BONE TUMOURS

Day : Tuesday
Date : 11-12-2007
Time : 1.00-2.00
 Bone Tumours
WHAT SHOULD YOU KNOW

•Understand the clinical algorithm

•Correlate clinical presentation with radiological features
•Understand the classification and types of bone tumours
•Comprehend the management of bone tumours
•Understand the necessity for a team-approach
•Correlate Pathological findings with clinical presentation
(Clinico-pathological correlation)
 BONE TUMOURS

• rare but

• may result in amputation

• disfigurement and great physical challenge

Classification of bone tumours
Simple classification
A i. Primary

ii Secondary- more common

B i. Benign - oma

ii. Malignant - sarcoma

Primary Bone tumors are classified
according to the cell of origin
Histologic Type Benign Malignant
Hematopoietic (40%)   Myeloma
    Malignant lymphoma
Chondrogenic (22%) Osteochondroma Chondrosarcoma
  Chondroma Dedifferentiated chondrosarcoma
  Chondroblastoma Mesenchymal chondrosarcoma
  Chondromyxoid fibroma  
Osteogenic (19%) Osteoid osteoma Osteosarcoma
  Osteoblastoma  
Unknown origin (10%) Giant cell tumor Ewing tumor
    Giant cell tumor
    Adamantinoma
Histiocytic origin Fibrous histiocytoma Malignant fibrous histiocytoma
Fibrogenic Metaphyseal fibrous defect (fibroma) Desmoplastic fibroma
    Fibrosarcoma
Notochordal   Chordoma
Vascular Hemangioma Hemangioendothelioma
    Hemangiopericytoma
Lipogenic Lipoma Liposarcoma
Neurogenic Neurilemmoma  
Diagnosis of Bone Tumours

1. Age of patient

2. Location of tumour

3. Radiological
appearance

4. Histological features
AGE(probably the most important clinical clue).

Age group Most common benign lesions Most common malignant tumors

non-ossifying fibroma
fibrous dysplasia
simple bone cyst Ewing's sarcoma
aneurysmal bone cyst leukemic involvement
0-20 osteochondroma (exostosis) metastatic neuroblastoma
osteoid osteoma osteosarcoma,
osteoblastoma Ewing's sarcoma,
chondroblastoma
chondromyxoid fibroma
eosinophilic granuloma
enchondroma
21 - 40 chondrosarcoma
giant cell tumor

metastatic tumors
myeloma
leukemic involvement
40 & above osteoma chondrosarcoma
osteosarcoma (Paget's associated)
MFH
chordoma
 SITE OF LONG BONE INVOLVEMENT
(most primary bone tumors have favored sites within long bones; this may provide a clue to
diagnosis).

Diaphyseal lesions centered in Diaphyseal intramedullary lesions:

the cortex: Ewing's sarcoma, lymphoma, myeloma.
Osteoid osteoma Common for fibrous dysplasia and
enchondroma

Metaphyseal intramedullary lesions:

Osteosarcoma is usually centered in the
metaphysis. Chondrosarcoma and
Metaphyseal lesions centered in the
fibrosarcoma often present as metaphyseal
cortex:
lesions. Osteoblastoma, enchondroma,
Classic location for a non-ossifying
fibrous dysplasia, simple bone cyst, and
fibroma (NOF). Also, a common site for
aneurysmal bone cyst are common in this
osteoid osteoma.
location.

Epiphyseal lesions:
Metaphyseal exostosis: Chondroblastoma (Ch) and Giant
Osteochondroma Cell Tumor (GCT) are almost
invariably centered in the epiphysis.
Chondroblastoma is a rare tumor
seen in children and adolescents with
open growth plates. GCT is the most
common tumor of epiphyses in
skeletally mature individuals with
closed growth plates. GCT often
shows metaphyseal extension.
Radiological Features
Benign Tumours
Osteochondroma

Also known as an exostosis, is a cartilage –capped

out growth.

Men are affected three times more often

than women

Develop in bones of endochondral origin and arise

from the metaphysis near the growth plate of long
bones especially about the knee
Development over time of an osteochondroma
beginning with an outgrowth from the
epiphyseal cartilage
 Osteochondroma

Clinically present as slow growing masses

Can be painful if they impinge on a nerve

or if stalk is fractured.

In many cases, they are detected as an

incidental finding.

Rarely they give rise to chondrosarcoma

 The white arrows point to a mushroom-shaped, peduculated
Osteochondroma bony excresence arising from the anteromedial aspect of the
of femur distal femoral metaphysis, attached to the parent bone and
pointing away from the metaphyisis
Osteochondroma
Chondroma
Benign tumours of hyaline cartilage

May arise within the medullary cavity-enchondroma

May arise on the surface of bone – subperiosteal chondroma

Enchondromas are the most common

Located in the metaphyseal region of tubular bones

Most enchondromas are asymptomatic and detected

as incidental finding
Enchondroma of the phalanx with
a
pathological fracture
Enchondroma with a nodule of hyaline cartilage
encased by a thin layer of reactive bone.
Osteoid osteoma and Osteoblastoma

Have identical histology

Osteoblastoma larger than osteoid

osteoma
Osteoid osteoma
< 2 cm in greatest dimension

Affects teenagers and adolescents

75 % of patients < 25 years

Affects cortex of femur or tibia

Painful lesion

Relieved by salicylate
Osteoid osteoma of
Femoral neck
Osteoid osteoma
1. Compare and contrast :
Osteosarcoma
Chondrosarcoma
Giant cell tumor
Ewing's tumor

with respect to:

Histogenesis
age of group affected
location in the skeleton
histologic hallmarks
clinical behaviour
prognosis
Osteosarcoma (OS)

Most common primary malignant

tumor of the bone

Mesenchymal tumor

Cancerous cells produce bone matrix

75 % occur in patients younger than 20

years of age
Osteosarcoma (OS)
Primary osteosarcoma arise in the
metaphysis of long bones of the
extremities

Secondary osteosarcomas occur in

older patients with Paget’s disease

More common in men than women

Common sites are distal end of femur

or proximal tibia
 Osteosarcoma (OS)

Patients with Mutation of Rb gene are

predisposed to osteosarcoma

Concurrent trauma to bones and joints

In the elderly OS often arises from pre

existing bone diseases eg: Paget’s
disease of bone
 Osteosarcoma (OS)
Clinical Presentation
Painful and progressively enlarging
masses

Spread through blood stream

The tumour breaks through the cortex

and lifts the periosteum

Often metastasizes to the lungs

Osteosarcoma
on distal end of
femur

Cortex is
destroyed
Neoplastic osteoblasts forming osteoid
Chondrosarcoma
Frequency is about half of
osteosarcoma

Second most common malignant

matrix producing tumor
Mean age for chondrosarcoma is 43
years

Men are affected more than women

 Chondrosarcomas

Commonly arise in the central

portions of the skeleton
including Pelvis, proximal femur, ribs,
sternum and shoulder girdle
 Chondrosarcoma

Present as painful progressively

enlarging masses

Prognosis depends on size of tumor

Spreads to lungs and skeleton

Chondrosarcoma
 Chondrosarcoma
Tumor has developed in the proximal
femur

Not destroyed the cortex

Has a bluish, glassy appearance ,

reminiscent of cartilage
Malignant neoplastic cells produce a chondroid matrix
The aggressiveness of chondrosarcomas can be predicted by their histologic grade. Grading system is based on three parameters: cellularity,
degree of nuclear atypia and mitotic activity.

Grade 1 (low-grade)
Very similar to enchondroma. However, the cellularity is
higher, and there is mild cellular pleomorphism. The nuclei are
small but often show open chromatin pattern and small
nucleoli. Binucleated cells are frequent. Mitoses are very rare.
Grade 1 chondrosarcomas are locally aggressive and prone to
recurrences, but usually do not metastasize.

Grade 2 (low-grade)
The cellularity is higher than in Grade 1 tumors. Characteristic
findings are moderate cellular pleomorphism, plump nuclei,
frequent bi-nucleated cells, and occasional bizarre cells. Mitoses
are rare. Foci of myxoid change may be seen. Unlike Grade 1
tumors, about 10% to 15% of Grade 2 chondrosarcomas
produce metastases.

Grade 3 (high-grade)
Characteristic findings are high cellularity,
marked cellular pleomorphism, high N/C
ratio, many bizarre cells and frequent
mitoses (more than 1 per hpf). These are
high grade tumors with significant
metastatic potential.
Giant cell tumour of bone (GCT)
Contains a profusion of multinucleated
osteoclast type giant cells

Relatively uncommon benign

But locally aggressive

Usually arises during 5th decade

Slight female predominance

 Giant cell tumour of bone (GCT)

Involve both epiphysis and

metaphysis

In adolescents limited to metaphysis

Common sites are distal femur and

proximal tibia
Ewing sarcoma(ES)
Primary malignant small round cell
tumour

Ewing sarcoma has the youngest

average age at presentations (10-15 years)

Boys slightly more often affected than

girls
Ewing sarcoma(ES)

Pelvis is the most common site

usually arises in the diaphysis of
long bones especially femur
followed by tibia and humerus
Ewing sarcoma
of tibia from a
child
 The following studies are required to support the diagnosis of ES and PNET:

Demonstration of t(11;22) or EWS-FLI-1 fusion transcript (present in both ES and PNET)

Immunostains(both ES and PNET are positive for CD99/O13. In addition, PNET shows positive staining
with neural markers)
EM (ES cells are undifferentiated and show prominent glycogen deposits; PNET shows neural
differentiation)
Ewing sarcoma(ES)
The pathways of spread include

Direct extension

Lymphatic or vascular dissemination

Intraspinal seeding
Secondary tumours of bone

Metastatic cancer to bone is

more common than primary
cancer of bone
75% of bone metastasis originate from

Cancers of prostate
breast
kidney
lung
thyroid
Metastatic lesions are multifocal

Produce a lytic and or blastic reaction

Bone metastasis
Bone metastasis
Prostatic carcinoma metastatic to bone