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Sarcoma
- 1. Sarcoma Mohammed Fathy Bayomy, MSc, MD Lecturer of Clinical Oncology Clinical Oncology & Nuclear Medicine Department Faculty of Medicine Zagazig University
- 2. STS represents 1% of all malignant tumors Median age 40-60 Slightly common in males STS outnumber bone sarcomas by ratio of 4:1
- 3. Radiation Chromosomal aberrations Chemical exposure Other risks Phenoxy herbicides Vinyl chloride monomer Dioxin Cyclophosphamide Melphalan Nitrosoureas Anthracyclines Stewart–Treves syndrome HHV8 HIV Paget’s disease of bone Radiation-induced sarcomas
- 4. Hereditary Neurofibro- matosis type I Familial retinoblastoma Osteosarcoma Rb1 gene Chr.13 Gardner’s syndrome Desmoids APC gene Chr. 5 Li–Fraumeni syndrome MPNST NF1 gene Chr.17 Osteosarcoma p53 gene Chr.17 Tuberous sclerosis Gorlin’s syndrome Rhabdo- myosarcoma TSC1 gene Chr.9 Rhabdo- myosarcoma PTCH1 gene Chr.9
- 5. SoftTissueSarcomas(STSs) Alveolar soft part sarcoma Pleomorphic sarcoma (NOS) Fibrosarcoma Liposarcoma Synovial sarcoma Clear cell sarcoma Epithelioid sarcoma Solitary fibrous tumor Hemangio- sarcoma Lymphangio- sarcoma Kaposi sarcoma Rhabdo- myosarcoma Schwannoma MPNST
- 6. Bonesarcomas Visceral Sarcomas Chondro- sarcoma Adamantinoma Osteogenic sarcoma Fibrosarcoma of bone MFH of bone Ewing sarcoma Parosteal sarcoma Giant cell tumor of bone Chordoma Rhabdo- myosarcoma Leiomyo- sarcoma GIST Tumours Hemangio- sarcoma Lymphangio- sarcoma
- 8. Extremities STSs Painless soft tissue swelling H & N STSs Proptosis Masses Neurologic abnormalities GIST tumours Bleeding Haematemesis Blood in faecesAnaemiaUterine sarcoma Vaginal bleeding Lower abdominal pain Retroperitoneal Sarcomas Back pain Lower limb edema Abdominal masses Bone sarcomas Localised soft tissue swelling Localised pain Pathologic fractures Joint effusions Limited & painful adjacent joints movement Constitutional symptoms
- 9. MRI Chest Imaging Pathological confirmation Laboratory Extremities STSs Visceral & Retroperitoneal Sarcomas CT &/or MRI
- 10. Bone Sarcoma Plain X-ray of bone Codman triangle Sun-ray spiculation Onion-skin appearance MRI Bone scan Pathological confirmation Laboratory Bone marrow aspirate and biopsy
- 11. Non-metastatic STSs Surgery Limb-sparing surgery (WLE) Limb amputation Adjuvant Radiotherapy High- grade > 5cm +ve margin Adjuvant Chemotherapy Five cycles of ifosfamide + epirubicin Neoadjuvant Radiotherapy Borderline resectable Definitive Radiotherapy Unresectable Medically inoperable EBRT Brachytherapy &/or Chemotherapy ± Metastatic STSs Surgery Metastasectomy Chemotherapy High-dose ifosfamide Doxorubicin + ifosfamide AD (Adriamycin, Dacrabazine) MAID (Mesna, Adriamycin, Ifosfamide, Dacrabazine) Gemcitabine + Docetaxel Limited number of pulmonary metastases
- 12. Resectable Surgery WLE Radical surgery Adjuvant Radiotherapy High-grade +ve margin Neoadjuvant RT Unresectable Locally Advanced Radiotherapy Palliative Surgery Chemotherapy Retroperitoneal/VisceralSarcomas Gastrointestinalstromaltissuetumours Resectable Surgery Radical surgery Marginally Resectable Adjuvant Targeted Therapy (TKI) Imatinib Neoadjuvant Targeted Therapy (TKI) Imatinib (3- 6months) Surgery Radical surgery Adjuvant Targeted Therapy (TKI) Imatinib Unresectable/Metastatic Targeted Therapy (TKI) Imatinib Sunitinib Regorafenib
- 13. Non-metastaticOsteosarcomaofBone Neodjuvant Chemotherapy High-dose MTX + leucovorin rescue Doxorubicin + Cisplatin Surgery Limb-sparing surgery Limb salvage surgery Osteoarticular resection Adjuvant Chemotherapy Whole bone resection Amputation Definitive Radiotherapy Refuse amputation Metastatic Surgery Metastasectomy Chemotherapy Palliative Radiotherapy Ewing’s Sarcoma Combined-modality approach Induction chemotherapy VAC/IEVACA Local radiotherapy &/or Surgery Consolidation chemotherapy VAC/IEVACA