Bone Cancer and Sarcoma Flashcards
What are sarcomas?
Tumours of the body’s supportive connective tissue
What can sarcomas arise from?
- Bone
- Muscle
- Cartilage
- Tendon
- Fat
- Synovial tissue
What % of cancers do sarcomas account for?
Less than 1%
What kind of sarcomas develop primarily in children and adolescents?
- Ewing’s sarcoma
- Osteosarcoma
What does the occurrence of Ewing’s sarcoma and osteosarcoma appear to correlate with?
The growth spurt
Are soft tissue sarcomas common in adults?
No, they are rare in adults
What % of childhood cancers do soft tissue sarcomas account for?
Approx. 6%
What is the aetiology of bone sarcomas?
For the majority of bone sarcomas, no specific aetiology has been established, although predisposing factors have been identified
What group has an increased incidence of osteosarcoma?
Children with familial retinoblastoma
What is the genetic abnormality in children with familial retinoblastoma?
13q chromosome deletion
What are radiation-associated sarcomas?
Sarcomas that develop within a radiation field
When do radiation-associated sarcomas develop?
Usually after a latent period of at least 3 years
Of what type are the majority of radiation-induced sarcomas?
Osteosarcomas
What is osteosarcoma often associated with in adults?
- Underlying bone abnormality, such as Paget’s disease or fibrous dysplasia
- Where the bone has been irradiated previously
What genetic association is there with soft tissue sarcomas?
- Neurofibromatosis type 1
- Hereditary retinoblastoma
- Li-Fraumeni syndrome
What exposures increases the risk of soft tissue sarcomas?
- Previous radiation therapy
- Exposure to chemicals such as vinyl chloride monomers
- Herbicides
Where does Ewing’s sarcoma typically arise from?
Axial skeleton
What genetic mutation is Ewing’s sarcoma associated with?
t(11;22) chromosomal translocation
How does a t(11;22) chromosomal translocation increase the risk of Ewing’s sarcoma?
It juxtaposes the EWS and FL11 genes, producing a hybrid transcript that is able to act as a master regulatory protein
Where do most osteosarcomas occur?
In the metaphyseal region of growing long bones, such as the distal femur, proximal tibia, and proximal humerus
What is Ewing’s sarcoma classically described as?
Classically a diaphyseal lesion
Does Ewing’s sarcoma always arise in the diaphysis?
No, it may arise from any region within an involved long bone
Where does Ewing’s sarcoma commonly arise?
- Flat bones of pelvis
- Scapula
Where are primary bone tumours of any histological subtypes extremely rare?
- Spine
- Sacrum
How do bone sarcomas typically present?
Localised pain
What course does the localised pain in bone sarcomas take?
Initially is insidious and transient, but worsens progressively
What other presenting features might be present with bone sarcomas?
- Localised soft tissue swelling, with or without associated erythema
- Joint effusions
- Decreased range of movement of adjacent joints
What might exacerbate the local symptoms of bone sarcomas?
Movement or weight bearing of the involved extremity
Are regional lymph nodes involved in bone sarcomas?
They are rarely involved
What constitutional symptoms may be present with bone sarcomas?
Constitutional symptoms are rare, however fever, malaise, and weight loss can be seen with Ewing’s sarcoma
How do soft tissue sarcomas usually present?
With painless soft tissue swelling
How might soft tissue sarcomas present if located within a body cavity?
- Pain
- Symptoms associated with pressure exerted on nearby structures
How might gastrointestinal stromal tumours present?
- Haematemesis
- Blood in faeces
- Anaemia
How might uterine sarcomas present?
- Vaginal bleeding
- Lower abdominal pain
What age does alveolar rhabdomyosarcoma present?
10-20
What are the most common sites of alveolar rhabdomyosarcoma?
Thigh
What is the primary therapy for alveolar rhabdomyosarcoma?
Neoadjuvant chemoradiation and surgery
What is the overall 5 year survival of alveolar rhabdomyosarcoma?
60%
What age does angiosarcoma present?
50-70
What are the most common sites of angiosarcoma?
- Skin
- Superficial soft tissues
What is the primary therapy of alveolar rhabdomyosarcoma?
Wide excision and adjuvant radiation
What is the overall 5 year survival of angiosarcoma?
15%
What age does embryonal rhabdomyosarcoma present?
0-10
What are the most common sites of embryonal rhabdomyosarcoma?
- Head and neck
- Genitourinary
What is the primary therapy for embryonal rhabdomyosarcoma?
Neoadjuvant chemotherapy and surgery
What is the overall 5 year survival for embryonal rhabdomyosarcoma?
40%
What age does fibrosarcoma present?
20-50
What are the most common sites of fibrosarcoma?
- Thigh
- Arm
- Head and neck
What is the primary therapy for fibrosarcoma?
Wide excision and adjuvant radiotherapy
What is the 5 year survival for well differentiated fibrosarcoma?
90%
What is the 5 year survival for poorly differentiated fibrosarcoma?
50%
What age does leiomyosarcoma present?
45-65
What are the most common sites of leiomyosarcoma?
- Retroperitoneal
- Uterine
What is the primary therapy for leiomyosarcoma?
Wide excision and adjuvant radiation
What is the overall 5 year survival of leiomyosarcoma?
40%
What age does liposarcoma present?
40-60
What are the most common sites of liposarcoma?
- Thigh
- Head and neck
What is the primary therapy for liposarcoma?
Wide excision and adjuvant radiation
What is the 5 year survival for myxoid liposarcoma?
66%
What is the 5 year survival for pleomorphic liposarcoma?
10%
What age does pleomorphic rhabdomyosarcoma present?
40-70
What are the most common sites of pleomorphic rhabdomyosarcoma?
- Thigh
- Upper arm
What is the primary therapy for pleomorphic rhabdomyosarcoma?
Wide excision and adjuvant radiation
What is the overall 5 year survival of pleomorphic rhabdomyosarcoma?
10%
What is the age of onset of synovial sarcoma?
20-40
What is the most common site of synovial sarcoma?
Leg
What is the primary therapy for synovial sarcoma?
Wide excision and adjuvant radiation
What is the overall 5-year survival of synovial sarcoma?
40%
What is rhabdomyosarcoma?
A sarcoma of skeletal muscle
What are the symptoms of rhabdomyosarcoma?
Visible, enlarging mass
What is Kaposi’s sarcoma associated with?
HHV8 infection
It is an AIDS-defining illness
What are the symptoms of gastrointestinal stromal tumours?
- Haemoptysis
- Blood in faeces
- Symptoms of anaemia
What is chondrosarcoma?
Sarcoma of cartilage tissue
Where do chondrosarcomas arise?
Bones in pelvis, shoulder, and limbs
What are the symptoms of chondrosarcoma?
- Painful bony swellings
- Pathological fractures
What is liposarcoma?
Sarcoma of fat tissue
What are the symptoms of liposarcoma?
Enlarging deep-seated mass in soft tissue
Where are liposarcomas found?
- Thigh
- Retroperitoneum
What is angiosarcoma?
Sarcoma of blood vessels
What are the symptoms of angiosarcoma?
Painful swelling
What are gastric leiomyosarcomas?
Sarcomas of the smooth muscle cells in the stomach
What are the symptoms of gastric leiomyosarcoma?
- Early satiety
- Weight loss
- Abdominal pain
What is an endometrial stromal sarcoma?
Sarcoma of the connective tissue cells in the endometrium
What are the symptoms of endometrial stromal sarcoma?
- Passing blood PV
- Lower abdominal pain
What is osteosarcoma?
Sarcoma of bone
What are the symptoms of osteosarcoma?
- Painful bony swelling
- Overlying erythema
- Pathological fractures
Are bone sarcomas visible on plain x-ray?
Many are
What is the purpose of MRI in bone sarcomas?
It is required to assess the extent of lesions as well as the relationship of tumours to nearby structures such as nerves, blood vessels, and joint
What features of bone sarcomas are best demonstrated by MRI?
- Intermedullary tumour extent
- Presence of skip metastases within the bone
What might an isotope bone scan be useful for in bone sarcoma?
Detecting distant osseous metastases
Are there any tumour markers for bone sarcomas?
There are no useful tumour markers, but alkaline phosphatase may be raised in some cases
Is a biopsy required in bone sarcoma?
Yes
Who should a biopsy be undertaken by in bone sarcoma?
A specialist orthopaedic oncologist
Why is it important that the biopsy is taken by a specialised orthopaedic oncologist in bone sarcoma?
To avoid seeding of the needle tract, which could compromise future curative therapy
What is the investigation of choice for soft tissue sarcomas?
MRI
What can identify lung metastases in soft tissue sarcomas?
Chest x-ray or chest CT
Why is it important to identify lung metastases in soft tissue sarcomas?
As these tumours will metastasise by blood-borne spread to the lung
Is biopsy required in soft tissue sarcomas?
Yes
What techniques can be used to obtain a biopsy in soft tissue sarcomas?
- Open biopsy
- Image-guided biopsy
What samples will patients with Ewing’s sarcoma require?
- Biopsy
- Bone marrow aspirate
- Trephine biopsy from distant sites
What is the purpose of a trephine biopsy from distant sites in Ewing’s sarcoma?
To check for metastasis
What blood test findings are often present with Ewing’s sarcoma?
- Anaemia
- Elevated ESR
- Elevated CRP
- Elevated LDH
What treatment should bone sarcomas be considered for rather than amputation?
Limb-sparing surgery with endoprosthetic replacement of the resected bone
What is a good prognostic factor for bone sarcoma when surgery is combined with adjuvant or neoadjuvant chemotherapy?
Positive response to chemotherapy
What must be ensured when surgery is being used in the management of bone sarcomas?
That the entire local tumour is removed, with wide clear margins
What % of osteosarcoma surgery is limb sparing?
Approx 95%
How can muscle be reconstructed after bone sarcoma surgery?
Using transfers from other parts of the body
What is the goal of using muscle transfers in bone sarcoma surgery?
To produce a functioning limb
When can radiotherapy be used for bone sarcomas?
- Non-resectable tumours
- Palliation of bone metastases
How can patients with metastatic bone sarcomas be managed?
- Palliative chemotherapy
- Radiotherapy
- Amputation
What is the purpose of the management of metastatic bone sarcomas?
Symptom control
What management is required for patients with soft tissue sarcomas?
Surgery
What is the aim of surgery in soft tissue sarcomas?
Complete excision of the tumour with clear margins
What might be required to achieve complete excision of the tumour with clear margins in soft tissue sarcomas?
En bloc dissection with removal of the muscular compartments
What is the purpose of en bloc dissection in soft tissue sarcomas?
It gives the lowest risk of local recurrence
In what % of cases of soft tissue sarcomas is limb amputation necessary?
5%
What treatment options are of limited use in soft tissue sarcomas?
Adjuvant or neoadjuvant chemotherapy or radiotherapy
What management do GIST respond to?
Tyrosine kinase inhibitors such as imatinib
What is the advantage of tyrosine kinase inhibitors in GIST?
They may significantly increase the median survival time in patients with disease unresponsive to conventional chemotherapy
What does the treatment of Ewing’s sarcoma require?
A multi-disciplinary approach
What does the treatment of Ewing’s sarcoma usually consist of?
Chemotherapy followed by surgical excision and then further chemotherapy
What is the 5 year survival rate of localised bone sarcomas?
60-70`5
What is the 5 year survival rate of metastatic bone sarcoma?
10-30%
What is the 5 year survival rate of soft tissue sarcomas?
70%
What is required with survivors of soft tissue sarcomas?
Careful monitoring
Why is careful monitoring required with survivors of soft tissue sarcomas?
As patients with solitary pulmonary metastasis can be cured by surgical resection
What is the 5 year survival rate of localised Ewing’s sarcoma?
55-60%
What is the 5 year survival rate of metastatic Ewing’s sarcoma?
10-20%
