Primary bone tumors can arise from bone or cartilage cells and are either benign or malignant. The most common primary malignant bone tumors are osteosarcoma, chondrosarcoma, Ewing's sarcoma, and fibrosarcoma. Osteosarcoma typically affects teenagers and presents as pain in the long bones. Chondrosarcoma is usually slow growing and affects older adults, most commonly in the pelvis or shoulder. Ewing's sarcoma affects bone or soft tissue in teenagers and young adults. Fibrosarcoma can occur as a soft tissue or bone tumor, usually in the femur or tibia. Secondary bone tumors most commonly spread from the lung, prostate, breast, or liver and deposit in

1. bone tumors (malignant)
Prepared by: Nibal Shawabkeh
Supervised by: Dr. Salim Abu Rmeileh
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2. Bone Tumors
 Benign
 Malignant
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Primary
Secondary

3. PRIMARY BONE TUMORS
 All components of bone are derived from the mesoderm  bone tumors
are
 potentially composed of any of its four stem cell types:
1. Fibroblast.
2. Chondroblast.
3. Osteoblast.
4. Reticulomylogenic cells.
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4. Primary bone tumors
 Osteosarcoma
 Chondrosarcoma
 Ewings sarcoma
 Fibrosarcoma
 Multiple myeloma
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5. 5
Classification Of Primary Tumors Of Bone
Tissue origin Benign neoplasm Malignant neoplasm
Bone Osteoma
Osteochondroma
Osteoid osteoma
osteoblastoma
Osteogenic sarcoma
Parosteal osteosarcoma
Cartilage Enchondroma
Chondroblastoma
Chondromyxoid fibroma
Juxtacortical chondroma
Osteochondroma
Chondrosarcoma
Primary
secondary
Fibrous Nonossifying fibroma
Desmoplastic fibroma
Fibrous dysplasia
Osteofibrous dyspasia
fibrosarcoma
Uncertain Giant cell tumor
Fibrous histiocytoma
Malignant Giant cell tumor
Malignant fibrous histiocytoma
admantanoma
Marrow elements Histiocytosis X Lymphoma
Reticulum cell sarcoma
Lymphosarcoma
leukemia
Fat lipoma liposarcoma
Vascular Hemangioma
Glomus tumor
hemangiopericytoma
Angiosarcoma
Hemangioendothelioma
Malignant hemangiopericytoma
Notochord -- chordoma
undifferentiated -- Mesenchymal sarcoma

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7. Osteosarcoma
 Arises from the osteoid tissue .
 Most common primary bone tumor in children .
 Age : 15- 19 years (teenagers) , also occur in the
elderly.
 Boys to girls ratio : 1.4:1.
 Usually occurs in the long bones.
 Most commonly : femur , tibia , humerus.
 Metastasis to lungs.
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8. Types of osteosarcoma
 conventional types (osteoblastic, chondroblastic, and fibroblastic).
 Telangiectatic.
 Multifocal.
 Parosteal.
 Periosteal.
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9. Etiology
 Rapid bone growth.
 Environmental factors : exposure to radiation.
 Genetics : bone dysplasia , Li-Fraumeni syndrome, Rothmund-Thomson syndrome.
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10. Symptoms
 Most common symptom is pain ( usually and night and with activity) .
 Swelling .
 Sometimes pathological fractures.
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11. Physical examination
 Depends on the site of the tumor :
 Mass
 Decreased range of motion
 Respiratory findings
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12. Diagnosis
 Depends on history and physical examination.
 Lab studies : most importantly ALP , LDH
 Imaging studies.
 Bone biopsy : only definitive method to determine whether a tumor is benign or
malignant .
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13. Imaging studies
 X ray : sun-burst" appearance and the apperance of Codman triangle.
 CT scanning
 MRI
 Bone scan
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14. sun-burst" appearance14

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16. Staging
 Stage I – Low-grade lesions
 Stage II – High-grade lesions
 Stage III – Metastases
 Subtypes : Substage A – Intracompartmental lesion (intramedullary lesion for bone tumors)
 Substage B – Extracompartmental lesion (extramedullary spread for bone tumors)
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17. Treatment
 Medical : use of chemotherapy as neoadjuvant.
 Surgical. : tumor resection with an adequate margin.
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18. prognosis
 Stage I : It has an excellent prognosis (>90%) with wide resection.
 Stage II : 2-year survival after the metastases of 50%, 5-year of 40% and 10 year of 20%
 Stage III: Overall survival prognosis is about 30%
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19. Chondrosarcoma
 This is a rare tumor of the cartilage of the bone
 It is the malignant form of chondroma
 Second most common primary bone tumor
 It grows slowly and rarely spreads. Its most common site is at the base of the
skull.
 range from low-grade tumors with low metastatic potential to high-grade,
aggressive tumors characterized by early metastasis.
 More common among older people ( 30 and 60 years old).
 Males and females are equally affected.
 Usually affects the hips , pelvis and shoulder .
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20. Chondrosarcoma types
 Conventional chondrosarcoma
 Dedifferentiated chondrosarcoma
 Clear cell chondrosarcoma
 Mesenchymal chondrosarcoma
 Juxtacortical chondrosarcoma
 Secondary chondrosarcoma
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21. Chondrosarcoma Grades
 Grade I (low grade) – Cytologically similar to enchondroma[2] ; cellularity is
higher, with occasional plump nuclei with open chromatin structure
 Grade II (intermediate grade) – Characterized by a definite and increased
cellularity; distinct nucleoli are present in most cells, and foci of myxoid
change may be seen
 Grade III (high grade) – Characterized by high cellularity, prominent
nuclear atypia, and the presence of mitosis
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22. Clinical Presentation
 Deep, dull, achy pain
 Pain at night
 Nerve dysfunction of the lumbosacral plexus or the sciatic or femoral
nerves, with pelvic lesions near a neurovascular bundle
 Limitation of joint range of motion and disturbance of joint function, with
chondrosarcomas close to a joint
 Pathologic fracture
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23. Diagnosis
 History and physical examination.
 Imaging studies.
 biopsy .
23

24. Imaging studies
 X ray : discrete calcification , appear large ,bony contour appears thinned and expanded .
 Periosteum overlying the tumor may be elevated.
 CT scan : detect subtle calcifications
 MRI : investigation of choice for assessing the extent of a chondrosarcoma.
 delineate the extent of soft-tissue involvement.
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26. Treatment
 Medical : radiotherapy & chemotherapy play limited roles in primary treatment.
 Surgical : Complete, wide surgical excision of the chondrosarcoma .
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27. Prognosis
 Conventional :
 Grade I : 90 % survival at 5 years
 Grade II : 81% survival at 5 years
 Grade III : 29% survival at 5 years
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28. Ewings sarcoma
 Rare disease in which the cancer cells are found in in the bone or in soft tissue .
 most common areas : pelvis, femur, humerus, ribs and clavicle.
 occurs most frequently in teenagers and young adults.
 a male to female ratio of 1.6:1
 Thirty percent are overtly metastatic at presentation.
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29. Etiology
 Genetic exchange: translocation between chromosomes 11 and 22.
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30. Symptoms
 Localized pain
 Back pain, which may indicate a paraspinal, retroperitoneal, or deep pelvic tumor.
 Palpable mass
 Systemic symptoms of fever and weight loss, which often indicate metastatic disease
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31. Diagnosis
 History and physical examination .
 Imaging studies
 Histology , Cytogenetic and molecular studies
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32. Imaging studies
 X ray : permeative lytic lesion with periosteal reaction.
 CT scan : used to define the extraosseous extent of the tumor, especially in the skull, spine,
ribs and pelvis.
 MRI
 Bone scintigraphy : can also be used to follow tumor response to therayp
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34. 34

35. 35

36. Treatment
 Treatment lasts 6-9 months and consists of alternating courses of 2 chemotherapeutic
regimens.
 Then the remaining tumor is surgically resected, irradiated, or both.
 The surgical resection may involve limb salvage or amputation.
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37. Prognosis
 Depends on the staging
 Five-year survival for localized disease is 70% to 80% when treated with chemotherapy.
 Long term survival for metastatic disease may be less than 10%
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38. Fibrosarcoma
 tumor of mesenchymal cell origin that is composed of malignant fibroblasts.
 It can occur as a soft-tissue mass or as a primary or secondary bone tumor.
 Fibrosarcoma of bone occurs slightly more commonly in men than in women.
 Usually diagnosed during the 4th decade of life
 usually located in the lower extremities, especially the femur and tibia
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39. Etiology
 Genetic mutations .
 Arises from preexisting lesions, such as bone infarcts , chronic osteomyelitis, and Paget
disease .
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40. Symptoms
 pain and swelling after a long duration of symptoms.
 Pathological fractures
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41. Work up
 Imaging studies.
 Biopsy
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42. Imaging studies
 X-ray : osteolytic area of destruction with a permeative or moth-eaten appearance.
 CT scan
 MRI
 Bone scan : useful in evaluation of tumor stage and detection of metastasis
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44. 44

45. Treatment
 Medical : Adjunctive therapy, such as radiation treatment and chemotherapy.
 Surgical therapy : surgical resection with a cuff of normal tissue (wide margins) and
reconstruction of the subsequent defect are necessary
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46. Prognosis
 primary fibrosarcoma of the bone has a worse prognosis than osteosarcoma, with a 5-year
survival rate of 65%.
 In high-grade primary fibrosarcoma, the 10-year survival rate is less than 30%.
 Secondary fibrosarcoma is associated with a very poor outcome, the survival rate at 10
years being less than 10%.
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47. Multiple Myeloma
 Definition
 Neoplastic proliferation of a single clone of plasma cells that produces a
monoclonal immunoglobin
 Proliferation often results in osteolytic lesions, osteopenia, and/or pathologic
fractures
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48. Etiology
 Not definitively known
 Relation to radiation, benzene, organic solvents, herbicides, and
insecticides has been proposed
 Chronic inflammatory diseases
 Kaposi’s sarcoma (HHV 8)
 Genetic?
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49. Epidemiology
 1% of all malignant disease
 10% of hematologic malignancies
 More frequent in men
 2X as common in AA
 Median: 66 years
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50. Manifestations
 Bone pain (60%)- back or chest
 Reduction in height
 Weakness and fatigue (32%)
 Weight loss (24%)
 Pallor
 Radiculopathy- thoracic or lumbosacral area
 Infection- 45% of deaths w/i 60 days of diagnosis
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51. Infection
 Impaired lymphocyte function
 Suppression of plasma cell function with hypogammaglobulinemia
 Neutropenia and corticosteroid treatment during chemotherapy
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52. Organisms
 S. pneumoniae
 H. influenzae
 E. coli
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53. Bone Pain
 Radiographic abnormalities in 80%
 Increased osteoclastic activity and decreased osteoblastic activity through
release of cytokines
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54. Imaging
 Skeletal survey: punched out lytic lesions, osteopenia, pathologic fractures.
 Skull, vertebral bodies, thoracic cage, pelvis, proximal humeri and femora
 MRI/CT: commonly used in patients with no explanation for bone pain after
survey
 Bone scanning: not often used (detects osteoblastic activity)
 PET scanning: possible in the future
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55. Renal Disease
 Renal failure common, d/t hypercalcemia or cast nephropathy
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56. Lab Findings
Anemia
Rouleaux
Plasmacytosis
Leukopenia and thrombocytopenia
Monoclonal proteins:
IgG (52%)
IgA (21%)
Bence Jones (16%)
IgD (2%)
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57. Bone Marrow Biopsy
 More than 10% plasma cells
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58. Treatment
 Autologous hematopoietic cell transplantation (HCT)
 Chemotherapy including corticosteroids
 Local radiation of bone lesions
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59. Secondary bone tumors
 2dry bone tumor most frequently occur in patient over 40 year old
 Common site of origin are lung ,prostate, breast & liver
 Common site of deposit are vertebrae, pelvis, femur &ribs
 Clinical presentation is extensive and non specific
 Most lesion present with oateolytic pattern
 2dry of unknown origin account for 24 ./.
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60. origin of bone secondary tumors60
1
LUNG
PROSTATE
BREAST
LIVER
G I T
UNKNOWN
0
5
10
15
20
25
1./.
6
origin of bone 2dry

61. Common site of deposit61
0
10
20
30
40
50
./.
1
ferquency
common site of deposit
spine
plvis
femur
ribs
multi site
Spine 47.7./.
Pelvis 18.2./.
Femur 15.4./.
Ribs 12.6./.
Multi 20.5./.

62. Main symptom :-62
1
pain
fructure
dysfunction
paraplegia
0
10
20
30
40
50
60
main symptom
pain
fructure
dysfunction
paraplegia
Pain 53.3./.
Fracture 10.3./.
Dysfunction 4.9./.
Paraplegia 2.1./.

63. How it reach the bone
 Direct spread from adjacent structure
 Heamatogenous
 Lymphatic
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64. Characteristic of bone 2dry:-
 May be lytic
 May be plastic
 May be mixed
this depend on complex factor the most important of the them :-
 site of primary
 Nature and histology of the tumor
 Local bone environment
the final mechanism is activation of osteoblast or osteoclast
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65. End of Lecture
March 2014
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