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Soft Tissue Sarcoma An Overview & Update Mohamed Abdulla (M.D.) Ass. Prof. Clinical Oncology Kasr El-Aini School of Medicine Cairo University April, 2007.

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Presentation on theme: "Soft Tissue Sarcoma An Overview & Update Mohamed Abdulla (M.D.) Ass. Prof. Clinical Oncology Kasr El-Aini School of Medicine Cairo University April, 2007."— Presentation transcript:

1 Soft Tissue Sarcoma An Overview & Update Mohamed Abdulla (M.D.) Ass. Prof. Clinical Oncology Kasr El-Aini School of Medicine Cairo University April, 2007

2   1% of all human cancers.   50 histological subtypes.   Small peak of incidence in early childhood “embryonal rhabdomyosarcoma”.   Increased frequency with age.   Limbs, Abdominal Cavity & Retroperitoneum, Thoracic then Head & Neck Region. Prevalence & Problems:

3 Mesenchymal Cell of Origin Bone Muscle Fat Cartilage Tendon Ligament

4 Molecular Data are More Important than Site of Origin in Determining Prognosis & Treatment Options Soft Tissue Sarcomas Aberrancies Cell Regulatory Pathway Rb High Grade Lesion Aggressive Behavior Poor Survival p53 Aggressive Behavior Karyotype Abnormalities Simple Tumor Type Regardless Cell of Origin Complex ? Aneuploidy

5 Sarcomas with Complex Karyotype Abnormalities: 1) 1) Fibrosarcoma. 2) 2) Leiomyosarcoma. 3) 3) Osteosarcoma. 4) 4) Chondrosarcoma. 5) 5) Liposarcoma. 6) 6) Rhabdomyosarcoma. 7) 7) Malignant Schwannoma & Neurofibrosarcoma. 8) 8) Angiosarcoma.

6 Evaluation of The 1 ry Lesion: 1) 1) Plain Radiography. Low Cost. Informative. Benign, Aggressive or Malignant Lesion. Calcification in Soft Tissue Extension

7 Evaluation of The 1 ry Lesion: MRI: Spiral CT-Scan: Tissue of Origin. Location & Extent. Relationship to Surroundings. Define Further Steps of Locoregional Management. Better for Chest & Abdominal Lesions.

8 1. 1. CT-Scan of The Chest FDG-PET Scan Isotopic Bone Scan Bone Marrow Examination Others. Evaluation of The Systemic Extent:

9 Biopsy: FNAC True Cut Biopsy Excisional Biopsy Incisional Biopsy. Adequate Amount of Tissues. Not to Compromise Oncologic Safety.

10 Surgical Aspects:

11 5-Yr Survival Stage% I86 II72 III52 IV10-20

12 Improvement of Disease Specific Survival “DSS” Proper Surgery Radiation Therapy Systemic Therapy Improvement in Local ControlEradication of Micrometastases

13 The Use of Radiation Therapy: High Grade Lesions. Intermediate Grade with Positive Margins. Recurrent Tumors. Low Grade Lesions. Retroperitoneal Disease. External Beam RTh or Brachytherapy. Optimal Timing. Dose to Be Delivered. Advanced Technology Local Recurrence 10% Amputation Rate 5%

14 Systemic Therapy: 75% of All Patients with Localized Extremity STS will not Relapse After Local Treatment. No Need For Further Therapy

15 High Risk Patients: Large Tumors > 5 cm. High Grade Lesions. Deep Lesions to Investing Fascia. Recurrent Tumors at Presentations. Leiomyosarcoma & PNST. Locations other than Extremities.?? Positive Margins.??

16 Doxorubicin Experience: The Lancet Sarcoma Meta-analysis; 1997: 10% in DFS (p < 0.05) 4% in OAS (p > 0.05) 7% in OAS with Extremities Disease (p < 0.05)

17 Ifosfamide Experience: Italian Cooperative Trial; 2001UCLA, JCO; 2001  DFS Advantage.  Insignificant Impact OAS.  Improvement is Restricted to HIGH RISK PATIENTS.

18 Adjuvant Chemotherapy: Superiority of Doxorubicin Based Regimens only in Terms of DFS. (Mayo Clinic; 1984 & EORTC; 1994). Sarcoma Meta-Analysis Collaboration: % reduction in risk of LR Distant Recurrence Free Survival Marginal Improvement in OS EORTC Adjuvant Trial (High Dose Therapy with Growth Factor Support).

19 Neo-adjuvant + Surgery vs Surgery: No Survival Advantage. (Gortzak et al, Eur J Cancer; 2001) Neo-adjuvant vs Adjuvant: No Superiority (DeLaney et al, Int J Oncol Biol Phys; 2003). Neo-Adjuvant Chemotherapy:

20 Adjuvant Treatment: Neo-Adjuvant: Yes Not Yet To Treat or Not??

21 Metastatic & Unresectable Disease: Doxorubicin/Ifosfamide with Dose Escalation. Gemcitabine & Combinations. Paclitaxel in Angiosarcoma. Bortezomib. 9-Nitrocampothecin. Imatinib Mesylate. Liposomal Doxorubicin.

22 Gemcitabine & Combinations. Single Agent Activity: 18% (2-13 months). Protracted Infusion : (Median Survival 13 ms). Gem/Docetaxel: 35% in all Types 50% in Leiomyosarcoma. Gem/Venoralbine.

23 Paclitaxel in Angiosarcoma: MSKCC Phase II. 80% 5 months. Liposomal Doxorubicin vs Doxorubicin: EORTC Phase II. 10% & 9% 4 months.


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