancer (5.3%) and
unknown source (6.6%).
(Singh VA et al, 2014)

Male: commonly prostate and lung


cancer
Female: breast cancer
10-20% have spinal cord
compression due to metastasis
(Siegal T, 1989)
Pathophysiology
Affect by 3 factors:
-metastasis pathway
-tissue receptivity on tumour emboli
(seed & soil theory)
-intrinsic factor of tumour cells
metastsis pathway:
-arterial system
-direct invasion
-lymphatics
-venous system (most common)
Clinical manifestations
Axial pain with or without radicular
pattern
-nocturnal & progressively worse
-not associate with position or
activity
-not alleviate by rest
-pain-associated scoliosis may
appear
Neurologic deficit
-5-30% shows neurologic symptoms
-due to mechanical compression by
the lesion
-due to vascular compromise of
spinal cord may appear.
-thoracolumbar deteriorates >
cervical lesion
Spinal deformity
General decline of physical condition
asymptomatic
Investigations
Laboratory:
-CBC (pancytopenia)
-ESR
-tumor marker (PSA, CEA, -fp & -
hCG)
Radiological (X-ray):
-AP view of spine (winking owl sign)
-lateral view of spine
Lateral plain radiograph of cervical 'Winking owl sign' due to osteolysis
spine. Radiolucency on C3 body with of right pedicle by T12 metastatic
compression fracture is observed. cancer. (Chong-Suh Lee & Chul-Hee
(Chong-Suh Lee & Chul-Hee Jung, Jung, 2012)
2012)
Cont.
Whole body bone scan
-30-50% of cancellous bone
destruction should occur in order to
observe abnormal findings.
-bone scan most sensitive for early
dianosis of tumour. (Boriani S et al, 1997)
-sensitive in osteoid formation, up to
2mm lesion.
A 63-year-old male patient with oral
cavity cancer. His whole body bone
scan shows metastasis to T12.
(Chong-Suh Lee & Chul-Hee Jung,
2012)
Cont.
CT scan
-useful for understanding level of cortical
bone erosion, pre-op assessment and
surgical planning.
MRI
-non-invasive, safe and free from radiation
-useful as screening test for whole body
metastatic spinal tumour.
-useful
Computed tomography scan of Giant Recurred giant cell tumor.(A)T2-
cell tumor in T11. Tumor destructed weighted magnetic resonance
vertebral body, pedicle and imaging (MRI): recurred tumor (black
transverse process, and invasion to arrow).(B)T1-weighted MRI with
thoracic cavity and spinal canal. enhancement.
(Chong-Suh Lee & Chul-Hee Jung, (Chong-Suh Lee & Chul-Hee Jung,
2012) 2012)
Biopsy
-confirmatory test
-require in active treatment such as
surgery
-direct biopsy during surgery prior
definitive surgery is beneficial
-method includes:
-percutaneous needle biopsy (accuracy
up to 89%
under CT/ultrasound)
-open incisional biopsy
-open excisional biopsy
Computed tomography guided biopsy.(A)Tumor at right
vertebral body (arrow).(B)Biopsy was done by transpedicular
approach.
Angiography
-useful for surgical planning
PET scan
-help in selection of
treatment plan
Staging
Enneking Staging
Weinstein-Boriani Biagini (WBB)
Staging
Tomita Scoring system
Treatment
Aim: to attain pain relief and
restoration of neural function for
enhancing quality of life.
Non-operative treatment
-conservative treatment
-hormonal treatment
-angiography & embolization
-radiotherapy
-corticosteroid & biphosphanates
Operational treatment
Based on Harringtons Scoring.
Surgical margin
Intra-lesional
Marginal
Wide
Radical
Method
Spinal decompression
-to relieve neurologic symptoms
Spinal stabilization
-to reduce already developed
deformity, and to recover immediate
spinal stability allowing early
ambulation.
Prognosis
Based on Bauer & Wedin criteria (Bauer HC & Wedin R, 1995)

Criteria:
-No organ metastasis
-No pathological fracture
-Solitary skeletal metastasis
-No lung cancer
-The primary tumor is breast carcinoma, renal
cell carcinoma, lymphoma, or myeloma

Prognosis:
-The one-year survival rate can be estimated from the number of the
above
criteria that are positive:
-45 positive criteria one-year survival 50%
-23 positive criteria one-year survival 25%
-01 positive criteria one-year survival 0%
References
https://www.ncbi.nlm.nih.gov/pmc/articles/
PMC3302920
/
https://www.ncbi.nlm.nih.gov/pmc/articles/
PMC3036978
/
https://www.ncbi.nlm.nih.gov/pmc/articles/
PMC3036978
/
https://
www.ncbi.nlm.nih.gov/pubmed/7740944
Apleys System of Orthopaedics and