dr.

Helmi Ismunandar SpOT

TUMOURS

Apley 9th ed
p.187-223
Introduction

 Tumours, tumour-like lesions and cysts are considered

together 
 similar clinical presentation and management,
 definitive classification of bone tumours is still
evolving and some disorders may yet move from one
category to another
 Benign lesions are quite common, primary
malignant ones rare  they mimic each other
 critical are the decisions on treatment
Classification

 Most classifications of bone tumours are

based on the recognition of the dominant
tissue in the various lesions
 knowing the cell line from which the tumour
has sprung may help with both diagnosis and
planning of treatment, however....
STAGING OF BONE TUMOURS

2 Conflicting principles:
 Lesion must be removed widely enough
 Damage must be kept to a minimum
The Balance depends
 How the tumour usually behaves (i.e how aggressive it is)
 How far it has spread
Aggressiveness

 Tumours are graded not only on their cytological characteristics, but...

 also on their clinical behaviour, i.e . the likelihood of recurrence and spread
after surgical removal
Benign Lesions

 the most aggressive are diftrcult to distinguish from a low grade sarcoma
and sometimes undergo malignant change  aggressive osteoblastoma
 Most are amenable to local (marginal) excision with little risk of
recurrence.
Malignant Tumours

 Divided into:
 Low-grade  moderately aggressive, long metastasize time e.g secondary
chondrosarcoma or parosteal osteosarcoma
 High-grade  very aggressive & metastasize early e.g osteosarcoma or
fibrosarcoma
SPREAD

 Local extent of the tumour  the most important factor  how much
tissue has to be removed
 Lesions that are confined to an enclosed tissue space (e.g a bone, a joint
cavity or a muscle group within its fascial envelope) 
INTRACOMPARTMENTAL
SPREAD

 extend into interfascial or extrafascial planes with no natural barrier to

proximal or distal spread (e.g. perivascular sheaths, pelvis, axilla) 
EXTRACOMPARTMENTAL
Surgical stage

 'Staging' the tumour is an important step towards selecting the operation

best suited to that particular patient, and carrying a low risk of recurrence.
 Locally recurrent sarcomas tend to be more aggressive, more often
extracompartmental and more likely to metastasize than the original
tumour.
Bone sarcomas are broadly divided as follows:
 Stage I : All low-grade sarcorma
 Stage II : Histologically high-grade lesions
 Stage III : sarcomas which have metastasized
Method of Treatment
Tumor Excision
 Intracapsular (intralesional) excision &
curettage
 Indication for benign lesions with a very low
risk recurrence or
 Incurable tumors which need debulking to
relieve local symptoms
 Adjunctive treatment such as the use of Acrylic
cement after curettage  risk of local
recurrence
Tumor Excision

 Marginal excision
 Just beyond the tumor
 Dissection of a malignant lesion
through the reactive zone 
significant risk of recurrence (up
to 50 %)
 Suitable method for benign
lesions.
 Resultingcavity can be filled with
bone graft
Tumor excision

 Wide excision
 Dissection well clear of the tumor through normal tissue
 Appropriate for low-grade intracompartmental lesions (grade IA)  risk of
recurrence below 10 %
 For grade IIA lesions in conjunction with chemotherapy
Tumor Excision

 Radical resection

the entire compartment in which the

tumour lies is removed en bloc without
exposing the lesion
Possible to perform sparing the limb, but
surrounding muscles, ligaments and
connective tissue will have to be
sacrifice
Radical resection

 In some cases  true radical resection achieved only by amputating at a

level above the compartment involved
 Require for high-grade tumor (IIA or IIB)
 Giant Cell Tumor
 Almost any kind of lesion in bone can contain
giant cells, sometimes numerous. In order to
qualify as a giant cell tumour, the neoplasm
has to have a combination of round to oval
mononuclear cells and more or less uniformly
distributed giant cells. Moreover, the nuclei of
the giant cells should be very similar to those
of the mononuclear cells.
Giant Cell Tumor
 Giant cell tumour is a benign, locally aggressive
neoplasm which is composed of sheets of
neoplastic ovoid mononuclear cells interspersed
with uniformly distributed large, osteoclastlike
giant cells.
Giant Cell Tumor
5 % of all primary bone tumor
 Lesion of uncertain origin appears in mature
bone
 Most commonly in the distal femur, proximal
tibia, proximal humerus and distal radius
 Extends right up to the subarticular bone
plate
 Mostly soliter
 Usually young adult
 Pain at the end of long bone slight swelling
 Palpable mass with warmth of the overlying
tissue
 history of trauma is not uncommon
 Pathological fracture occurs in 10-15 % of
cases
 X-ray
 Radioluscent area situated eccentrically at the end of a long
bone and bounded by the subchondral bone plate
 Endosteal margin may be quite obvious, but in aggressive
lesions it is ill-defined
 The centre sometimes has a soap-bubble appearance due to
ridging of the surrounding bone. The cortex is thin and
sometimes ballooned; aggressive lesions extend into the soft
tissue.
 The appearance of a 'cystic’ lesion in mature bone, extending
right up to the subchondral plate, is so characteristic that the
diagnosis is seldom in doubt
Campanacci
 gradingsystem for GCTs that is based on the
radiographic appearance of the tumors
 Stage I (10% lesion)
Normal contour of bone
Lesion is radioluscent, enlarging with sharply defined
margin
 Stage II (active lesion, 60% lesion)
Cortical erosion (+)
Deformity & expansion of bone
Limited endosteal
 Stage III (aggressive lesion, 30% lesions)
Destructive
Poorly outlined margin
Extend to subchondral bone or soft
tissue
Cortical breakthrough (+)
Pathologic fracture
 Check blood calcium, phosphate and alkaline
phosphatase to exclude Brown Tumor associated with
hyperparathyroidism
 staging procedures are essential  potential for
agressive behaviour
 CTscan & MRI  extent of the tumor (within the bone
or beyond? Whether the articular surface has been
breached?)
 Biopsy is essential
 Frozen section before operative treatment
 As a separate procedure
PATHOLOGY
 Gross: reddish, fleshy appearance, come away in
pieces quite easily when curetted but is difficult
to remove completely from the surrounding
bone.
 Aggressive lesions have a poorly defined edge &
extend well into surrounding bone
 Histologically the striking feature is an
abundance of multinucleated giant cells
scattered on a background of stromal cells with
little or no visible intercellular tissue.
Aggressive lesions tend to show more cellular
atypia and mitotic figures, but histological
grading is unreliable as a predictor of tumour
behaviour.
 Metastase in the lungs  rare!
 The tumor has the potential to transform
into an osteosarcoma
Treatment

 Well-confined, slow growing lesion with

benign histology  Curettage & stripping
of the cavity followed by swabbing with
hydrogen peroxide or liquid nitrogen and
then the cavity is packed with bone chips
 More aggressive tumors and recurrent
lesions  should be treated by excision
followed, if necessary, by bone grafting or
prosthetic replacement
OSTEOSARCOMA
 Characterizedby malignant cells that
produce osteoid matrix  spreading
rapidly outwards to the periosteum &
surrounding soft tissues
 20
% of all primary malignant bone tumor
 2nd most common after myeloma
 Classic
form  intramedullary
osteosarcoma
 Usually in age 4-25 y.o  if happen in patients >
50 y.o  secondary sarcoma arising in abnormal
bone : Paget’s disease
 May afffect any bone, most commonly involves
the long-bone metaphyses  especially around
the knee & proximal end of humerus
CLINICAL FEATURE

 Pain worse at night & gradually

increases in severity
 Restriction of function (joint movement or
deformity)
 Lump (+)  palpable mass with tenderness
 Pathological fracture may occur in 5-10%
cases
 Lab  ESR & Alkaline Phosphatase
X-RAY

 Radioluscent area with hazy

ostelytic mixed with
osteoblastic lesions
 Endosteal margin is poorly
defined
 X-RAY
 If the cortex breached  tumor extend to adjacent
tissues  streak of new bone (+) radiating outwars
from cortex : Sunburst effect
 When the tumors emerges from the cortex 
reactive new bone forms at the angles of periosteal
elevation  Codman’s triangle
 Both may occasionally be seen in other rapidly
growing tumors
DIAGNOSIS
 Diagnosis
can be made with confidence on the x-ray
appearances  most cases
 Must differentiate with atypical lesions 
post-traumatic swelling
Infection
Stress fracture
Aggressive cystic lesions
 Staging :
Chest x-ray  lung metastase (pulmonary CT
more sensitive)
MRI : best method for local staging of
intraosseous & extraosseus tumor extent
Radioisotope scan : show up skip lesions
 10% of patients have pulmonary metastases by the
time they are first seen
 Biopsymust be carefully planed to allow
for complete removal of the tract when
the tumors is excised  Before treatment
begin
PATHOLOGY
 Generally large (> 5 cm) lesions
 Tumors destroys and replaces normal bone,
extends within the medulla and across the
pyseal plate
 Spread into soft tissues with
ossification at the periosteal margin
 Streaks of new bone extending into
the extraosseus mass
HISTOPATHOLOGY
 Malignantstromal
tissue showing osteoid
formation
 Chondroblastic
differentiation
 Thetumor cells can be
spindle, epitheloid , or
small & round
 TREATMENT

 Betterdiagnostic &
staging procedures
 Average age of the Prognosis of survival rate markedly
IMPROVED
patients increase
 Advancesin
chemotherapy 
control metastatic
spread
TREATMENT
 Important to eradicate the primary lesion
completely  lower local reccurence 
lower the mortality rate
 clinical
assessment + advanced imaging +
biopsy  diagnosis & staging (+)
• multiagent neoadjuvant for 8-12 weeks

• the tumor is resectable and there are no skip

lesions

• Wide resection
• Replace segment of bone with large bone graft
or custom made implant

• Limb salvage or Amputation

 Specimen is examined  assess the
response to preoperative chemotherapy
 Iftumor necrosis is marked (more than
90%)  chemotherapy is continued for
another 6-12 months
 If the response is poor  different
chemotherapic regimen
 Methotrexate, Adriamyvin, Cisplatin,
Iphosphamide
 Selection
of patients for limb salvage or
Amputation based on:
Extent of local disease
Present of pathological fracture
 patient’s age & growth potential
Outcome
 Long-term survival after wide resection &
chemotherapy  50 % in 1980; over 60 %
in recent years
 Limb salvage rate at 20 years : 84 %
 Tumor-replacement implant usually
function well with fairly high complication
rate (wound breakdown & infection)
Variants Of Osteosarcoma

 Parosteal osteosarcoma
 Periosteal osteosarcoma
 Paget’s osteosarcoma
Parosteal Osteosarcoma

 Low-grade malignant lesion

 Situatedon the surface of tubular bones 
distal femoral or proximal tibia metaphysis
 Usually young adult with Slow-growing
lesion near the bone end
 Painless
 Dense bony mass (sclerotic) on the
surface of the bone 
juxtacortical
 Cortex not eroded
 Thin gap remains between cortex
and tumor
 Easily mistaken as a benign bone
lesion
Periosteal Osteosarcoma

 More like an classic osteosarcoma, but

situated on the surface of the bone
 Occurs in young adult
 Symptom: local pain & swelling
PAGET’S SARCOMA

 Mostosteosarcoma appearing after 50

years  fall into this category
 Malignant transformation  rare
 Warning signs:
Pain
Swelling
Pathological fracture