BONE TUMOURS

Natasha Eleena
Nor Maghfirah
Hani Farhana
Nur Fadhila
CLASSIFICATIONS OF BONE
TUMORS
 Classifications
A. Tissue types
B. Benign vs Malignant
C. Age of patient
D. Location of lesion
 A. TISSUE TYPES
Predominant Tissue Benign Malignant
Bone forming Osteoma Osteosarcoma:
Osteoid osteoma • Central
Osteoblastoma • Peripheral
• Parosteal
Cartilage forming Enchondroma Chondrosarcoma:
Osteochondroma • Central • Juxtacortical
• Peripheral • Clear-cell
• Mesenchymal
Fibrous tissue Fibroma Fibrosarcoma
Fibromatosis
Miscellaneous Giant cell tumor Ewing’s sarcoma
Vascular tissue Hemangioma Angiosarcoma
Hemangiopericytoma Malignant hemangiopericytoma
Hemangioendothelioma
 B. BENIGN VS MALIGNANT
BENIGN
• Well circumscribed
• No periosteal reaction
• Sclerotic border
• Small zone of transition : sharp, ill
defined border, sign of slow growth
MALIGNANT
• Gross periosteal reaction
• Large, permeative, moth eaten
• Metastasis
• Wide zone of transition : ill-defined
border, sign of aggressive growth
C. AGE OF PATIENT
• ABC : Aneurysmal bone cyst
• CMF : Chondromyxoid
fibroma
• EG : Eosinophilic granuloma
• GCT : Giant cell tumour
• FD : Fibrous dysplasia
• HPT : Hyperparathyroidism
with Brown tumour
• NOF : Non Ossifying Fibroma
• SBC : simple Bone Cyst
D. LOCATION
OF LESION
 D. LOCATION OF LESION
1. Transverse plane
 Central – Enchondroma
 Eccentric – Giant cell tumor,
Osteosarcoma, Chondromyxoid fibroma
 Cortical – Non ossifying fibroma, Osteoid
osteoma
 Parosteal – Parasteal osteosarcoma,
Osteochondroma

2. Longitudinal plane
 Diaphyseal – Ewing’s, Osteoid osteoma,
Mets, Adamantinoma, Fibrous Dysplasia
 Epiphyseal – Chondroblastoma, Giant cell
tumor, Ganglion of bone
 Metaphyseal – EVERYTHING!!!
INVESTIGATION IN GENERAL
 General Investigations
• Imaging
Plain X-ray, CT scan, MRI, Radionuclide
scanning

• Blood tests

• Biopsy
Plain X-rays

 Most useful of all imaging techniques

 Obvious abnormality:
 A discrete lump
 Bone destruction
 Cortical thickening
 Cyst-like structures

 Site of lesion:
 solitary/multiple
 well-defined/ill-defined

 Bone surfaces: Periosteal new bone formation

 Soft tissues: Swellings and calcifications
CT scan

 Shows more accurately intraosseous and extraosseous

extension of tumor and the relationship to
surrounding structures

 Reliable method of detecting pulmonary metastases

Magnetic Resonance Imaging (MRI)

 Greatest value in assessment of tumor spread:

 within the bone
 nearby joints
 soft tissues

 Useful in assessing soft-tissue tumors and

cartilaginous lesions.
Radionuclide scanning with 99mTc-HDP

 Shows non-specific reactive changes in the bone

 Helpful in revealing site of a small tumor or
presence of skip lesions or silent secondary
deposits
Blood tests

 Full blood count (Anemia)

 Serum protein electrophoresis: Abnormal globulin
fraction containing Bence Jones protein in patients
with myeloma
 Raised serum acid phosphatase: Prostatic ca.
 ESR and Serum calcium may be raised
PRINCIPLE OF MANAGEMENT
 Principles of Mx
• Multidisciplinary team approach
• Tumor Excision
– Intracapsular (intralesional)
excision and curretage
– Marginal excision
– Wide excision
– Radical excision
• Limb Salvage
• Amputation
• Multi-agent chemotherapy
• Radiotherapy
 Principle of management
Benign tumors
I. Observe, biopsy , excision

Suspected malignant tumours, the patient need to

be admitted for detailed assessment in order to :
II. Confirm the diagnosis.
III. Establish grade of malignancy
IV. Define precisely how far the tumour has spread
OSTEOSARCOMA
Osteosarcoma is a highly malignant tumour
arising within the bone and spreading rapidly
outwards to the periosteum and surrounding
soft tissues.

commonly involves the long-bone metaphyses:

• knee
• proximal end of the humerus
 Primary Secondary
Osteosarcoma: Osteosarcoma

Seen above 45
Commoner
yrs of age

15-25 yrs of May be

age associated
with Pagets
disease,
No known fibrous
premalignant dysplasia,
conditions irradiation, etc

More
malignant
than
secondary
 Features
i. Pain (first symptom)
 constant, worse at night and gradually increases in severity.

ii. Presents with a lump.

iii. Pathological fracture (rare).

On examination:
iv. Tenderness
v. Palpable mass (later case)
vi. Overlying tissues may appear swollen and inflamed.
vii. prominent veins.
viii.Movements at the joint may be affected

The ESR is usually raised and there may be

an increase in serum alkaline phosphatase.
 Imaging
Xray:
Irregular destruction of metaphysis.
Cortex is eroded.
New bone formation in the matrix of tumour.
Periosteal reaction

“Sunray appearance”: Tumour grows into soft tissues. New bone is laid
along the blood vessels in the tumour, giving a sun ray appearance

Codman’s triangle: Triangular area of subperiosteal new bone at the

ends of the tumour
(a) The metaphyseal site; increased density, cortical erosion and periosteal reaction
are characteristic.
(b) Sunray spicules and Codman’s triangle;
(c) the same patient after radiotherapy.
(d) A predominantly osteolytic tumour.
 Diagnosis and staging
Diagnosis can be made with confidence on the x-ray
appearances

Conditions to be excluded are post-traumatic swellings,

infection, stress fracture and the more aggressive ‘cystic’ lesions

Evaluate spread: Commonest metastasis is to lung. So chest

Xray or CT is required.
Bone scans to detect skip lesions.
CT and MRI to determine soft tissue spread
 Confirm: Biopsy

The dominant features in the histological The same tumour showed areas of
sections were malignant stromal tissue Chondroblastic differentiation.
showing osteoid formation (pink
masses).
 Treatment
Amputation with wide margin

Limb salvage: Done in early cases. Radical excision. Bone

defects managed with grafts or prosthesis and chemotherapy

Chemotherapy: Pre or post operative. Methotrexate,

Cisplatinum, Endoxan, etc are used with various protocols

Radiotherapy: In surgically inaccessible areas or patients

refusing surgery
Pulmonary metastases, especially if they are
small and peripherally situated, may be
completely resected with a wedge of lung tissue.

Postoperative x-rays showing an endoprosthetic replacement following wide

resection of the lesion (Stanmore Implants Worldwide).
GIANT CELL TUMOR
 Giant cell tumor / osteoclastoma
Represents 5 per cent of all primary bone tumours,
lesion of uncertain origin

Generally classified as benign but can recur after removal

20-40 yrs of age

Appears in mature bone, most commonly:

• distal femur,
• proximal tibia,
• proximal humerus
• distal radius.

characteristically it extends right up to the subarticular bone plate.

 Features
 The patient is usually a young adult
 Pain at the end of a long bone
 Sometimes there is slight swelling.
 A history of trauma is not uncommon
 Pathological fracture occurs in 10–15 per cent of
 cases.

On examination:
• Palpable mass
• Warmth of the overlying tissues
 Imaging
X-ray
Radiolucent area situated eccentrically at the end of a long bone and bounded by the
subchondral bone plate.

The 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.
 Multiloculated (30%) Pure lytic (70%)

Giant-cell tumours The tumour always abuts

against the joint margin.
CT scans and MRI
• Reveal the extent of the tumour
• Detailed staging

Biopsy:
Spindle cells with multinucleated giant cells. (These
giant cells used to be mistaken for osteoclasts)

The tumour has the potential to transform into an

osteosarcoma.
A low-power view of the biopsy shows the abundant multinucleated giant cells lying in
a stroma composed of round and polyhedral tumour cells. There are numerous mitotic
figures
It is prudent to obtain estimations of
• blood calcium,
• phosphate
• alkaline phosphatase concentrations

so as exclude an unusual ‘brown tumour’

associated with hyperparathyroidism.
 Treatment
Well-confined, slow-growing lesions with benign
histology
Thorough curettage and ‘stripping’ of the cavity
with burrs and gouges, followed by swabbing with
hydrogen peroxide or by the application of liquid
nitrogen; the cavity is then packed with bone
chips.
More aggressive tumours, and recurrent lesions,
excision followed by bone grafting or
Prosthetic replacement.

In sites like spine, radiotherapy may be done

Excision and bone grafts.

EWING’S SARCOMA
 INTRODUCTION
• Identified by James Ewing
• Arising from endothelial cells of bone marrow
• Age: Most commonly between the ages of 10
and 20 years
 SKELETAL DISTRIBUTION
• Any bone can be affected
• Usually in a tubular bone and especially in the
tibia, fibula or clavicle
• Most common diaphysis > metaphysis ; rarely
in epiphysis
 Clinical features
• The patient presents with:
• Pain – often throbbing in character
• Generalized illness and pyrexia, together with
a warm, tender swelling
 Diagnostic approach
• Lab investigations
– Hb reduced
– Leucocytosis
– Increased ESR
– Aspirate may grossly resemble pus
 IMAGING
• X-RAY

– Diaphyseal lesion with irregular destruction (Moth eaten

appearance)

– Periosteal new bone formation (Onion peel appearance)

– Often the tumour extends into the surrounding soft

tissues, with radiating streaks of ossification and reactive
periosteal bone at the proximal and distal margins. These
features (the sunray’ appearance and Codman’s triangles)
but they are just as common in Ewing’s sarcoma.
Ewing’s tumour Examples
of Ewing’s tumour in
(a) the humerus, (b) the
mid-shaft of the fibula
and
(c) the lower end of the
fibula.
• Bone scan
– may shows heavy radioisotope uptake in tumor area

• MRI
– necessary to identify soft-tissue extension and marrow involvement
– often shows a large extraosseous component 

• CT chest 
– is required for appropriate staging to look for pulmonary metastasis

• Bone marrow biopsy 

– Required as part of workup for Ewing's to rule out metastasis to the
marrow 
• Gross appearance
– Soft, gray white , ocassionally shiny
– Areas of haemorrhage and necrosis
– Cortex may be partially or completely destroyed
• Histology :

Ewing’s tumour – histology :

There is a monotonous pattern of small round cells clustered around
blood vessels
 TREATMENT
• The prognosis is always poor and surgery
alone does little to improve it
• Radiotherapy has a dramatic effect on the
tumour but overall survival is not much
enhanced
• Chemotherapy is much more effective,
offering a 5-year survival rate of about 50 per
cent
(Souhami and Craft, 1988; Damron et al., 2007).
• The best results are achieved by a combination of all three
methods:

• A course of preoperative neoadjuvant chemotherapy; then

wide excision if the tumour is in a favourable site, or
radiotherapy followed by local excision if it is less accessible;
and then a further course of chemotherapy for 1 year

• Postoperative radiotherapy may be added if the resected

specimen is found not to have a sufficiently wide margin of
normal tissue
OSTEOCHONDROMA
(CARTILAGE-CAPPED EXOSTOSIS)
 OSTEOCHONDROMA
• A developmental lesion;

→ starts as a small overgrowth of cartilage at the edge of the physeal plate

→ develops by endochondral ossification into a bony protuberance still
covered by the cap of cartilage.
• Any bone that develops in cartilage may be
involved; the commonest sites are:
the fast-growing ends of long bones
the crest of the ilium

• The patient is usually a teenager or young adult

when the lump is first discovered
Sign and symptoms:

• Occasionally there is pain (due to an overlying

bursa or impingement on soft tissues)
• Rarely, paraesthesia (due to stretching of an
adjacent nerve)
• In long bones, growth leaves the bump stranded
further down the metaphysis.
– bump may go on growing but will stop enlarging at
the end of the normal growth period for that bone
– Any further enlargement after the end of the growth
period is suggestive of malignant transformation.

• Multiple lesions may develop as part of a heritable

disorder – hereditary multiple exostosis 
 Pathology
• At operation the cartilage cap is seen placed on top of
a narrow base or pedicle of bone.

• The cap consists of simple hyaline cartilage; in a

growing exotosis the deeper cartilage cells are
arranged in columns, living rise to the formation of
endochondral new bone.

• Large lesions may have a ‘cauliflower’ appearance,

with degeneration and calcification in the centre of the
cartilage cap.
 X-ray:
• There is a well-defined exostosis
emerging from the metaphysis, its base
coextensive with the parent bone.

• It looks smaller than it feels because

the cartilage cap is usually invisible on x-
ray.

• However, large lesions undergo 

cartilage degeneration and calcification
→ x-ray shows the bony exostosis
surrounded by clouds of calcified
material. (speckling/ popcorn
appearance)
 TREATMENT
• If the tumor causes symptoms it should be excised

• If in an adult, it has recently become bigger or painful, then

operation is urgent because it suggest malignancy

– This is seen most often with pelvic exostoses

– If there are suspicious features, further imaging and staging

should be carried out before doing a biopsy

– If the histology is benign but it keeps on enlarging after end of

growth period → should be treated as a chondrosarcoma.
 Complication

• The incidence of malignant transformation is difficult to assess

because troublesome lesions are so often removed before they
show histological features of malignancy.

• Features suggestive of malignant change are:

1. Enlargement of the cartilage cap in successive examinations
2. A bulky cartilage cap (more than 1 cm in thickness)
3. Irregularly scattered flecks of calcification within the
cartilage cap
4. Spread into the surrounding soft tissues. MRI may be
needed to reveal these changes.
OSTEOID
OSTEOMA
 OSTEOID OSTEOMA

• It is a tiny bone tumor

• Usually < 1cm in diameter
• Usually less than 30 years old
• Male is common
• Any bone can be affected especially femur or tibia
• But not in skull
 OSTEOID OSTEOMA

• CLINICAL PRESENTATION:
 Persistence pain
can be localized or refer over wide area
dull, aching pain that is moderate in intensity but can worsen and become severe—
especially at nigh
not usually activity-related
Pain relieve by salicylates
 Late presentation
Limping
Muscle wasting and weakness
In spinal lesion, can cause intense pain, muscle spasm and scoliosis
 OSTEOID OSTEOMA

• XRAY:
Small radiolucent area- nidus
Lesion is in diaphysis are surrounded by dense sclerosis and
cortical thickening
Can see the nidus clearly in CT scan
Lesion in metaphysis show less cortical thickening
Further away, the bone may be osteoporotic
 OSTEOID OSTEOMA

• Difficult to distinguish an osteoid osteoma from a small Brodie’s

abscess withoud biopsy
• We also need to exclude Ewing’s sarcoma and chronic periostitis
 OSTEOID OSTEOMA

• PATHOLOGY:
Excised lesion appears as a dark-brown or reddish ‘nucleus’
surrounded by dense bone
Central area consists of unorganized sheets of osteoid and bone
cells
No risk of malignant transformation
• TREATMENT:
oMost will disappear on their own over several years. Over-the-
counter NSAIDs, such as aspirin, ibuprofen, and naproxen will provide
pain relief
oComplete removal or destruction of the nidus
oThe lesion is carefully localized by Xray and/or CT scan and then
excised in a small block of bone
oCan also be destroyed by CT scan localized radio-ablation
oIf the excision can weaken the host bone, prophylactic internal
fixation may be needed
ENCHONDROMA/
CHONDROMA
 ENCHONDROMA

• Islands of cartilage may persist in the metaphysis

of bones formed by enchondromal ossification
• Some of them can grows and take on the
characteristics of a benign tumor
• Usually asymptomatic
• Accidental finding on Xray or after pathologic
fracture
 ENCHONDROMA

• Seen at any age (mostly in young people)

• Any bone preformed in cartilage (tubular bone of
hand and feet)
• Can be solitary or multiple and part of a
generalized dysplasia
When multiple enchondromas coexist, the diagnosis of
enchondromatosis should be considered.

Multiple enchondromas may occur in 3 distinct disorders:

Ollier disease
Maffucci syndrome
Metachondromatosis
Ollier disease
Is a non-hereditary disorder characterized by multiple
enchondromas with a predilection for unilateral distribution.
The enchondromas can grow large and can be disfiguring. 
Maffucci syndrome 
Is non-hereditary and is less common than Ollier disease.
This syndrome results in multiple hemangiomas in addition
to enchondromas.
Metachondromatosis
Consists of multiple enchondromas and osteochondromas.
Of the 3 disorders, metachondromatosis is the only one that
is hereditary, which is by autosomal dominant transmission.
• XRAY:
Well defined, centrally placed radiolucent area at the
junction of metaphysis and diaphysis
Sometime, bone is slightly expended
In mature lesion, there are flecks or wisps of
calcification within lucent area (pathopneumonic feature)
 ENCHONDROMA

• PATHOLOGY:
Lesion consist of pearly-white cartilaginous tissue
Often with central area of degeneration and calcification
Histology finding:
Simple hyaline cartilage
• COMPLICATION:

Malignant changes (<2%) for patient with solitary

lesions but as high as 30% for multiple lesion
(Ollier’s disease) and up to 100% in person with
hemangiomas (Maffucci’s syndrome)
• Sign of malignant transformation in patient >30 years old:
Onset of pain
Lesion enlargement
Cortical lesion

• Biopsy less help because cartilage usually looks benign

during early changes
• TREATMENT:
Not necessary
If tumor appear to enlarge and present of pathologic fracture, it
should be removes through curettage
The defect is filled with bone graft of bone cement