LECTURE 9 - finished Flashcards
Define osteosarcoma
A highly malignant osteoid producing tumour comprised of primitive osteoblasts.
What are some risk factors associated with the development of osteosarcoma
OverSeas Ricky Fucks OG RAP
Osteogenesis imperfecta
Genetic factors:
- germ-line mutations the p53 gene
- Patients with retinoblastoma defects
Radiation
Areas of rapid bone growth
Paget’s disease
What do osteosarcoma cells look like histologically?
OG RAP 90’s high intensity tunes on the SpinDial (spindle)
The cells are spindle shaped with variable cytologic atypia (e.g. pleomorphism & ↑ed mitotic activity)
90% of all osteosarcomas are high grade intramedullary types
In terms of histology, how is osteosarcoma diagnosed?
Need to see presence of:
• Osteoid
• New bone
Where does osteosarcoma common metastasise to?
OverSeas spread via the Down Low
Distant bone
Lung
What is the most common primary bone malignancy? What % of primary bone malignancies does it account for
Osteosarcoma
22%
Osteosarcoma age of onset?
1st peak = 10-30
2nd peak = > 60 and usually arises from a benign or low grade bone lesion
Gender distribution of osteosarcoma
M:F = 1.5:1
Describe the pathology of osteosarcoma
OverSeas Playas Alternate Demoing and Recording, spreading to the people and the softies, may even cross the plates. Their extra spread can have new beats involved too. (lame as fuck but helps)
Osteosarcoma destroys & replaces bone and we can observe alternating areas of:
• bone loss
• abnormal new bone production (esp. at periosteal margins secondary to periosteal lifting)
It quickly spreads outwards to the periosteum & surrounding soft tissues.
Commonly the extraosseous part of the lesion has new bone extending into it.
It may cross the epiphysial plate.
What is the distribution of osteosarcoma?
Any bone may be affected, but usually occur in:
- Long bone metaphyses
• Knee
• Proximal humerus
What is the clinical picture of osteosarcoma?
The most common presentation is pain and a mass occurring near a joint.
• Pain
- Usually the presenting symptom
- Constant
- Worse at night
- Gradual increase in severity
- Lump/swelling (increasing in size)
- Swelling & erythema of overlying tissue due to ↑ed tumour vascularity
How are osteosarcomas diagnosed?
The diagnosis is suspected from the clinical presentation, and confirmed by imaging, and other diagnostic procedures.
Important lab findings in osteosarcoma
- ↑ ESR
* ± ↑ serum alk. phosphatase
What are some radiological features of osteosarcoma
OverSeas Rad Fucks TRI P PE D down a BREACHed HOLe.
• codman’s TRIangle (reactive periosteal bone formation)
• Pulmonary metastases
• Poor definition of Endosteal margin
• Dense osteoblastic areas
• cortical Breaching (sunray/sunburst sign)
• Hazy OsteoLytic areas
Some ddx for osteosarcoma?
OverSeas dicks drink SCOBE:
- Stress fracture (pathology may look similar)
- cost traumatic Callus or myositis ossificans
- Osteomyelitis
- Benign bone tumour
- Ewing’s sarcoma
Prognosis for osteosarcoma
Localised osteosarcoma is now associated with a 5 year survival rate of ≈ 60%
Define chondrosarcoma
A collective term for a group of malignant mesenchymal tumours mainly made up cartilage cells that produce cartilage matrix
What is the second most common primary malignant tumour of bone?
Chondrosarcoma
Which age group does Chondrosarcoma usually affect
30-50
Gender distribution of Chondrosarcoma?
M:F = 1.5:1
What are the classifications for Chondrosarcoma?
Primary chondrosarcoma
• uncommon
• appear centrally or peripherally in the bone
• metaphyses of chondral bones are usually affected
• found in children
Secondary chondrosarcoma
- Arise from benign cartilage defects e.g.
• Osteochondroma (=peripheral)
• Enchondroma (=central)
What is the pathology of chondrosarcoma
- Slow growing tumour
- Can become very large
- Derived from cartilage
- Produces cartilage
- Late metastasis
What is the most common distribution of chondrosarcoma?
Most common in the femur, humerus, ribs and on the surface of the pelvis
What is the clinical picture of chondrosarcoma
Cholas can fuck T-Pains Pelvic ROM PS
2 pains:
• local PAIN (deep, dull ache)
• PAIN at night
• PELVIC tumour ssx:
- urinary frequency or obstruction
- may present as “groin muscle pulls”
- nerve dysfunction: esp. lumbosacral plexus, sciatic or femoral nerves)
- ↓ed ROM when tumour is near a joint
- Pathological fracture
- Swelling
How are chondrosarcomas diagnosed
The diagnosis is suspected from the clinical presentation, and confirmed primarily by imaging, and biopsy
What are the radiological features of chondrosarcoma?
- Large lesion (> 5 cm)
- Radiolucent area
- Endosteal scalloping
- Stippled calcification
- Globular mass on surface of bone
- Periosteal new bone formation
DDx for chondrosarcoma
- Chondroma
- Fibrosarcoma
- Fibrous dysplasia
- Osteosarcoma
Prognosis chondrosarcoma
The overall 5-year survival rate is 48-60%
Define fibrosarcoma
A tumour of mesenchymal cell origin.
Composed of malignant fibroblasts in a collagen matrix
Epidemiology of fibrosarcoma
- Rare in bone
- <5% of all primary tumours of bone
- Most common in adults 30-60 yrs
- M:F ≈1:1
Classifications of fibrosarcoma
Primary:
- Arise from bone
- Arise from connective tissue
May originate in bone or invade bone from nearby connective tissue
Secondary: - Arise at the site of pre-existing bone pathology e.g: • Paget's disease • fibrous dysplasia • irradiated giant cell tumour • bone infarct • chronic osteomyelitis
Outline the pathology of fibrosarcoma
Fibrosarcoma is a fibroblastic connective tissue tumour i.e.. composed of large fibroblasts.
Fibrosarcoma cells produce a collagen matrix but do not produce osteoid or chondroid
Distribution of fibrosarcomas
Occurs in the metaphysis or metadiaphysis of long bones.
Usually occur in the lower limbs, esp. femur & tibia
Most common sites: Distal femur and proximal tibia followed by the pelvis
What is the clinical picture of fibrosarcoma usually due to?
- Localised painful mass
* Pathological fracture
Radiological features of fibrosarcoma
Rad Fucks fib POEMS
- Periosteal reaction is seen with cortical destruction
- Osteolytic lesion
- Extension into soft tissue (common)
- Margins: well-defined or ragged
Prognosis of fibrosarcoma
5-year survival rate: ≈65%
Ewings sarcoma definition
Ewings sarcoma is a primary malignant tumour of bone. The small, round tumour cells resemble those of lymphoma or neuroblastoma.
Epidemiology of Ewing’s Sarcoma
- ≈10-15% of all bone sarcomas
- Median age is 15 years
- Peak incidence in the second decade of life
Where does ewings sarcoma usually metastasise to?
Ewing’s sarcoma is a malignant neoplasm which commonly metastasises to:
• Lungs
• Other bones
• Bone marrow
What are the 2 main affected regions in ewings sarcoma?
- Lower extremity
* Pelvis
What is the clinical picture of ewings sarcoma?
Ewan, dont PALP peoples BACK PAIN • Palpable mass • Asymmetrical neurological ssx • Localised pain • Pathologic fracture
• Back pain may indicate a paraspinal, retroperitoneal, or deep pelvic tumour
What manifestations can indicate metastatic disease?
- Systemic symptoms (fever &weight loss)
- Petechiae/purpura
- Pulmonary ssx indicating lung metastases
How is ewings sarcoma diagnosed?
The diagnosis is suspected from the clinical presentation, & confirmed primarily by imaging, biopsy & blood tests
Typical lab tests seen in ewings sarcoma?
- ↑ed Serum ALP
* Hypercalcaemia (=>widespread disease)
What are some radiological manifestations of ewings sarcoma?
- large
- poorly marginated
- > 80% extend into adjacent soft tissues
What are 3 common radiological findings in ewings sarcoma?
Ewans rad? PLS
Common findings include:
• permeative: 76%
• laminated (onion skin) periosteal reaction: 57%
• sclerosis: 40%
DDx for ewings sarcoma
- Osteomyelitis
- Lymphoma (Reticulum cell sarcoma)
- Small cell osteosarcoma
- Metastatic Ca
