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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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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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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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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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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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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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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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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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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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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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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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./.
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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