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SOFT TISSUE SARCOMAS

SOFT TISSUE SARCOMAS. Dr. Neil D’Souza. SARCOMA. Greek sarx  meaning "flesh " Cancer  that arises from transformed  cells of  mesenchymal origin Thus , malignant tumors  made of  cancellous bone , cartilage, fat, muscle, vascular, or hematopoietic  tissues are sarcomas

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SOFT TISSUE SARCOMAS

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  1. SOFT TISSUE SARCOMAS Dr. Neil D’Souza

  2. SARCOMA

  3. Greeksarx meaning "flesh" • Cancer that arises from transformed cells of mesenchymalorigin • Thus, malignant tumors made of cancellousbone, cartilage, fat, muscle, vascular, or hematopoietic tissues are sarcomas • Human sarcomas are quite rare • Sarcomas can arise from bone or soft tissue

  4. Soft tissue is • Tissue that connects, surrounds and supports the skeletal system • Non-epithelial, extra skeletal tissue excluding reticuloendothelial system, glial tissue • Also includes peripheral nervous tissue • Embryologically it is mainly from mesoderm but few from neuroectoderm

  5. SOFT TISSUE SARCOMA

  6. 1% of adult malignancy, 15% of paediatric malignancies • Arises from pluripotent mesenchymal stem cell without in situ changes • More common in males (4:1)

  7. STS are named based on their tissue which it resembles • Liposarcoma—fat • Fibrosaroma—fibroblast • Malignant fibrous histiocytoma—mesenchyme/histiocytes • Leiomyosarcoma—smooth muscle • Rhabdomyosarcoma—skeletal muscle • Chondrosarcoma—chondroblast • Angiosarcoma—blood vessels

  8. Liposarcoma

  9. Fibrosarcoma - Thigh

  10. Malignant fibrous histiocytoma, pleomorphic type - Thigh

  11. Leiomyosarcoma Uterus and cervix

  12. Rhabdomyosarcoma

  13. Commonest soft tissue sarcoma is • Liposarcoma/ malignant fibrous histiocytoma • In the extremities both MFH and liposarcoma • In the retroperitoneumit is liposarcoma

  14. INCIDENCE • In adults • MFH and liposarcoma • Rhabdomyosarcoma • Leiomyosarcoma • Synovial sarcoma • Malignant peripheral nerve sheath tumour (MPNST) • Fibrosarcoma

  15. In children • Rhabdomyosarcoma, • Neuroblastomaare common

  16. SITES • 35% occurs in lower limb (commonest site) • 15% upper limb • 15% retroperitoneum • 15% viscera • 10% trunk • 10% other areas

  17. ETIOLOGY • Genetic • von Recklinghausen disease • Gardner’s syndrome • Tuberous sclerosis • Basal cell naevussyndrome • Li-Fraumeni syndrome • Stewart—Treves syndrome • Gorlin’s syndrome

  18. Chemicals • PVC, tetra-chloro-dibenzo-dioxin, arsenic, thorotrast, vinyl chloride, arsenic, pesticide • Viral • HIV in Kaposi’s sarcoma, cytomegalovirus • Ionising radiation • Malignant fibrous histiocytoma

  19. Lymphangiosarcoma • In post-mastectomy lymphoedema • Retinoblastoma associated sarcoma

  20. CLINICAL FEATURES • Painless, smooth, firm/hard, warm and vascular swelling of short duration with progressive increase in size • 30% of patients may present as pain • Compression of adjacent structures

  21. Soft tissue sarcoma upper chest wall

  22. Features of secondaries in lung—cough, haemoptysis, chest pain • Secondariesin liver as a principal site especially in visceral STS • One has to maintain a high index of suspicion in any soft tissue mass • Deep to deep fascia, • > 5 cm, • Any new enlarging or symptomatic soft tissue mass

  23. INVESTIGATIONS • Preoperative evaluation of STS is done: • To obtain a tissue diagnosis • Determine the “exact extent” of the tumour • To evaluate metastatic disease

  24. Tissue Diagnosis • Incision biopsy • Best method of diagnosis • It provides adequate tissue sample • Trucutbiopsy/core needle biopsy • Acceptable first diagnostic step • But it is not useful in visceral STS • Done using 14 gauge needle • US / CT guidance

  25. Excision biopsy • Done in cutaneous or subcutaneous tumors is < 3 cm where wide local re-excision is possible • Otherwise avoided as it may contaminate the tumour bed and restricts therapeutic options

  26. Assessment of the Extent of Tumour • Provides a 3–dimensional extentand helps in accurate planning of surgical procedure • It assesses macroscopic and not microscopic extent

  27. MRI • Investigation of choice as it determines the vascularity, relation to vessel and fascial planes (extent and invasion) • CT scan • Also used to see the extent and invasion but not equivalent to MRI • CT abdomen is betterin GI/retroperitoneal sarcomas • SPECT—3 dimensional reconstruction feasible

  28. US • Less sensitive • Initial investigation in GI leiomyosarcoma/retroperitoneal sarcomas • Useful in extremity lesions to assess vascular system • X ray • Used in initial phases to differentiate STS from bony lesions

  29. Angiogram • To delineate adjacent vasculature when there is a need for accurate assessment of vasculature • Intra-arterial chemotherapy for unresectabletumours • Upstaged by CECT/MRA

  30. Evaluation of Metastatic Disease • Chest X-ray • To look for lung secondaries • CT chest • To see early lung secondaries • Done in all deep seated, high grade and tumour more than 5 cm

  31. US abdomen • To check for liver secondaries • CT abdomen and pelvis • Better choice

  32. Other Investigations • Radionuclide scintigraphy (Gallium-67) • p-MRS (p-Magnetic Resonance Spectroscopy) and FDG-PET to assess the metabolic activity of tumour • Immunohistochemistry and FISH(fluorescence in situ hybridization) • Tumour markers • Haematocrit, peripheral smear, ESR, serum alkaline phosphatase, serum creatinine

  33. STAGING

  34. TREATMENT • Principles of Treatment • Surgery is the main treatment modality • In low grade tumours without any spread • Function/limb sparing complete wide excision is sufficient • If microscopic margin is positive for tumour then postoperative EBRT is given

  35. In high grade tumour < 5 cm size • Function sparing wide excision with more than 1 cm clearance margin is sufficient • If clearance margin is less than 1 cm or shows microscopic positive margin, RT is given • In high grade tumour which is between 5-10 cm size • Function/ limb sparing complete wide excision with RT is given

  36. In high grade tumour >10 cm in size • Initially neoadjuvantchemotherapy; then functional/limb sparing complete wide excision with RT is given • All limbs should be conserved if possible but with curative intent

  37. Surgery • Enneking classification of surgical procedures • Intralesional – • Very high recurrence 100% - not used • Marginal excision • Through the reactive zone - high recurrence rate 70% • Wide excision means • Through normal tissues beyond the reactive zone; • Recurrence rate of 30%

  38. Radical excision • If the margin is more than 5 cm outside the reactive zone • It is like compartment excision with very low recurrence rate • Other procedures • Compartmental excision; function/limb sparing • Vascular resections with vascular reconstruction • Amputation

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