Soft Tissue Malignancies FRCR CO2A Flashcards
What are soft tissue sarcomas?
Mesenchymally derived malignant tumours that may arise anywhere in the body.
What is the incidence of soft tissue sarcomas?
Approximately 45 per million.
What is the overall survival rate for soft tissue sarcoma at 5 years?
Approximately 50%.
What is the primary treatment for soft tissue sarcoma?
Surgical treatment.
What is the role of adjuvant radiotherapy in soft tissue sarcoma?
Offered where there is a significant risk of local relapse.
What percentage of patients with intermediate or high-grade tumours develop metastatic disease?
50%.
What is the usual spread pattern for soft tissue sarcomas?
Haematogenous spread to the lungs or liver.
What chemotherapy agent is commonly used for palliative treatment of soft tissue sarcoma?
Doxorubicin.
What is the typical response rate to chemotherapy for soft tissue sarcoma?
Around 20%.
Name two classifications for soft tissue sarcomas.
ICD-O-3 and WHO classifications.
What are some common subtypes of soft tissue sarcomas?
- Leiomyosarcoma
- Liposarcoma
- Fibrosarcoma
- Synovial sarcoma
- Vascular sarcoma
- Nerve sheath tumours
- Malignant phyllodes tumours.
What is the age-standardised incidence rate trend for soft tissue sarcoma in England from 1985 to 2009?
Increased from 34 cases per million to 48 cases per million.
At what age is the peak incidence of soft tissue sarcomas observed?
Gradually increases with age, peaking in those aged 30 and above.
What percentage of sarcomas are diagnosed in patients under 30 years of age?
16%.
What are the common sites for soft tissue sarcomas?
- Limbs (23%)
- Connective tissues of the trunk (13%).
What risk factors are associated with soft tissue sarcoma?
- Exposure to radiation
- Vinyl chloride monomer
- Dioxins and chlorophenols.
What is the Trojani grading system used for?
Grading sarcomas based on differentiation, mitoses, and necrosis.
How do sarcomas typically spread?
By local invasion or haematogenous spread.
What is the significance of a soft tissue mass greater than 5 cm?
Should be referred to a diagnostic centre as a suspected soft tissue sarcoma.
What type of biopsy is recommended for suspected soft tissue sarcomas?
Wide-needle biopsy.
What imaging is recommended for confirmed cases of sarcoma?
CT thorax and local imaging, usually by MRI.
What is the recommended TNM classification for soft tissue sarcomas?
T1, T2, N0, N1, M0, M1.
What is the aim of treatment for soft tissue sarcoma?
Maximise the chance of a cure while minimising treatment-related morbidity.
What is the typical margin for excising a primary soft tissue sarcoma?
1 cm margin.
What is the controversy surrounding the timing of radiotherapy?
The optimum time for RT is still debated.
What is the significance of local control in soft tissue sarcoma treatment?
Quality of life and limb function depend on local control.
True or False: Chemotherapy is the primary treatment for soft tissue sarcoma.
False.
What is the primary context for the use of preoperative radiotherapy (RT)?
Preoperative RT is becoming increasingly common in the UK, although its optimum timing is controversial.
How has preoperative RT been compared to postoperative RT?
Preoperative RT has not been compared with surgery alone in a randomized trial but has been compared with postoperative RT.
What significant finding was reported after 50 Gy of preoperative RT?
A statistically significant increase in wound healing problems was found.
What has continued follow-up shown regarding overall survival with preoperative RT?
There is a marginal but significant improvement in overall survival with preoperative RT.
What are the typical dose and fractionation for phase 1 radiotherapy?
The dose is 50 Gy in 25 fractions over 5 weeks.
What is the target volume for phase 1 CTV in limbs?
The phase 1 CTV typically comprises the operative bed plus 5 cm longitudinally and 2 cm radially.
What is the purpose of limb immobilization during RT planning?
To ensure reproducibility of the set-up.
What is the significance of using a photograph during limb positioning for RT?
It helps record the exact position for treatment.
How does preoperative RT planning differ from postoperative RT planning?
The tumour is still in situ and there is typically no scar in preoperative planning.
What is the recommended dose for phase 2 radiotherapy?
The dose is 10–16 Gy in 5–8 fractions over 1–1.5 weeks.
What is the standard first-line chemotherapy for soft tissue sarcoma?
Single-agent doxorubicin at a dose of 60–75 mg/m2.
True or False: Doxorubicin has been shown to improve survival compared to best supportive care.
False.
What combination of drugs was tested in the first EORTC study?
Cyclophosphamide, vincristine, doxorubicin, and dacarbazine (CYVADIC).
What was the conclusion of the second EORTC study regarding ifosfamide?
It showed no difference compared to no chemotherapy, but local control rates were higher than expected.
What is the peak age for osteosarcoma incidence?
The peak age is 15, with a second peak in the elderly.
What are some genetic predispositions associated with osteosarcoma?
Hereditary retinoblastoma, Rothmund–Thomson syndrome, and Li–Fraumeni syndrome.
What is the average annual incidence of bone sarcoma in England from 1985–2009?
Approximately 7.9 per million persons.
What is the most common primary bone malignancy in adults?
Myeloma.
What is the five-year relative survival rate for soft tissue sarcoma in England?
Around 54%.
Fill in the blank: Trabectedin is approved by NICE when treatment with _______ and ifosfamide has failed.
anthracyclines.
What is the common treatment approach for metastatic disease in soft tissue sarcoma?
Metastectomy, usually pulmonary.
What is a notable feature of the epidemiology of Ewing sarcoma?
It shows a broader age range but becomes increasingly uncommon beyond age 30.
What type of radiotherapy is being compared in the IMRiS trial?
Intensity Modulated Radiotherapy (IMRT) and conventional 3D conformal planning.
What is the role of gemcitabine and docetaxel in the treatment of uterine leiomyosarcoma?
They have shown high response rates.
What is the significance of the phase II trial conducted by the EORTC?
It compares preoperative radiotherapy plus surgery versus surgery alone in retroperitoneal sarcoma.
What is a common risk factor for the development of osteosarcoma?
Paget’s disease of bone.
What is the incidence of Ewing sarcoma among different ethnicities in the US?
It is less common in Black and Chinese populations.
What type of cancer do chordomas represent?
Rare cancers derived from notochordal remnants.
What is the primary source for further information on clinical trials related to soft tissue sarcomas?
http://csg.ncri.org.uk/.
What are the known risk factors for Ewing sarcoma?
There are no known risk factors for Ewing sarcoma, though it is less common in US populations of Black and Chinese ethnicity, suggesting genetic factors might play a role.
What benign tumors can lead to chondrosarcoma?
Chondrosarcoma may arise from benign cartilaginous tumours such as osteochondromas and chondromas.
What is Maffucci’s syndrome?
Maffucci’s syndrome is a combination of multiple enchondromas that usually affect the hands and angiomas.
What are chordomas?
Chordomas are rare cancers that arise from embryonic notochord remnants along the length of the neuraxis.
Where does osteosarcoma commonly metastasise?
Osteosarcoma commonly metastasises to the lung and less commonly to bone.
What are skip metastases?
Skip metastases are non-contiguous deposits in the same or neighbouring bone.
What percentage of Ewing sarcoma patients present with metastases?
Ewing sarcoma presents with metastases in around 25% of patients.
What is the typical cure rate for Ewing sarcoma before chemotherapy?
The typical cure rate for Ewing sarcoma before chemotherapy is around 5%.
What is the commonest symptom of a primary bone tumour?
The commonest symptom of a primary bone tumour is non-mechanical or night pain.
What imaging is the correct first investigation for a suspected primary bone tumour?
A plain X-ray is the correct first investigation.
What is Codman’s triangle?
Codman’s triangle is a characteristic radiographic finding in osteosarcoma.
What is required for the investigation of bone sarcoma?
A biopsy is essential for the investigation of bone sarcoma.
What staging systems are used in bone cancer?
The Enneking system and the TNM system are used in bone cancer staging.
What is the preferred treatment of chondrosarcoma?
Complete surgical excision is the preferred treatment of chondrosarcoma.
What is the recommended dose of radiotherapy for chordoma?
Radiotherapy may be given ideally to a dose of 70 Gy for chordoma.
What is the focus of sandwich chemotherapy?
Sandwich chemotherapy focuses on combining neoadjuvant and postoperative chemotherapy.
What agents are commonly used in the treatment of osteosarcoma?
Cisplatin and doxorubicin are commonly used in the treatment of osteosarcoma.
What is the role of immunotherapy in osteosarcoma?
Immunotherapy in osteosarcoma remains contentious, with some studies showing potential benefits.
What are the prognostic factors associated with poorer outcomes in osteosarcoma?
Raised lactate dehydrogenase, alkaline phosphatase, presence of metastatic disease, poor histologic response, pelvic tumour site, male gender, and age greater than 18.
What was the survival rate for Ewing sarcoma before the use of multi-agent chemotherapy?
The survival rate for Ewing sarcoma before the use of multi-agent chemotherapy was only 6%.
What combination of drugs was first reported for Ewing sarcoma treatment?
Cyclophosphamide and vincristine were first reported for Ewing sarcoma treatment.
What is the significance of the first Intergroup Ewing’s Sarcoma Study (IESS-1)?
The IESS-1 showed a 5-year disease survival of 60% with the addition of doxorubicin.
Fill in the blank: The addition of _____ improved outcomes in Ewing sarcoma treatment.
doxorubicin
What is the current standard treatment for osteosarcoma?
The current standard treatment for osteosarcoma is with MAP chemotherapy.
What percentage of patients with localised disease will relapse?
Around 30% of patients with localised disease will relapse.
What is osfamide used for?
It is used in the treatment of Ewing’s sarcoma
Referenced in Craft et al., 1998.
What was the outcome of the first American Intergroup Ewing’s trial?
Survival in localized disease improved with ifosfamide and etoposide, but remained poor at 22% in metastatic disease
Referenced in Grier et al., 2003.
What was investigated in the second American Intergroup trial?
Dose intensity with randomization to treatment protocols of either 30 or 48 weeks
Referenced in Womer et al., 2012.
What was the improvement in 5-year event-free survival with 2-weekly chemotherapy?
Increased from 65% on the standard arm to 73% in the 2-weekly arm
No increased toxicity was noted.
What chemotherapy agents were compared in the EICESS-92 trial?
Vincristine, doxorubicin, actinomycin-D with either cyclophosphamide or ifosfamide
Known as VAIA or VACA.
What was found regarding cyclophosphamide and ifosfamide in the EICESS-92 trial?
Cyclophosphamide had similar effects as ifosfamide but was associated with increased toxicity.
What was the treatment regimen in the EuroEWING99 trial?
6 cycles of vincristine, doxorubicin, ifosfamide, and etoposide (VIDE)
Patients without metastases were randomized to further treatment.
What is the recommended dose for definitive radiotherapy in Ewing sarcoma?
55 Gy in 30 fractions.
Is recurrent Ewing sarcoma often curable?
No, recurrent and locally relapsed Ewing sarcomas are seldom curable.
What treatment options are available for recurrent Ewing sarcoma?
Palliative radiotherapy and drugs including temozolomide, irinotecan, and topotecan.
What are the reported 5-year survival rates for localized Ewing sarcoma?
Ranges from 55% to 70%.
What factors are associated with a poorer outcome in Ewing sarcoma?
- Large tumours
- Axial tumours
- Increasing age.
What areas does the EuroEWING2012 trial aim to examine?
- Comparison of VIDE induction and VAI/VAC consolidation
- Addition of bisphosphonate during consolidation
- High-dose therapy with BuMel followed by stem cell rescue
- High-dose therapy for patients with pulmonary and/or pleural metastases.
What is ankylosing spondylitis?
An inflammatory arthritis of the spine and sacro-iliac joints, predominantly affecting young males.
What is the recommended radiotherapy dose for ankylosing spondylitis?
10 Gy in 10 fractions with orthovoltage.
What is heterotopic bone formation (HBF)?
Postoperative ossification that can develop 3–6 weeks after surgery.
What is the dose for radiotherapy to prevent recurrent HBF?
8 Gy in a single fraction within 4 days of surgery.
What are desmoid tumours also known as?
Aggressive fibromatosis.
What is the recommended dose for desmoid tumours after local resection?
50 Gy in 2 Gy fractions.
What is synovitis?
An inflammatory reaction affecting the synovial lining of joints.
What is the treatment for synovitis?
Intra-arterial injection of 90Y to give 185 MBq.
What does arterial restenosis refer to?
Restenosis of coronary arteries after balloon angioplasty.
What is the reduction in restenosis incidence due to radiotherapy post-angioplasty?
Reduced from 54% to between 17% and 25%.
