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BONE TUMOURS
By Dr Shahid Manzur
Associate Professsor
Radiology Department
BVH
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
0-20
non-ossifying fibroma
fibrous dysplasia
simple bone cyst
aneurysmal bone cyst
osteochondroma (exostosis)
osteoid osteoma
osteoblastoma
chondroblastoma
chondromyxoid fibroma
eosinophilic granuloma
Ewing's sarcoma
leukemic involvement
metastatic neuroblastoma
osteosarcoma,
Ewing's sarcoma,
21 - 40 enchondroma
giant cell tumor
chondrosarcoma
40 & above
osteoma
metastatic tumors
myeloma
leukemic involvement
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 intramedullary lesions:
Ewing's sarcoma, lymphoma, myeloma.
Common for fibrous dysplasia and
enchondroma
Metaphyseal lesions centered in the
cortex:
Classic location for a non-ossifying
fibroma (NOF). Also, a common site for
osteoid osteoma.
Epiphyseal lesions:
Chondroblastoma (Ch) and Giant
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.
Metaphyseal exostosis:
Osteochondroma
Metaphyseal intramedullary lesions:
Osteosarcoma is usually centered in the
metaphysis. Chondrosarcoma and
fibrosarcoma often present as metaphyseal
lesions. Osteoblastoma, enchondroma,
fibrous dysplasia, simple bone cyst, and
aneurysmal bone cyst are common in this
location.
Diaphyseal lesions centered in
the cortex:
Osteoid osteoma
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
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
Osteochondroma
Osteochondroma
of femur
The white arrows point to a mushroom-shaped, peduculated
bony excresence arising from the anteromedial aspect of the
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
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 (OS)
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
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
Chondrosarcoma
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
Involve both epiphysis and
metaphysis
In adolescents limited to metaphysis
Common sites are distal femur and
proximal tibia
Giant cell tumour of bone (GCT)
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
Pelvis is the most common site
usually arises in the diaphysis of
long bones especially femur
followed by tibia and humerus
Ewing sarcoma(ES)
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)
The pathways of spread include
Direct extension
Lymphatic or vascular dissemination
Intraspinal seeding
Ewing sarcoma(ES)
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
Thanks

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bone tumors 2.ppt

  • 1. BONE TUMOURS By Dr Shahid Manzur Associate Professsor Radiology Department BVH
  • 2. 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)
  • 3. BONE TUMOURS • rare but • may result in amputation • disfigurement and great physical challenge
  • 4. Classification of bone tumours Simple classification A i. Primary ii Secondary- more common B i. Benign - oma ii. Malignant - sarcoma
  • 5. Primary Bone tumors are classified according to the cell of origin
  • 6. 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
  • 7. Diagnosis of Bone Tumours 1. Age of patient 2. Location of tumour 3. Radiological appearance 4. Histological features
  • 8. AGE(probably the most important clinical clue). Age group Most common benign lesions Most common malignant tumors 0-20 non-ossifying fibroma fibrous dysplasia simple bone cyst aneurysmal bone cyst osteochondroma (exostosis) osteoid osteoma osteoblastoma chondroblastoma chondromyxoid fibroma eosinophilic granuloma Ewing's sarcoma leukemic involvement metastatic neuroblastoma osteosarcoma, Ewing's sarcoma, 21 - 40 enchondroma giant cell tumor chondrosarcoma 40 & above osteoma metastatic tumors myeloma leukemic involvement chondrosarcoma osteosarcoma (Paget's associated) MFH chordoma
  • 9. SITE OF LONG BONE INVOLVEMENT (most primary bone tumors have favored sites within long bones; this may provide a clue to diagnosis). Diaphyseal intramedullary lesions: Ewing's sarcoma, lymphoma, myeloma. Common for fibrous dysplasia and enchondroma Metaphyseal lesions centered in the cortex: Classic location for a non-ossifying fibroma (NOF). Also, a common site for osteoid osteoma. Epiphyseal lesions: Chondroblastoma (Ch) and Giant 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. Metaphyseal exostosis: Osteochondroma Metaphyseal intramedullary lesions: Osteosarcoma is usually centered in the metaphysis. Chondrosarcoma and fibrosarcoma often present as metaphyseal lesions. Osteoblastoma, enchondroma, fibrous dysplasia, simple bone cyst, and aneurysmal bone cyst are common in this location. Diaphyseal lesions centered in the cortex: Osteoid osteoma
  • 11. 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
  • 12. Development over time of an osteochondroma beginning with an outgrowth from the epiphyseal cartilage
  • 13. 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 Osteochondroma
  • 14. Osteochondroma of femur The white arrows point to a mushroom-shaped, peduculated bony excresence arising from the anteromedial aspect of the distal femoral metaphysis, attached to the parent bone and pointing away from the metaphyisis
  • 16. 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
  • 17. Enchondroma of the phalanx with a pathological fracture
  • 18. Enchondroma with a nodule of hyaline cartilage encased by a thin layer of reactive bone.
  • 19. Osteoid osteoma and Osteoblastoma Have identical histology Osteoblastoma larger than osteoid osteoma
  • 20. 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
  • 23. 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
  • 24. 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
  • 25. 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)
  • 26. 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)
  • 27. 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 (OS)
  • 28.
  • 29. Osteosarcoma on distal end of femur Cortex is destroyed
  • 31. 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
  • 32. Chondrosarcomas Commonly arise in the central portions of the skeleton including Pelvis, proximal femur, ribs, sternum and shoulder girdle
  • 33. Present as painful progressively enlarging masses Prognosis depends on size of tumor Spreads to lungs and skeleton Chondrosarcoma
  • 34.
  • 36. Tumor has developed in the proximal femur Not destroyed the cortex Has a bluish, glassy appearance , reminiscent of cartilage Chondrosarcoma
  • 37. Malignant neoplastic cells produce a chondroid matrix
  • 38.
  • 39. 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.
  • 40. 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
  • 41. Involve both epiphysis and metaphysis In adolescents limited to metaphysis Common sites are distal femur and proximal tibia Giant cell tumour of bone (GCT)
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  • 46. 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
  • 47. Pelvis is the most common site usually arises in the diaphysis of long bones especially femur followed by tibia and humerus Ewing sarcoma(ES)
  • 48. Ewing sarcoma of tibia from a child
  • 49.
  • 50. 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)
  • 51. The pathways of spread include Direct extension Lymphatic or vascular dissemination Intraspinal seeding Ewing sarcoma(ES)
  • 52. Secondary tumours of bone Metastatic cancer to bone is more common than primary cancer of bone
  • 53. 75% of bone metastasis originate from Cancers of prostate breast kidney lung thyroid Metastatic lesions are multifocal Produce a lytic and or blastic reaction