PRIMARY BONE

TUMORS
PRESENTER: ONDARI N.J
FACILITATOR: PROF. GAKUU

28-10-2013

Outline

Introduction
Classification
Epidemiology
Evaluation
Staging
Principles of management
Selected tumors
Therapeautic advances

Introduction

Forms 0.2% of human tumor burden

Primary malig bone tumors make 1% of

all malignant tumors

Carcinoma commonly metastasize to

LN except BCC

Sarcomas commonly metastasize

hematogenously

Most have male predominance excep

GCT, ABC

Classification

Based on tissue of origin

Bone
Cartilage
Fibrous tissue
Bone marrow
Blood vessels
Mixed
Uncertain origin

Evaluation

History

Physical examination

Investigations; labs, imaging

Biopsy

Analytic approach to evaluation

of the bone neoplasm

Evaluation; history

Age

Symptomatology
Pain
Swelling
History of trauma
Neurological sympts

Pathological fracture

Evaluation; physical
examination

Lump/swelling
5S MTC

Effusion

Deformities

Regional nodes

Evaluation; imaging

Plain radiograph

CT scan

MRI

Radionuclide scanning

PET

Radiography

Information yielded by radiography

includes :
Site of the Lesion

Borders of the lesion/zone of transition

Type of bone destruction

Periosteal reaction

Matrix of the lesion

Nature and extent of soft tissue

involvement

Radiographic features of bone tumors

Site of the Lesion

Determined by the laws of field behavior and

developmental anatomy of the affected bone, a
concept first popularized by Johnson.

Parosteal osteosarcoma -posterior aspect of the distal femur

Chondroblastoma -epiphysis of long bones before skeletal
maturity
Adamantinoma and osteofibrous dysplasia have a specific
predilection for the tibia
A lesion's location can also exclude certain entities from the
differential diagnosis.
E.g Giant cell tumor -articular end of bone.

Location in relation to the central axis of the bone esp

in long tubular bone, such as humerus, radius, femur,
or tibia.

For example, simple bone cyst, enchondroma, or a focus of

fibrous dysplasia -always centrally located
Eccentric location is Xteristically observed in aneurysmal
bone cyst, chondromyxoid fibroma, and nonossifying fibroma

Predilection of Tumors for Specific Sites

in the Skeleton

Site
of
the
lesion.
Parosteal
osteosarc
oma

Adamantino
Chondroblas ma
toma

Site of the lesion.

Distribution of various
lesions in a long
tubular bone in a
growing skeleton

Distribution of various
lesions in a long tubular
bone after skeletal
maturity

Site of the lesion.

Location of epicenter of lesion usually

determines site of its origin (medullary, cortical,
periosteal, soft tissue, or in the joint)

Distribution of various lesions in a ver

Malignant lesions are
seen predominantly in
its anterior part (body)

Benign lesions
predominate in its
posterior elements.

Borders/margins of the
Lesion

Margins determined by GRate hence benign or

malignant
Three types of lesion margins are encountered:

Sharp demarcation by sclerosis (IA margin),

sharp demarcation without sclerosis (IB margin)
Ill-defined margin (IC margin)

Slow-growing lesions -sharp sclerotic borders;

usually indicates that a tumor is benign

E.g nonossifying fibroma, simple bone cyst

Indistinct borders- typical of malignant or

aggressive lesions

Post- Radio- or chemo of malignant bone tumors

Can exhibit sclerosis and a narrow zone of transition

Borders of the lesion

determine its growth rate.
sharp sclerotic

sharp lytic

ill-defined.

Borders
of
the
lesion.
A: Sclerotic border
B: A wide zone of
typifies a benign
lesion e.g
nonossifying fibroma
in the distal femur.

transition typifies an
aggressive or malignant
lesion e.g plasmacytoma
involving the pubic bone
and supraacetabular
portion of the right ilium

Type of Bone Destruction

Mechanisms of bone destruction

Direct effect of tumor cells
Incr osteoclastic activity

Cortical bone is destroyed less rapidly than trabecular

bone.
Loss of cortical bone appears earlier on radiography
trabecular bone must be destroyed (about 70% loss of
mineral content) before the loss becomes
radiographically evident

Bone destruction can be described as

geographic (type I) - benign lesions
moth-eaten (type II) and
permeative (type III) - rapidly growing infiltrating tumors

Patterns of bone destruction.

geographic

moth-eaten

a uniformly affected
area within sharply
defined borders

rapidly growing
infiltrating lesions

giant cell tumor.

myeloma

permeative type
characteristic of
round cell tumors

Ewing sarcoma

Periosteal Response

the pattern of periosteal reaction is an indicator of the

biologic activity of a lesion .
periosteal reactionsthat can be categorized as;

uninterrupted (continuous) or I
nterrupted (discontinuous).

Any widening and irregularity of bone contour may represent

periosteal activity.

An uninterrupted periosteal reaction indicates a longstanding (slow-growing), usually indolent, benign process.
There are several types of solid periosteal reaction:

a solid buttress e.g aneurysmal bone cyst and chondromyxoid

fibroma;
a solid smooth or elliptical layer e.gosteoid osteoma and
osteoblastoma;
a single lamellar reaction, such as accompanies Langerhans cell
histiocytosis

Sunburst (hair-on-end) or onion-skin (lamellated) pattern .

Codman triangle

Types of periosteal reaction.

An uninterrupted periosteal reaction usually indicates a benign
process, whereas an interrupted reaction indicates a malignant
or aggressive nonmalignant process

Examples of Nonneoplastic and Neoplastic

Processes Categorized by Type of Periosteal
Reaction

Interrupted type of periosteal reaction

sunburst
pattern
-osteosarcom
a

lamellated or
onion-skin
type in ewing
sarcoma

Ewing sarcoma
-lamellated type

Codman
triangle
(arrow)

Type of Matrix

The matrix represents the intercellular material produced

by mesenchymal cells

Type of matrix allows differentiation of some similarappearing

E.g differentiating osteoblastic from chondroblastic processes.

Calcifications in the tumor matrix, point to a

chondroblastic process.

E.g osteoid, bone, chondroid, myxoid, and collagen material .

Calcifications typically appear as punctate (stippled), irregularly

shaped (flocculent), or curvilinear (annular or comma-shaped,
rings and arcs).
Differential diagnosis of stippled, flocculent, or ring-and-arc
calcifications includes enchondroma, chondroblastoma, and
chondrosarcoma.

A completely radiolucent lesion may be either

fibrous or cartilaginous in origin

tumor-like lesions, such as simple bone cysts or intraosseous
ganglion

Types of matrix: osteoblastic

The matrix of a typical osteoblastic lesion is characterized by
the presence of the following features
A. fluffy, cotton-like
densities within the
medullary cavity, e.g
in this case of
osteosarcoma of the
distal femur

B. presence of the
wisps of tumor-bone
formation, like in this
case of osteosarcoma
of the sacrum

C. by the presence of
a solid sclerotic
mass, such as in
parosteal
osteosarcoma

Types of matrix: chondroid matrix

A: Schematic representation of various
appearances of chondroid matrix calcifications.

B: Enchondroma
displays a typical
chondroid matrix

C: Chondrosarcoma
with characteristic
chondroid matrix

Soft Tissue Mass

A bone lesion associated with a soft

tissue mass should prompt the
question of which came first.

Is the soft tissue lesion an

extension of a primary bone tumor,
or is it a primary soft tissue tumor
invading bone?

Radiographic features differentiating primary soft tissue tumor

invading bone from primary bone tumor invading soft tissues.

Benign Versus Malignant

Nature

clusters of features that can be gathered from

radiographs can help in favoring one designation
over the other .
Benign lesions usually have

Malignant tumors often

well-defined sclerotic borders

exhibit a geographic type of bone destruction
the periosteal reaction is solid and uninterrupted, and
there is no soft tissue mass.
exhibit poorly defined borders with a wide zone of
transition;
bone destruction appears in a moth-eaten or permeative
pattern, and
the periosteum shows an interrupted, sunburst, or
onion-skin reaction with an adjacent soft tissue mass.

NB-benign lesions may also exhibit aggressive

features

Radiographic features that may help

differentiate benign from malignant
lesions

Grading of bone sarcomas

Criteria for grading

Correlates with prognosis in some tumors

E.g chondrosarcoma, malig vascular tumors

Some not amenable to histological grading e.g

monomorphic tumors

Cellularity
Nuclear features
Mitotic figures
necrosis

Ewing, MM, lymphoma

Some always high grade

Sometimes not useful in predicting prognosis

Adamantinoma, chordoma

Staging of bone tumors

Benign tumors (Enneking staging of benign

tumors)

Stage 1 - latent
Stage 2 - active
Stage 3 - aggressive

Malignant tumors

TNM staging
AJCC staging system
Musculoskeletal tumor society staging
system(enneking)

Surgical staging

Note

Benign tumors - classified using Arabic

numerals(1,2,3)
Malignant tumors - classified using roman
numerals(I,II,III)

William F. Enneking M.D

Enneking classification
systems

Enneking classification of benign

tumors

Latent, active, aggressive

Enneking surgical staging of malignant

tumors

Enneking classification of local

procedures

Intracapsular, marginal, extended, radical

Enneking classification of amputations

Intracapsular, marginal, extended, radical

Enneking classification of local proce

Enneking classification of amputatio

Enneking staging of benign

tumors

Stage 1; Latent

Well defined margin

Grows slowly and then stops

Heals spontaneously eg osteoid osteoma

Neglible recurrence after intracapsular resection

Stage 2; Active

Progressive growth limited by natural barriers

Well defined margin but may expand thinning cortex e.g ABC

Negligible recurrence after marginal excision

Rx marginal resection

Stage 3; aggressive

Growth not limited by natural barriers e.g GCT

Mets present in 5% of these pts

Have high recurrence after intracapsular or marginal

resection

Extended resection preferred

Enneking surgical Staging of

malignant tumors
Incorporates

degree of differentiation
Low grade(stage I) or
High grade(stage II)

Local extent of tumor

Intracompartmental - A
Extracompartmental - B

distant spread
metastasis

Enneking surgical Staging of

malignant tumors

AJCC staging for bone

sarcomas

Based on

Tumor grade
Low

grade(I)
High grade(II)

Tumor size
<8cm

-A
>8cm -B

Presence and location of mets

Skip

mets -III
Pulm mets -IVA
Non-pulm mets -IVB

Bone biopsy

Options

Needle biopsy
90% accuracy at determining malignancy
Accuracy at determining specific tumor much lower
Absence of malignant cells less re-assuring than
incisional biopsy

Core biopsy

Provides accurate diagnosis in 90% of cases

incisional biopsy

Primary resection instead of biopsy can be done

in;

Small(<3cm) subc mass- marginally resected if

likely malignant
Characteristic radiographic appearance of benign
lesion
Painful lesion in an expendable bone e.g prox
fibula, distal ulna

Tumour Biopsy Principles

1
1.Biopsy done only after evaluation & imaging is complete.

determine xteristics and local extent of the tumor and mets

Staging helps determine the exact anatomic approach to tumor
Biopsy superimposes radiologic changes at the biopsy site, and
there4 can alter the interpretation of the imaging studies.

2. Place small incisions whenever possible- skin & capsule

3. The biopsy track be considered contaminated with tumor
cells.
Track excised en bloc with the tumor subsequently.
4. The surgeon should be familiar with incisions for limb
salvage surgery, and also with standard and nonstandard
amputation flaps.

Examples of poorly performed biopsi

Needle biopsy track
Multiple needle tracks
contaminated patellar contaminate quadriceps
tendon
tendon

Needle track placed

posteriorly, location
that would be
extremely difficult to
resect en bloc with
tumor if it had
proved to be
sarcoma.

Tumour Biopsy Principles

2
5. If a tourniquet is used;

The limb is elevated before inflation

Avoid exsanguination by compression.

6. contaminate as little tissue as possible.

Avoid transverse incisions

The deep incision should go thru single muscle
compartment (muscle belly) rather than
through an intermuscular plane.
Major neurovascular structures should be
avoided.
Care should be taken not to contaminate flaps.
Minimal retraction should be utilized to limit
soft tissue contamination.

Example of poorly performed bi

Transverse incisions should not be used

Tumour Biopsy Principles

3
7. If possible soft tissue extension of a bone lesion
should be sampled
8. If a hole must be made in the bone, it should be
round or longitudinally oval to minimize stress
concentration and prevent a subsequent fracture.
A fracture may preclude a subsequent limb salvage
surgery.
PMMA is plugged into the hole to contain a
hematoma - minimal.

9. Biopsy should be taken from the periphery of the

lesion, which contains the most viable tissue.

Biopsy material may be sent for M/C/S if in doubt

regarding infection

If hole must be made in bone during biopsy,

defect should be round to minimize stress
concentration, which could lead to
pathological fracture

Examples of poorly performed biop

Biopsy resulted in irregular
defect in bone, which led to
pathological fracture

Tumour Biopsy Principles

4

10. A frozen section should be sent intraop to

ensure that diagnostic tissue has been
obtained.

If a tourniquet has been used it should be deflated

and meticulous haemostasis ensured before
closure.

11. Drains should not be used routinely.

If a drain is used, it should exit in line with the

incision.
The wound should be closed tightly in layers.

12. operating surgeon should accompany

specimen to pathologist if feasible

Discuss with the pathologist about clinical findings,

imaging, intraop findings and the specimen

Example of poorly performed biops

Drain site was not
placed in line with
incision

Principles of
management

Multidisciplinary team approach

Benign asymptomatic tumors
If certain observe
If in doubt biopsy
Benign symptomatic or enlarging tumors
Biopsy
Excision/ curretage
Suspected malignant tumors
If primary admit for work-up
Staging
Choices; amputation, limb sparing surgery,
adjuvant therapy

Benign tumors - not

aggressive
Bone-forming tumors
Osteoid osteoma
Bone island
Cartilage lesions
Chondroma
Osteochondroma
Fibrous lesions
Nonossifying fibroma
Cortical desmoid
Benign fibrous histiocytoma
Fibrous dysplasia
Osteofibrous dysplasia
Desmoplastic fibroma

Cystic lesions
Unicameral bone cyst
Aneurysmal bone cyst
Intraosseous ganglion cyst
Epidermoid cyst

Fatty tumors
Lipoma

Vascular tumors
Hemangioma

Other nonneoplastic lesions

Paget disease
Brown tumorhyperparathyroidism
Bone infarct
Osteomyelitis

Aggressive benign
tumors

Giant cell tumor

Chondroblastoma

Chondromyxoid fibroma

Osteoblastoma

Langerhans cell histiocytosis

Osteoid Osteoma

Bone Island

CARTILAGE LESIONS
Chondroma

Enchondroma

Olliers disease

Maffuci synrom

CARTILAGE LESIONS
Osteochondroma

Fibrous lesions
Nonossifying fibroma

Fibrous dysplasiaPolyostotic Fibrous dysp

Shepherds crook
appearance

Cystic lesions
Unicameral bone cyst

Aneurysmal
bone cyst

Aggressive benign tumors

Giant cell tumor

Chondroblastoma

Aggressive benign tumors

Chondromyxoid fibroma

Malignant Tumors of
Bone

Osteosarcoma
Chondrosarcoma
Ewing sarcoma
Chordoma
Adamantinoma
Malignant vascular tumors
Malignant fibrous histiocytoma and
fibrosarcoma
Multiple myeloma and plasmacytoma
Lymphoma
Metastatic carcinoma

Osteosarcoma

Chondrosarcoma

Ewing Sarcoma
may be confused with osteomyelitis
Commonly affects diaphysis with onion
skin appearance

Adamantinoma
Bubble-like appearance
85% occur in tibia

The end
Thank you