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11 Cards in this Set
- Front
- Back
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Histology |
Most common Malignant Fibrous Histocytoma Liposarcoma Synovial sarcoma Leiomyosarcoma |
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Spread
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Local: longitudinal extension to adjacent tissues via tissue planes, nerves and blood vessels Lymph: infrequent, excluding rhabdomyosarcoma and high grade synovial Metastatic: lungs in high grade |
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Epidemiology and Aetiology |
Rare, 1 % of all cancer 6% of childhood cancers 50% occur in extremities Most common in proximal thigh and buttock Remainder present in trunk, retroperitoneum, internal organs and head and neck region M>F Causative factors largely unknown Rare genetic conditions seem to be more predisposed to developing an STS (neurofibromatosis, garnder's syndrome, li-fraumeni syndome) RT induced malignancy (~10 yrs lately) Exposure to some chemicals |
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Tumour Origin |
Muscle Fibrous tissue Nervous tissue Fat Blood vessels Tendons Cartilage |
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Staging |
T1 = < 5 cm T2 = > 5 cm T1a/T2a = superficial to muscular fascia T1b/T2b = deep to muscular fascia
Low grade: exhibit well or moderately differentiated cells. The cell mitotic rate is slow and there is no necrosis High grade: poorly or un differentiated cells, a high mitotic rate and necrotic tissue |
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Clinical Presentations |
Symptomatic until advanced disease Pain and swelling Abdomen lesions: abdomen pain, vomiting, consitpation
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Detection and Diagnosis |
Clinical examination MRI Biopsy CT Functionl imaging FBC Ultrasound Endoscopy Hysteroscopy Genetic/previous radiation history |
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Disease Management |
Surgery first line of treatment + RT Other options: RT alone Chemo
Surgical resection only Usually employed for low grade extremity sarcomas, < 5 cm in size where possible to get clear surgical margins Tumour mass + 2 cm margin is resected
Amputation Reserved for massive disease where funtional limb-preservation not feasible Where local recurrence after previous resection and RT Chemo Poor results Palliation
Isolated limb perfusion Locally advanced disease of the limb, where amputation indicated ILP using NTF, melphalan and interferon = 76% response
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RT |
Pre-op RT Complicated wound healing
Intra-operative RT Brachy – iridium 192 Electron beam therapy Improved local control
Post-op Indicated in presence of gross residual disease Large tumours > 5 cm Head and neck sarcoma (hard to excise adequately) EBRT or brachy (or both)
RT alone Indicated in patients unfit/refuse surgery Where site precludes full surgical resection (retroperitoneum, h+n) Palliation
Considerations Ensure adequate lymphatic drainage corridor over entire treatment length for limbs/extremities Field edges at well defined boen margin in uninvolved bones Skin dose in surgical site important consideration for wound healing
Prescription Pre-op: 50 Gy in 25 Tumours < 10 cm: CTV = GTV + 5 cm longitudinal margin >10 cm: GTV + 7-10 cm longitudinal margin 28-35 Gy with chemo Post-op: 66-70 Gy in 33-35# Ph 1: CTV + 5 cm longitudinal margin (<10 cm) or 7-10 cm (>10 cm). 45-50 Gy Ph2: GTV + 2 cm. 66-70 Gy Ph3: 1cm. 72-76 Gy |
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Side Effects |
Erythema Desquamation Lymphoedema Diarrhoa/nausea/vomiting Wound complications Skin atrophy Fibrosis Contractures Arrest of growth Radiation induced sarcoma |
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Prognosis |
Low grade risk of mets at 5 yrs: < 10% High grade risk of mets at 5 yrs: 50% |
