Malignant Bone

Tumours and Staging of

MSK Tumours
Osteosarcoma
 Introduction and
Epidemiology
A malignant mesenchymal tumor in which
the cancerous cells produce bone matrix.
Most common primary malignant tumor of
bone approximately 20% of primary
bone cancers.
Bimodal age distribution: 75% occur in
persons younger than 20 years of age, the
rest in patients above 60 (assc with
Pagets dz, bony infarcts, radiation).
May affect any bone but most commonly
involves the long-bone metaphyses,
especially around the knee.
 Pathogenesis
Approximately 70% of osteosarcomas
have acquired genetic abnormalities
such as ploidy changes and chromosomal
aberrations, none of which are specific.
Examples of abnormalities:
a) RB gene mutation
b) p53 gene mutation
c) Abnormalities in INK4a gene
)Strong association with sites of active
bone growth.
 Morphology and
Description
Site of origin (intramedullary, intracortical,
or surface).
Degree of differentiation
Multicentricity (synchronous,
metachronous)
Primary (underlying bone is unremarkable)
or secondary to preexisting disorders such
as benign tumors, Paget disease, bone
infarcts, previous irradiation.
Histologic features (osteoblastic,
chondroblastic, fibroblastic, telangiectatic,
small cell, and giant cell).
 Most common subtype

Arises in the metaphysis of

long bones and is primary,
solitary, intramedullary and
poorly differentiated.
 Gross Features
Big bulky tumors that are gritty, gray-white, and
often contain areas of hemorrhage and cystic
degeneration.
Frequently destroy the surrounding cortices and
produce soft-tissue masses.
They spread extensively in the medullary canal,
infiltrating and replacing the marrow
surrounding the preexisting bone trabeculae.
Infrequently, they penetrate the epiphyseal
plate or enter the joint.
When joint invasion occurs, the tumor grows
into it along tendoligamentous structures or
through the attachment site of the joint capsule.
 Histological Features
The formation of bone by the tumor cells is
characteristic.
The tumor cells vary in size and shape and
frequently have large hyperchromatic nuclei.
Bizarre tumor giant cells are common, as are
mitoses.
The neoplastic bone usually has a coarse,
lace-like architecture but also may be
deposited in broad sheets or as primitive
trabeculae.
Other matrices, including cartilage or fibrous
tissue, may be present in varying amounts.
 Variants of OsteoSa
1. Parosteal Sa
.This is a low-grade sarcoma situated on the
surface of one of the tubular bones, usually at
the distal femoral or proximal tibial metaphysis.
2. Periosteal Sa
.True osteosarcoma, but characteristically the
sections show a prominent cartilaginous
element.
3. Pagets Sa
.High grade tumour with f eatures of Pagets
disease, but with areas of bone destruction and
soft-tissue invasion
 Clinical Features
Painful, progressively enlarging masses.
Pain is usually worse at night.
Sometimes a sudden fracture of the bone
is the first symptom.
Features of metastases to the lungs,
brain and other bones.
 Radiological Features
Hazy osteolytic areas may alternate with
unusually dense osteoblastic areas.
The endosteal margin is poorly defined.
Often the cortex is breached and the
tumour extends into the adjacent tissues.
When this happens, streaks of new bone
appear, radiating outwards from the
cortex (sunburst effect).
Where the tumour emerges from the
cortex, reactive new bone forms at the
angles of periosteal elevation (Codmans
triangle).
Ewings Sarcoma
 Introduction and
Epidemiology
Ewing sarcoma family of tumors
encompasses Ewing sarcoma and primitive
neuroectodermal tumor (PNET), which are
primary malignant small round-cell tumors of
bone and soft tissue.
Believed to arise from endothelial cells in the
bone marrow.
Occurs most commonly between the ages of
10 and 20 years, usually in a tubular bone
and especially in the tibia, fibula or clavicle.
Boys are affected slightly more frequently
than girls, whites are affected more than
blacks.
 Pathogenesis
Most Ewing sarcoma/PNET have a
translocation involving the EWS gene on
chromosome 22 and a gene encoding an
ETS family transcription factor.
The most commonly involved ETS gene is
FLI1, as part of a (11;22) (q24;q12)
translocation.
 Morphology and
Description
The tumor is soft, tan-white, and frequently
contains areas of hemorrhage and necrosis.
It is composed of sheets of uniform small,
round cells that are slightly larger than
lymphocytes.
They have scant cytoplasm, which may
appear clear because it is rich in glycogen.
The presence of Homer-Wright rosettes is
indicative of neural differentiation.
Relatively few mitotic figures in relation to
the dense cellularity of the tumor.
 Clinical Features
Similar to osteoSa.
With systemic manifestations such as
intermittentfevers,anemia,leukocytosis,
increasedESR.
 Radiological Features
An area of bone destruction that is
predominantly in the mid-diaphysis.
New bone formation may extend along
the shaft and sometimes it appears as
fusiform layers of bone around the lesion
onion peel effect.
Sunburst appearance and Codmans
triangle.
 Secondary
Metastatic Disease
 Origins
Adults:
a) Male: Prostate
b) Female: Breast
c) Others: RCC, thyroid ca, lung ca, liver
ca.
)Children:
)Neuroblastoma, Wilms tumor, OsteoSa,
Ewing Sa, rhabdomyoSa
Staging of MSK
Tumours
 Enneking System
Grade
G0 - Benign lesion
G1 - Low-grade malignant lesion
G2 - High-grade malignant lesion
Site
T0 - A benign tumor that is confined within a true capsule
and the lesion's anatomic compartment of origin (ie, a
benign intracapsular, intracompartmental lesion)
T1- An aggressive benign or malignant tumor that is still
confined within its anatomic compartment (ie, an
intracompartmental lesion)
T2 - A lesion that has spread beyond its anatomic
compartment of origin (ie, an extracompartmental lesion)
Metastasis
M0 - No regional or distant metastasis
M1 - Regional or distant metastasis
Staging of Malignant
Tumors