The document describes several primary malignant bone tumors including: 1. Multiple myeloma, osteosarcoma, chondrosarcoma, Ewing's sarcoma, and chordoma. 2. It provides details on characteristics such as common age of onset, tumor location preferences, radiographic features on plain films and MRI/CT, and histological features. 3. Specific subtypes are also described like parosteal osteosarcoma, telangiectatic osteosarcoma, solitary plasmacytoma, and adamantinoma.

1. 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

3. Radiographic features that may help differentiate benign
from malignant lesions

4. 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

5. SITE OF ORIGIN
EPIPHYSIS MALIGNANT GCT
METAPHYSIS OSTEOSARCOMA
CHONDROSARCOMA
FIBROSARCOMA
DIAPHYSIS EWING’S SARCOMA
MULTIPLA MYELOMA
ADAMANTINOMA
MFH

6. OSTEOSARCOMA
MALIGNANT BONE FORMING TUMOUR
PRIMARY SECONDARY
 INTAOSSEOUS-TELANGIECTATIC,SMALL CELL,LOW GRADE
 SURFACE- PAROSTEAL ( common surface OS )
- PERIOSTEAL
 MULTICENTRIC

7. 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

8. Types of matrix: Osteoblastic
solid sclerotic mass,parosteal
osteosarcoma
wisps of tumor-bone formation,
osteosarcoma of the sacrum

9. 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"

10. “Sunburst” periosteal formation
Codman’s Triangle

14. Central Osteosarcoma
Expansile lytic destruction
homogeneous sclerosis 50%
Osteolytic 25% mixed 25%
Aggressive features such as cortical or
medullary bone destruction

16. 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

19. 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

20. 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

21. string sign

22. •A well-defined and
homogeneous sclerotic mass
with a broad base to the
underlying diaphysis of the
fibula.

23. •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.

24. 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

25. Periosteal OS
Lytic in appearance,
cortical erosion
periosteal reaction.

26. PERIOSTEAL

28. 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.

29. 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.

31. 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

32. 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

33. 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

34. •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

36. Types of matrix: chondroid matrix
Enchondroma
chondroid matrix
CHONDRSARCOMA

39. Proximal tibia
diaphysis.
subtle calcifications,
Endosteal
scalloping hallmark
of
chondrosarcoma.
MR better defines
the extension of the
lesion.
endosteal
scalloping.

40. CHONDROSARCOMA ARISING FROM OSTEOCHONDROMA
calcified mass arising from the proximal fibula.
high uptake on the bone scan

41. Axial T2 WI
SECONDARY CHONDROSARCOMA

42. 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.

44. 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

45.  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

48. 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

49. Biopsy material
highly cellular,
infiltrative neoplasm -sheets of tightly
packed, round cells with very scant
cytoplasm ("round blue cell tumor").

50. osteosarco
ma
Ewing’s Infection

51. 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

52. 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

54. 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".

55. 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

56. 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

57. 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

58. 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

59. 1.numerous, well-circumscribed lytic bone lesions
(more common):
 punched out lucencies –
pepperpot skull or raindrop skull
 2.endosteal scalloping

60. 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

61. RAINDROP SKULL

63. numerous lytic lesions without reactive
sclerosis
"soap-bubbly" appearance in the ischia.
lytic lesions in proximal femora.

64. 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

65. 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.-

66. Expansile soft tissue lesion involving
only the L1 vertebra.
Prominent residual thickened
trabeculae "MINI BRAIN" appearance.

67. 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