Bone Malignancies

Bone Maligancies
Can be either Primary or Secondary, though the latter is more common.
Osteosarcoma
- Most common Primary bone cancer of childhood ~about 20% of all bone
tumors, and 2.4% of pediatric cancers making it the 8 th most common
childhood malignancy.
- Mesenchymal in origin
Associations
- Hereditary retinoblastoma
- Li-Fraumeni syndrome
- Bloom Syndrome
- Werner syndrome
Secondary associated in patients with

- Pagets disease
- Electrical burns
- Trauma
- Exposure to Beryllium
- Orthopaedic prosthetics.
- Bone infarction and infection
Osteosarcomas can be further categorised by anatomic relationship to

the bone 3,8:
intramedullary/central: ~80%
surface: ~10-15%
intracortical osteosarcoma: rare
extraskeletal osteosarcoma: ~5%
Epidemiology
Bimodal peaks – between 0-14 and >65y(which is more likely from sec. causes)
African ethnicity most affected with an incidence of 6.8 cases/yr/million people
Higher incidence in males than females.
Pathophysiology
Primary occurs near the metaphysis of long bones, with the commonest location
being the femur (commonly the distal aspect) then tibia (commonly proximal)
and the humerus (Proximal portion)
Secondary have a much wider distribn, with a higher incidence in the flat bones –
the pelvis in particular.
History and Exam

- The Sx are gradual, most common being bone pain with activity +/- h/o
trauma
- There’s maybe an ass with swelling,
- Physical exam – palpable mass maybe tender and warm +/- overlying
pulsation
 Joint involvement with red ROM
 Local or regional Lymphadenopathy
 Resp findings with metastatic forms.
Evaluation according to the National. Comprehensive Cancer Network’s
2020 guidelines initial evaluation of Osteosarcoma.
1. Clinical Hx and exam
2. Labs
a. LDH
b. ALP – high due to increased osteoblastic activity, very high levels
poor prognostic indicator; evaluate levels during Rx to assess the
therapy success.
3. Imaging
a. Radiographs
i. Medullary and cortical bone destruction
ii. Wide zone of transition
 The zone of transition is the point at which normal
bone can be distinguished from abnormal bone.
iii. Aggressive periosteal reaction
1. Sunburst appearance
2. Codmans Triangle
iv. Soft tissue mass
b. CT
i. Used for biopsy and staging purposed.
c. MRI
i. For accurate local staging and assessment for limb spating
resection
Treatment and prognosis

- Aggressive surgical resection with +/- amputation followed by chemo
- The outcome dependant on age, sex, size, and rtpe but the most
importanct factor is the degree of necrosis post induction chemotherapy
i.e 90% necrosis is ass with a much better prognosis.
EWING SARCOMA
- Aggressive tumor of adolescents and young adults – 10-15% of all
sarcomas
- Characterized by non-random x-somal translocations producing fusion
genes – t(11:22).
Location
Most commonly affected sites – pelvis, axial skeleton and femur, however, can
affect any bone or soft tissue.
Epidemiology
Rare – 3/1,000,00
2nd most common primary malignant bone tumor in children - ~3% of all
paediatric malignancies.
Males:Females -1.5:1, and ~90% are <20y with a peak age of 10-15
Presentation
Hx – Symptoms for >6 months
Sx
- Pain worse at night
- Swelling and erythema
- Mass
- Fevers*
- Weight loss*
(* - metastatic disease)
- Back pain
Exam
- Swelling with local tenderness.
Evaluation
- Radiograph
o moth-eaten lesions, Codman triangle of
periosteum
o Onion-skin periosteal reaction
It can be difficult to differentiate between Ewing sarcoma and
osteomyelitis on CT and ragiographs.
- MRI
o Assess soft tissue extension
o Marrow involvement
o Assess response to Rx
- CT
o Important for staging
Rx
- Systemic chemotherapy is the main stay followed by local therapy with
radiotherapy and surgery
- Nonmetastatic disease has a 5-year survival rate of 75% to 80%, while
metastatic disease is around 30%.
Chondrosarcoma
- the third most common primary malignancy of bone after myeloma and
osteosarcoma
- tumor of adulthood and older age >50y
- The most common locations of involvement of chondrosarcoma include
o the bones of the axial skeleton (pelvis, scapula, sternum, and ribs)
followed by the proximal femur and proximal humerus
Hx
Local swelling and pain are the most common presenting symptoms. The
symptoms are usually of long duration (months to years).
Other symptoms are dependent on tumor grade and location.
Imaging
X rays – may show the following
- Lytic lesions in ~50%
- Intralesional calcification
- Endosteal scalloping
- Moth eaten appearance in high grade
MRI
- Useful for determining the size of the tumor soft tissue involvement
CT
- Most useful for low grade cartilage neoplasms to determine the cortical
involvement.
Treatment
o Intra-lesional curettage
 For grade 1 lesions in the extremities
o Wide surgical excision
 Grade 2 or 3 lesions
 Any lesion in the pelvis
o Wide surgical excision plus multiagent chemo
 Mesenchymal chondrosarcoma
Prognosis
Grade I – 90%
Grade II- 60-70%
Grade III – 30-50%
De-differentiated - <20%
Multiple Myeloma
- Neoplastic proliferation of plasma cells
- Patients typically are >40 years
- Dx – BM biopsy
- Rx – Chemotherapy and radiation
Most common primary bone malignancy, occurs in 3-4:100,000

Affects M>F, and 2x more common in African Americans
Presentation
Sx – Localised bone pain – usually spine or ribs
- Pathologic #
- CRAB
Imaging
Radiograph
- Multiple punched out Lytic lesions
MRI
- More sensitive than XR for pelvis and spine
Labs
FBC, Ca, SPEP, B2 microglobulin
Rx
- Non-operative
o Annual surveillance – for asymptomatic myeloma
o Chemotherapy +/- stem cell transplant +/- bisphosphonates
- Operative
o Complete or Impending fracture
o Life expectance >3 months.
Bone Metastases.
A frequent site for metastasis, and typically associated with a poor prognosis.
Majority of the Responsibility of the mets to the bones are from the cancers of
the breasts, prostate
Bone metastases are a major cause for morbidity, characterized by severe pain,
impaired mobility, pathologic fractures, spinal cord compression, bone marrow
aplasia and hypercalcemia
The distribution of skeletal metastases roughly mirrors the distribution of red
marrow, presumably reflecting increased blood flow in red marrow compared to
yellow marrow
The conversion of red to yellow marrow progresses from distal to proximal of the
extremities, so first hands and feet, then forearms/lower legs, then
humeri/femora, then pelvis/spine.
The metastasis can be classified as osteolytic, osteoblastic or mixed.

- Lytic – destruction of bone
o Seen in multiple myeloma (MM), renal cell carcinoma, melanoma,
non-small cell lung cancer, non-hodgkin lymphoma, thyroid cancer
or langerhans-cell histiocytosis.
- Blastic – deposition of bone
o present in prostate cancer, carcinoid, small cell lung cancer,
Hodgkin lymphoma or medulloblastoma
Pathologic fractures occur in 10-30% of all cancer patients, with proximal parts of
the long bones being the most frequent fracture site, and the femur accounting
for over half of all cases
Mirels’ score
Used to predict the highest risk of pathological fractures among long bones
affected by mets.
Based as shown.
Score 1 2 3
Site Upper Limb Lower Limb Trochanteric
Pain Mild Moderate Functional
Lesion Blastic/sclerotic Mixed Lytic
sclerotic/lytic
lesion
Size <1/3 of bone 1/3-2/3 of bone >2/3 of bone
diameter diameter diameter
Max score of 12, a score of ≥9 suggests prophylactic fixation, a score of 8 Rx

based on clinical judgement and <8 clinical mgmt. and radiotherapy
Has a sensitivity of ~90% on predicting a fracture, but with a specificity of

~35%1
(1 Piccioli, A., Spinelli, M. S., & Maccauro, G. (2014). Impending fracture: A difficult
diagnosis. Injury, 45(S6), S138–S141.
https://doi.org/10.1016/j.injury.2014.10.038)

Bone Malignancies

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Bone Malignancies

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Bone Maligancies

Can be either Primary or Secondary, though the latter is more common.

Secondary associated in patients with

Osteosarcomas can be further categorised by anatomic relationship to

History and Exam

Treatment and prognosis

Most common primary bone malignancy, occurs in 3-4:100,000

The metastasis can be classified as osteolytic, osteoblastic or mixed.

Max score of 12, a score of ≥9 suggests prophylactic fixation, a score of 8 Rx

Has a sensitivity of ~90% on predicting a fracture, but with a specificity of

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