7- Bone cancers Flashcards
Bone cancers background
Metastatic bone cancer is much more common than Primary (osteosarcoma, chondrosarcoma, fibroscarcoma, Ewings)
Primary cancers:
- Rare
- Start in the bone known as bone sarcomas
- Sarcomas are cancers which start in the bone, muscle, fibrous tissue, blood vessels, fat tissue and develop anywhere in the body
types of primary bone cancer
Primary tumours arise from cells which constitute the bone and can divided into benign and malignant types
Bone forming tissues: Osteosarcoma
Cartilage forming tissues : Chondrosarcoma
Fibrous tissue: Fibrosarcoma
Marrow tumours: Ewings, Myeloma
Bone metastasis
Most common primary sites:
- Breast cancer (lytic)
- Prostate (sclerotic)
- Lung cancer (lytic)
Most common sight for bony metastases: the Spine
Management:
- tends to be palliative
- dexamethasone for MSCC
- radiotherapy
- prophylactic nailing of certain long bones if pathological fractures likely
Osteosarcoma
Background
- Most common primary bone cancer in children
- Bimodal age distribution
- Location:
1) distal femur
2) proximal tibia
Types of osteosarcoma
depend on how well-differentiated the cells are when oncogenic events occur
- Osteoblastic- most highly differentiated
- Chrondroblastic
- Fibroblastic- least highly differentiated
Risk factors for osteosarcoma
- 10-20yo
- Usually tall for age
- Males>females
- p53 mutation (Li-Fraumeni syndrome)
- RB1 mutation (familial retinoblastoma)
- Prior radiation therapy or chemotherapy
- Pagets/ osteochondromas
pathophysiology of osteosarcoma
Mutation occurs in rapidly dividing osteoblasts e.g. pubertal growth spurt
presentation of osteosarcoma
- Persistent bone pain
- Worse at night
- May wake them up
- Others:
o Bone swelling
o Palpable mass
o Restricted joint movement - Weight loss, fatigue, headache
Red flags for osteosarcoma
- Bone pain that persists and gets worse
- Wakes up from sleep
- Swelling and redness over bone
- Weak bone that fractures easily
Diffferentials: osteomyelitis or multiple myeloma
investigations for osteosarcoma
VERY URGENT XRAY within 48 hours
If Xray suggest sarcoma -> VERY URGENT SPECIALIST ASSESSMENT within 48 hours
BLOOD TESTS
- Raised alkaline phosphatase (ALP)
Other investigations
- MRI
- Bone scan
- PET
- Bone biopsy
E.g. needle biopsy or surgical biopsy during tumour excision
x-ray osteosarcoma
- Poorly defined lesion in bone, with destruction of normal bone and ‘fluffy’ appearance
- Periosteal reaction (irritation of bone lining)- “sun-burst” appearance
- Soft tissue mass
management of osteosarcoma
- Aggressive surgical resection of lesion = LIMB AMPUTATION
- Adjuvant chemotherapy improves outcomes
MDT support
o Paediatric oncologists and surgeons
o Specialist nurses
o Physiotherapy
o OT
o Psychology
o Dietician
o Prosthetics and orthotics
o SS
complications of osteosarcoma
- Leading cause of death in the young
- Pathological fractures
- Metastatic disease
Ewings sarcoma
Background
- Second most common primary bone cancer in children
- Paediatric malingnancy
- Malignancy of neural crest cell
- Most commonly occurs in bone, rare cases in soft tissue (extraosseous ewings)
- Requires aggressive treatment
- Flat bones
o Tibia
o Fibula
o Knee joint
o Femur
o Pelvis
o Ribs
risk factors for ewings sarcoma
- Males
- 10-20yo
- Caucasian
- Rare in those older than 30yo
Pathophysiology for Ewings sarcoma
- Ewing sarcoma is a small, round blue cell tumour
- Due to uncontrolled cell turnover and tumour formation
Presentation Ewings sarcoma
- Bone pain: can be misinterpreted as growing pain or sports injuries
- Restricted movement of joint
- Fatigue, weight loss, fever
- Fracture susceptibility
- Red flags
o Unexplained bony lump
investigations for Ewings sarcoma
URGENT X-RAY WITHIN 48 HOURS
If x-ray suggest cancer- specialist assessment within 48 hours
Bloods: FBC, UandE, LFTS, ESR< CRP, ALP and bone profile
Anaemia, leucotosis, elevated WBC, elevated ESR, elevated LDH, elevated ALP and elevated CRP
Imaging
- Initial XRAY
- MRI, CT, PET- staging
Bone biopsy- definitive
Ewings sarcoma x-ray
Lytic lesion with periosteal reactions , producing layers of reactive bone (periosteal reaction) leading to characteristic “onion skin” appearance.
management of Ewings sarcoma
Needs to be aggressive: chemo, surgery radiotherapy
Chemotherapy
- Shrinks tumour prior to surgery and destroy remaining tumour cells which may have spread from primary site
Surgery
- Limb sparing surgery
–>Resection of tumour and either a metal implant or autologous bone graft
- If tumour spread to widely- amputation performed
Radiotherapy
- Not used in every patient
- Can be used to shrink tumour prior to surgery
Complications of Ewings sarcoma
- Metastatic disease
o Lungs
o Bones
o Bone marrow - Recurrence
Chondrosarcoma
Background
- Most common in adulthood
- Starts in the early forms of cartilage cells
- Can start anywhere there is cartilage
- Most commonly Axial skeleton
o Pelvis
o Legs
o Arms/knee
o But also: trachea, larynx, chest wall, shoulder blades, ribs or skull) - Can arise from chondromas
- Locally aggressive
Risk factors chondrosarcoma
- Rare in people younger than 20
- Risk goes up as people get older
- Enchodromas – a type of benign bone tumour that begins from cartilage and usually affects hands
presentation of chondrosarcoma
Presentation
- Large mass on affected bone
- Feeling of pressure around mass
- Pain that increases gradually over time
- Pain usually worse at night and may be relieved by taking NSADIs
- Local swelling
Investigations for chondrosarcoma
- Xray- popcorn calcification (lytic lesion)
- CT
- MRI
- Biopsy
- PET
grading of chondrosarcoma
Grading
* Low-grade (grade I) chondrosarcomas, also called atypical cartilaginous tumors, tend to grow the slowest and are very unlikely to spread.
* Intermediate-grade (grade II) chondrosarcomas are slightly more likely to spread.
* High-grade (grade III) chondrosarcomas are the most likely to spread.
management of chondrosarcoma
Management
* Surgery. Removal of the tumor. If the tumor is on an arm or leg, the surgeon will try to save the limb. In some cases, amputation might be needed.
* Physical therapy. This treatment helps to regain strength and use of the affected area after surgery
* Tend to be chemotherapy and radiotherapy resistant
High-grade undifferentiated pleomorphic sarcoma (UPS) of bone
This cancer was previously known as malignant fibrous histiocytoma (MFH) of bone. Undifferentiated pleomorphic sarcoma (UPS) most often starts in soft tissues (connective tissues such as ligaments, tendons, fat, and muscle). It’s rare in bones, but when it does occur, it usually affects the legs (often around the knees) or arms. This cancer most often occurs in elderly and middle-aged adults. It’s rare in children. It tends to grow locally, but sometimes it can spread to distant parts of the body, like the lungs.
Fibrosarcoma of bone
Fibrosarcoma is another type of cancer that develops more often in soft tissues than it does in bones. It usually occurs in middle-aged adults. Bones in the legs, arms, and jaw are most often affected.
Giant cell tumor of bone
This type of primary bone tumor can be either benign (not cancer) or malignant. The benign form is more common. These tumors are most common in people in their 20s and 30s.
Giant cell bone tumors typically affect the legs (usually near the knees) or arms. They don’t often spread to distant parts of the body, but after surgery they can come back (even more than once) in the place where they started. With each recurrence, the tumor becomes a bit more likely to spread to other parts of the body (most often to the lungs). Rarely, a malignant giant cell bone tumor spreads to other parts of the body without first recurring locally.
Chordoma
This uncommon type of bone tumor occurs in the bones of the spine, most often at the bottom of the spine (sacrum) or the base of the skull. It develops most often in adults older than 30. It’s about twice as common in men as in women. Rarely it can develop in children, as well.
Chordomas tend to grow slowly and often do not spread to other parts of the body. They often come back in the same area if they are not removed completely. If they do spread, they most often go to the lymph nodes, lungs, or liver.
summary of primary bone cancer
