PRIMARY MALIGNANT BONE TUMORS 1. MULTIPLE MYELOMA ( M/C)

2. OSTEOSARCOMA

3. CHONDROSARCOMA

4. EWING’S SARCOMA

5. CHORDOMA

Others

MALIGNANT FIBROUS HISTIOCYTOMA

ANGIOSARCOMA

FIBROSARCOMA

Radiographic features that may help differentiate benign from malignant lesions

Age in years

Tumour

<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

AGE OF ONSET

SITE OF ORIGIN

EPIPHYSIS MALIGNANT GCT

METAPHYSIS OSTEOSARCOMA

CHONDROSARCOMA

FIBROSARCOMA

DIAPHYSIS EWING’S SARCOMAMULTIPLA 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

solid sclerotic mass,parosteal osteosarcoma

wisps of tumor-bone formation, osteosarcoma of the sacrum

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

Codman’s 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

•MRISagittal 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

SITES—LONG 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

•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

DIFF WITH ENCHONDROMA

Types of matrix: chondroid matrix

Enchondromachondroid matrix

CHONDRSARCOMA

Proximal tibia diaphysis.subtle calcifications,

Endostealscalloping hallmark of chondrosarcoma.

MR better defines the extension of the lesion.

endostealscalloping.

CHONDROSARCOMA ARISING FROM OSTEOCHONDROMA

calcified mass arising from the proximal fibula.high uptake on the bone scan

Axial T2 WI

SECONDARY CHONDROSARCOMA

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 SARCOMASmall 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 yrslocalized 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 Sharpey’s fibers

Codman’s 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

ma

Ewing’s Infection

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

CHORDOMA

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 patterns1. 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

•vertebrae (most common)

•ribs

•skull

•shoulder girdle

•pelvis

•long bones

SITES

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