PRIMARY MALIGNANT BONE TUMORS

1. MULTIPLE MYELOMA ( M/C)

2. OSTEOSARCOMA

3. CHONDROSARCOMA

4. EWINGS SARCOMA

5. CHORDOMA

Others
MALIGNANT FIBROUS HISTIOCYTOMA
ANGIOSARCOMA
FIBROSARCOMA
Radiographic features that may help differentiate benign
from malignant lesions
 AGE OF ONSET

Age in Tumour
years
<1 Neuroblastoma

1-10 Ewing sarcoma-tubular bones

10-30 Osteosarcoma, Ewing sarcoma-flat bones

30-40 Fibrosarcoma,malignant fibrous

histiocytoma,lymphoma,Malignant GCT

>40 Chondrosarcoma,chordoma,multiple myeloma

 SITE OF ORIGIN

EPIPHYSIS MALIGNANT GCT

METAPHYSIS OSTEOSARCOMA
CHONDROSARCOMA
FIBROSARCOMA

DIAPHYSIS EWINGS SARCOMA

MULTIPLA MYELOMA
ADAMANTINOMA
MFH
 OSTEOSARCOMA

MALIGNANT BONE FORMING TUMOUR

PRIMARY SECONDARY

INTAOSSEOUS-TELANGIECTATIC,SMALL CELL,LOW GRADE

SURFACE- PAROSTEAL ( common surface OS )

- PERIOSTEAL

MULTICENTRIC
PRESENTATION
-PAIN
-PALPABLE MASS
-PATHOLOGICAL #

AGE 10-30 YRS

SITE METAPHYSIS OF LONG BONES

-DISTAL FEMUR
-PROXIMAL TIBIA
-OTHERS HUMERUS,RIBS,ILEUM,JAW BONES

LUNG - Cannonball mets, subpleural nodule > cavitate > rupture

>Spontaneous pneumothorax
Skeletal mets Skip lesions
 Types of matrix: Osteoblastic

wisps of tumor-bone formation,

osteosarcoma of the sacrum

solid sclerotic mass,parosteal

osteosarcoma
 Radiographic features
Plain film

Medullary and cortical bone destruction

Wide zone of transition

Permeative or moth-eaten appearance

Aggressive periosteal reaction

Sunburst
Codman triangle

Soft-tissue mass

Tumour matrix ossification/calcification

ill-defined "fluffy" or "cloud-like"
Sunburst periosteal formation

Codmans Triangle
 Central Osteosarcoma

Expansile lytic destruction

homogeneous sclerosis 50%

Osteolytic 25% mixed 25%

Aggressive features such as cortical or

medullary bone destruction
 Multicentric Osteosarcoma

Early presentation 5-10 yrs

Course rapid, fatal
Early pulmonary mets

8yrs old female with pain in LL

since 6 months

Lab- Elevated Alkaline

Phosphatase
Parosteal Osteosarcoma

Origin-surface of the bone.

Grows -surrounding soft tissues, may also

infiltrate bone marrow.

M/C on the posterior side of the distal

femur.

Slow growing, 30-50 yrs

Ossification in a parosteal osteosaroma -

in the center than at the periphery.

D/D myositis ossificans present close to

the cortical bone, but maturation develops
from the periphery to the center
Radiographic features
large lobulated exophitic, 'cauliflower-like'
Mass with central dense ossification

string sign thin radiolcent line separating the

tumour from cortex, Cleavage plane.

+/- soft tissue mass.

cortical thickening without aggressive periosteal

reaction is often seen.

tumour extension into medullary canal,freqently

string sign
A well-defined and
homogeneous sclerotic mass
with a broad base to the
underlying diaphysis of the
fibula.
Radiograph.
Homogeneous ossified mass
adjacent to the cortical bone of the
distal femur

MRI
Sagittal T1-weighted MR
very low signal intensity due to the
ossified matrix and the cortical bone
which is unimpaired.
Periosteal osteosarcoma

Broad-based surface soft-tissue mass

Extrinsic erosion of thickened underlying

diaphyseal cortex

M/c Femur, Tibia

Perpendicular periosteal reaction extending into

the Soft-tissue component:

Periosteal reaction common,

Sunburst
Codman triangle
Periosteal OS

Lytic in appearance,

cortical erosion

periosteal reaction.
PERIOSTEAL
Telangiectatic Osteosarcoma

Uncommon
telangiectatic component 90% .
Large blood filled spaces
separated by thin bony
septations.
Asymmetric expansion
Lysis of bone
Aggressive growth pattern
Cortical destruction
Minimal peripheral sclerosis

CT, MR Fluid fluid levels.

 presence of nodular septal
thickening, osteoid matrix
mineralization in a soft-tissue
mass, and an aggressive
growth pattern can aid in
distinguishing telangiectatic
OS from ABC.
 CHONDROSARCOMA
Malignant cartilaginous tumour
> 40 YRS
SITESLONG BONES,PELVIS,RIBS,SPINE,STERNUM, skull base)

TYPES

PRIMARY INTRAMEDULLARY
JUXTACORTICAL
CLEAR CELL,MYXOID,EXTRASKELETAL

SECONDARY

osteochondroma
solitary osteochondroma
hereditary multiple exostoses)
enchondroma
solitary enchondroma Ollier disease Maffucci syndrome
C/F

PAIN
PATHOLOGICAL #
PALPABLE MASS
Hyperglycaemia paraneoplastic phenomenon.

H/P
multilobulated (due to hyaline cartilage nodules)

central high water content and peripheral

enchondral ossification.

This accounts not only for the high T2 MRI

rings and arcs calcification or popcorn calcification

Plain film

lytic (50%)

intralesional calcification

rings and arcs / popcorn calcification)

endosteal scalloping: affecting more than two thirds of the

cortical thickness (c.f. less than 2/3 in enchondromas)

moth eaten /permeative appearance and periosteal

reaction distinguishing between enchondroma and
chondrosarcoma
DIFF WITH ENCHONDROMA

Elderly patient

Location in long bones

Size > 5 cm

Uptake on bone scan

Endosteal scalloping on MRI

Cortical involvement

Early enhancement on dynamic

contrast enhanced series
Types of matrix: chondroid matrix

Enchondroma CHONDRSARCOMA
chondroid matrix
Proximal tibia
diaphysis.
subtle calcifications,

Endosteal
scalloping hallmark
of
chondrosarcoma.

MR better defines
the extension of the
lesion.
endosteal
scalloping.
CHONDROSARCOMA ARISING FROM OSTEOCHONDROMA

calcified mass arising from the proximal fibula.

high uptake on the bone scan
SECONDARY CHONDROSARCOMA

Axial T2 WI
 rings-and-arcs
calcifications

The differential diagnosis is

enchondroma or low grade
chondrosarcoma.

The CT shows the

calcifications with subtle
endosteal thinning of the
cortical bone

Final diagnosis: low grade

chondrosarcoma.
 EWING SARCOMA
Small round blue cell tumour

Second most common malignant bone tumor in children (after osteosarcoma)

medullary cavity,
usually of long bones in the lower extremities

Femur most common

pelvis
upper limb
spine and ribs sacrococcygeal region

SYMPTOMS
Age 5-15 yrs
localized pain and swelling
Additional symptoms may include
Fever
Weight loss
Anemia
Leukocytosis
Elevated erythrocyte sedimentation rate
 Poorly marginated,
Lytic
destructive lesion
Permiative / moth eaten (mottled)

Soft tissue mass or infiltration is common

Soft tissue mass may produce saucerization (scalloped depression
in cortex)

Periosteal reaction

Lamellated - onion-skinning due to successive layers of periosteal

development
Sunburst or spiculated - hair-on-end appearance when new bone
is laid down perpendicular to cortex along Sharpeys fibers
Codmans triangle - formed between elevated periosteum
with central destruction of cortex
16 yr old white male with pain in his
left upper arm.

Mild swelling and tenderness

Pain progressively getting

worse for ~ 3 months

Recent onset of mild fever

Biopsy material
highly cellular,

infiltrative neoplasm -sheets of tightly

packed, round cells with very scant
cytoplasm ("round blue cell tumor").
osteosarco Ewings Infection
ma
 CHORDOMA
Embryonic remnants of the primitive notochord
earliest fetal axial skeleton, extending from the Rathke's pouch to
the coccyx)

locally aggressive

1. sacro-coccygeal: 30-50% 2-3

2. spheno-occipital: 30-35%
3. vertebral body: 15-30%

(30-60 years)
spheno-occipital -20-40 years
sacrococcygeal -age group (peak 40-60 years).

Clinical Findings
Low back pain
Constipation or fecal incontinence
Rectal bleeding
Sciatica from nerve root compression
Frequency, urgency, straining on micturition
Imaging Findings

Large presacral mass (>10cm) with displacement of the rectum

and/or bladder

Solid tumor with cystic areas in 50%

Destroys multiple sacral and coccygeal segments

Sequestered bone fragments are common

Sclerotic rim in 50%

May have amorphous calcifications, especially peripherally

May cross the sacroiliac joint

Mild-moderate enhancement
SPHENO-OCCIPITAL

The clival region 2nd most

common

Typically the mass

projects in the mid-line
posteriorly indenting the
pons.

This characteristic
appearance has been
termed the 'thumb sign".
Multiple myeloma

Four main patterns

1. disseminated form: multiple defined lesions: axial
skeleton

2. disseminated form: diffuse skeletal osteopenia

3. solitary plasmacytoma: -vertebral body/pelvis

4. osteosclerosing myeloma
Clinical presentation
60-70 YRS
bone pain:
initially intermittent, but becomes constant
worse with activity/weight bearing, and thus is
worse during the day
anaemia:
typically normochromic/normocytic
renal failure/proteinuria:
pathological fracture:
vertebral compression fracture
long bone fracture (e.g. proximal femur)
amyloidosis
recurrent infection: e.g. pneumonia due to leukopaenia
Lab findings :
reverse albumin/globulin ratio
monoclonal gammopathy (IgA and/or IgG
peak)
proteinuria: Bence Jones proteins in urine
hypercalcaemia

SITES
vertebrae (most common)
ribs
skull
shoulder girdle
pelvis
long bones
Radiographic features

Plain film

skeletal survey

diagnosis of multiple myeloma,

in assessing response,
potential complications (e.g. pathological fracture).

1. lateral skull
2. frontal chest film
3. cervico-thoraco-lumbar spine
4. shoulders
5. pelvis
6. femurs
1.numerous, well-circumscribed lytic bone lesions
(more common):
punched out lucencies
pepperpot skull or raindrop skull

2.endosteal scalloping
Generalized osteopaenia (less common):
often associated with

vertebral compression fractures

Wrinkled vertebra of Myeloma
vertebra plana

Less involvement of Pedicles

PEDICLE sign of Multiple Myeloma

Osteoblastc lesions- Ivory vertebra

RAINDROP SKULL
 numerous lytic lesions without reactive
sclerosis
"soap-bubbly" appearance in the ischia.
lytic lesions in proximal femora.
 SOLITARY BONE PLASMACYTOMA (SBP)

Thoracic vertebrae-M/C
Lumbar, sacral, and cervical vertebrae.
Rib, sternum, clavicle, or scapula
C/F
painful mass, pathologic fracture, or root
or spinal cord compression syndrome.

Diagnostic criteria
single area of destruction due to clonal plasma cells
bone marrow plasma cell infiltration <5% of all nucleated
cells
absence of osteolytic bone lesions or other tissue
involvement absence of anemia, hypercalcemia or renal
impairment
low or absent serum / urine monoclonal protein
preserved levels of uninvolved immunoglobulins
Radiographic features
Plain film
Solitary expansile lytic lesion
thinning and destruction of cortex
bubbly/trabeculated appearance.
Characteristic absence of sclerotic reaction
.
CT
Expansile lytic lesion with thinned out cortex,
characteristic 'mini-brain' appearance solitary vertebral
lesions.-
Expansile soft tissue lesion involving
only the L1 vertebra.

Prominent residual thickened

trabeculae "MINI BRAIN" appearance.
ADAMANTINOMA OF LONG BONES

2ND TO 3RD DECADES

LOCALLY AGGRESSIVE

DULL PAIN OF GRADUAL ONSET.

TIBIAL DIAPHYSIS (ESPECIALLY ANTERIOR CORTEX)

MULTI-LOCULAR OR SLIGHTLY EXPANSILE OSTEOLYTIC

LESION

SOLITARY FOCUS OR MULTICENTRIC LUCENCIES

CORTICAL LUCENCIES COMBINED WITH SCLEROSIS.

LACK OF PERIOSTEAL REACTION