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
Parosteal Site of the lesion.
osteosarcoma Adamantinoma
Chondroblastoma
 Site of the lesion.
Distribution of various lesions Distribution of various lesions in a
in a long tubular bone in a long tubular bone after skeletal
growing skeleton 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 vertebra.
Malignant lesions are seen Benign lesions predominate in its
predominantly in its anterior posterior elements.
part (body)
 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 transition
typifies a benign lesion e.g typifies an aggressive or
nonossifying fibroma in the malignant lesion e.g
distal femur. 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 permeative type
moth-eaten
a uniformly affected characteristic of
rapidly growing round cell tumors
area within sharply infiltrating lesions
defined borders

giant cell tumor. myeloma 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 long-standing (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

lamellated or Ewing sarcoma -

sunburst onion-skin lamellated type
pattern - type in ewing
sarcoma
osteosarcoma

Codman
triangle
(arrow)
 Type of Matrix
The matrix represents the intercellular material produced by
mesenchymal cells
E.g osteoid, bone, chondroid, myxoid, and collagen material .
Type of matrix allows differentiation of some similar-appearing
E.g differentiating osteoblastic from chondroblastic processes.
Calcifications in the tumor matrix, point to a chondroblastic
process.
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
B. presence of the
densities within the
wisps of tumor-bone
medullary cavity, e.g
formation, like in this
in this case of
case of osteosarcoma
osteosarcoma of the
distal femur 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 C: Chondrosarcoma
displays a typical with characteristic
chondroid matrix 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
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.
Malignant tumors often
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
Cellularity
Nuclear features
Mitotic figures
necrosis
Correlates with prognosis in some tumors
E.g chondrosarcoma, malig vascular tumors
Some not amenable to histological grading e.g monomorphic tumors
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 procedures
Enneking classification of amputations
 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

Skipmets -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 biopsies
Needle biopsy track Multiple needle tracks
contaminated patellar tendon contaminate quadriceps 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 biopsy
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 biopsies
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.
Ifa 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
Discusswith the pathologist about clinical findings,
imaging, intraop findings and the specimen
 Example of poorly performed biopsy
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 Cystic lesions
Osteoid osteoma Unicameral bone cyst

Bone island Aneurysmal bone cyst

Intraosseous ganglion cyst

Cartilage lesions
Epidermoid cyst
Chondroma
Fatty tumors
Osteochondroma
Lipoma

Fibrous lesions Vascular tumors

Nonossifying fibroma Hemangioma

Cortical desmoid Other nonneoplastic lesions

Benign fibrous histiocytoma Paget disease

Fibrous dysplasia Brown tumor-hyperparathyroidism

Osteofibrous dysplasia Bone infarct

Desmoplastic fibroma 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 synrome
CARTILAGE LESIONS
Osteochondroma
 Fibrous lesions
Nonossifying fibroma

Fibrous dysplasia Polyostotic Fibrous dyspalsia

Shepherds crook
appearance
 Cystic lesions
Unicameral bone cyst
Aneurysmal bone cyst
 Aggressive benign tumors
Chondroblastoma
Giant cell tumor
 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

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