Recomendados
Mais conteúdo relacionado
Mais procurados
Mais procurados (20)
Semelhante a Soft tissue sarcoma
Semelhante a Soft tissue sarcoma (20)
Mais de Junaid Ahmad
Último
Último (20)
Soft tissue sarcoma
- 1. Soft Tissue Sarcoma Dr Junaid Ahmad
- 2. ∗ A heterogeneous group of tumors of connective tissue ∗ Two third of Soft tissue sarcomas in extremities ∗ Constitute 1% of cancers in adults but 15% in children ∗ The overall 5-year survival rate 50% to 60%. ∗ Most of die with lung metastasis Sarcomas
- 3. ∗ Malignant fibrous histiocytoma is most common type in elderly ∗ Liposarcoma in middle age ∗ Leimayosarcoma in young ∗ Embryonal/alveolar rhabdomyosarcomas are the most common type in children Histological Subtypes
- 4. ∗ I. Fibrosarcoma ∗ 1. Adult fibrosarcoma ∗ 2. Inflammatory fibrosarcoma ∗ 3. Myxofibrosarcoma ∗ II. Fibrohistiocytic tumors ∗ 1. Dermato Fibro sarcoma Protruberans ∗ 2. Heterogenous tumors without specific differentiation(Formerly MFH) ∗ i. Undifferentitaed pleomorphic sarcoma. ∗ ii. Undifferentiated pleomorphic sarcoma with giant cells with inflammation. ∗ iii. Angiomatoid MFH ∗ III. Lipomatous tumors ∗ 1. Atypical lipoma ∗ 2. Liposarcoma ∗ i. Well differentiated ∗ a. Lipoma like ∗ b. Sclerosing ∗ c. Inflammatory ∗ ii. Dedifferentited liposarcoma ∗ iii. Myxoid or round cell LS ∗ iv. Pleomorphic LS ∗ IV. Smooth muscle Classification
- 5. ∗ 1. Leiomyosarcoma ∗ 2. Epitheloid LMS ∗ V. Skeletal Muscle ∗ 1. RhabdoMayoSacroma ∗ i. Embryonal ∗ ii. Botryoid ∗ iii. Spindle call ∗ iv. Alveolar ∗ v. Pleomorphic ∗ 2. RMS with ganglionic differentiation (Ectomesenchymoma) ∗ VI. Blood and lymph vessels ∗ 1. Epitheloid hemangioendothelioma. ∗ 2. Angiosarcoma nd lymphangiosarcoma ∗ 3. Kaposi’ s sarcoma ∗ VII. Malignant perivasular tumors ∗ 1. Malignant glomus tumor or glomangiosarcoma. ∗ 2. Malignant hemangio pericytoma ∗ VIII. Malignant synovial tumors ∗ Malignant GCT of tendon sheath. ∗ IX. Malignant neural tumors ∗ 1. MPNST (Neurofibrosarcoma) ∗ 2. Malignant granular cell tumor Classification
- 6. ∗ 3. PNET(primitive NET) ∗ i. Neuroblastoma ∗ ii. Ganglioneuroblastoma ∗ iii. nauroepithelioma ∗ X. Paraganglionic tumors ∗ Malignant paraganglioma ∗ XI. Extra skeletal Cartilaginous and oseeous tumors ∗ 1. Extra skeletal chondrosarcoma ∗ a. Myxoid ∗ b. Mesenchymal ∗ 2. Extraskeletal osteosracoma ∗ XII. Pluripotential malignant mesenchymal tumor ∗ 1. Malignant mesenchymoma ∗ 2. Alveolar soft part sarcoma ∗ 3. Epitheloid sarcoma ∗ 4. Malignant extra renal rhabdoid tumor ∗ 5. Desmoplastic small cell tumor ∗ 6. Extraskeletal Ewing s sarcoma ∗ 7. Clear cell sarcoma ∗ 8. GIST ∗ 9. Synovial sarcoma Classification
- 7. ∗ Liposarcoma ∗ Leiomyosarcoma ∗ Unclassified sarcoma ∗ Synovial sarcoma ∗ Malignant peripheral nerve sheath tumor ∗ Rhabdomyosarcoma ∗ Fibrosarcoma ∗ Ewing sarcoma ∗ Angiosarcoma ∗ Osteosarcoma ∗ Epithelioid sarcoma ∗ Chondrosarcoma ∗ Clear cell sarcoma ∗ Alveolar soft part sarcoma ∗ Malignant hemangiopericytoma Common Histological Subtypes
- 8. ∗ 20% of all Soft tissue sarcomas ∗ Thighs and retroperitoneum. ∗ Three principal groups: ∗ 1. Atypical lipomatous tumour/ WD LS and dedifferentiated LS ∗ Adipocytic (lipoma like) ∗ Sclerosing ∗ Inflammatory ∗ Spindle cell. ∗ 2. Myxoid or round cell LS ∗ 3. Pleomorphic LS Liposarcoma
- 9. ∗ Malignant tumors composed of spindle cells ∗ Showing smooth muscle features. ∗ Location: Retroperitoneal, intra abdominal pelvic sites, uterus ∗ Smooth muscle actin and desmin. ∗ Grading of LMS difficult. ∗ Large tumor size, high grade and high mitotic rate are the prognostic factors. Leiomyosarcoma
- 10. ∗ 1. Embryonal ∗ Small cell tumor ∗ Orbit or genito urinary tract of children. ∗ Botyriod type usually in the mucosa lined visceral organs vagina and urinary bladder ∗ Polypoid tumour ∗ Also seen in adults but poorer prognosis ∗ 2. Alveolar type: ∗ Extremities ∗ Young adults and adolescents. Rhabdomyosarcoma
- 11. ∗ Malignant tumor with cells that resemble morphologically and functionally endothelial cells. ∗ No clear distinction of those from lymphatics and capillaries. ∗ Sometimes associated with lymphedema. ∗ Stewart treve syndrome ∗ Lymphangiosarcoma of skin in the lymphedematous arm post mastectomy ∗ Radiation therapy Angiosarcoma
- 12. ∗ Rare sporadically ∗ But 8 to 13% in those with NF-1 ∗ Affect major nerves of extremities or chest wall. ∗ Originate from nerve sheath. ∗ Most are high grade ∗ Stain positive for S-100 ∗ MPNST with Rhabdomayosarcoma elements termed Triton tumor Malignant Peripheral Nerve Sheath Tumor
- 13. ∗ This concept is challenged now. ∗ Merely in histological appearance. ∗ None of them show histiocytic differentiation. ∗ Low grade- recur locally, but rarely metastasize. ∗ Slow and persisitent growth. ∗ Unpredicted radial extensions. ∗ Stains positive for CD34. ∗ Response with Imatinib Fibrohistiocytoma
- 14. ∗ Spindle cell tumor ∗ Young adults 15-35 yrs of age ∗ 80% in extremities, 10 % in Head and Neck ∗ Unrelated to synovium ∗ Stain positive for keratin, vimentin, S-100+/- Synovial sarcoma
- 15. ∗ Epitheloid sarcoma ∗ Unknown lineage ∗ Adolescent and young adults ∗ Extremity and perineal area ∗ Tends to propogate along tendon and nerve sheaths. ∗ Lung and lymph nodal metastasis common. ∗ 5 year survival 66%. Synovial sarcoma
- 16. ∗ Reserved for those undifferentiated Pleomorphic sarcomas with no line of differentiation by current technology. ∗ Aggressive course ∗ Many develop metastasis within 3 years of diagnosis. High grade undifferentiated pleomorphic sarcoma/ pleomorphic MFH
- 17. ∗ Most of time cause is unknown ∗ Few known etiologies are ∗ Radiation Exposure ∗ Occupational Chemical Exposure ∗ Trauma ∗ Chronic Lymphedema ∗ Genetic Conditions (NF, RB) Cause
- 18. ∗ Different type of known mutations are ∗ Point Mutations ∗ Translocations ∗ Amplifications ∗ Oncogenic Mutations ∗ Complex Genomic Rearrangements Molecular Basis
- 19. ∗ Asymptomatic painless masses ∗ Venous thrombosis in extremities ∗ Compress adjoining structures ∗ Sometimes painful, edema and swelling when bone or nearby neurovascular bundle involved ∗ Sometimes a traumatic even draws attention to it Clinical Picture
- 20. ∗ Lipoma ∗ Lymphangioma ∗ Leiomyoma ∗ Neurinoma ∗ Primary or metastatic carcinoma ∗ Melanoma ∗ Lymphoma Differential Diagnosis
- 21. ∗ Superficial small lesions (<5 cm) that are new or that are not enlarging as indicated by clinical history can be observed. ∗ Enlarging masses and masses larger than 5 cm or deep to the fascia should be evaluated with a history, imaging, and biopsy. Assessment
- 22. ∗ Size of tumor and skin involvement (defect) ∗ Pulses and sensation (for vascular and nerve reconstruction) ∗ Involved muscle groups (for tendon/vascularized muscle transfer) ∗ Age and fitness for surgery ∗ Lifestyle (for limb preservation) Clinical Assessment
- 23. ∗ Should be before any invasive procedure ∗ MRI is the choice in extremities ∗ An x ray may help in bone involvement ∗ CT may be helpful in intra abdominal and few types of sarcomas ∗ CT chest and MRI brain may be required to see metastasis ∗ Ultrasonography if MRI is contraindicated Diagnostic Imaging
- 24. ∗ PET scan is only a slight better than CT ∗ Follow up 3 monthly MRI are done to see recurrence Diagnostic Imaging
- 25. ∗ Fine-Needle Aspiration ∗ Core Needle Biopsy (choice) ∗ Incisional Biopsy (25% changed plan) ∗ Excisional Biopsy Biopsy Techniques
- 26. ∗ Light Microscopy/Morphology (25-40% disagree) ∗ Electron Microscopy ∗ Cytogenetics; immunohistochemistry and molecular genetic testing. ∗ Other molecular diagnostic techniques include Flow cytometry, fluorescence in situ hybridization (FISH), and polymerase chain reaction–based methods. Pathologic Assessment and Classification
- 27. ∗ Pathological classification is more important ∗ Type of tumor ∗ Histologic Grade of Aggressiveness ∗ Nodal Metastasis (Rare in adult sarcomas) ∗ Distant Metastasis (CT chest) Staging and Prognostic Factors
- 28. ∗ Parameters by French federation of cancer centre three tire system (FNCLCC) ∗ Differentiation score ∗ Mitoses ∗ Necrosis ∗ Some ungradable e.g. epitheloid, clear cell, angiosarcoma Grading
- 29. ∗ Primary: ∗ T1 tumour 5 cm or less in greatest dimension (a superficial, b deep). ∗ T2 tumour more than 5 cm in greatest dimension (a superficial, b deep). ∗ Regional nodes: ∗ N0 none. ∗ N1 regional nodes. ∗ Distant metastases: ∗ MX, M0, M1 TNM G Staging
- 30. ∗ Histological grade G ∗ GX cannot be assessed ∗ G1 well differentiated ∗ G2 moderately differentiated ∗ G3 poorly differentiated ∗ G4 undifferentiated ∗ G1/2 would be low grade tumors TMN G
- 32. ∗ Kattan et al studied Prognostic Factors ∗ Age ∗ Histology ∗ Grade ∗ Location ∗ Depth ∗ Size Prognostic Factors.
- 33. ∗ The treatment algorithm for soft tissue sarcomas depends on tumor stage, site, and histology. Treatment
- 34. ∗ Surgery ∗ Limb sparing surgery / Wide Local Excision ∗ Locoregional Lymphadenectomy. ∗ Amputation. ∗ Isolated Regional Perfusion. ∗ Radiation Therapy ∗ Systemic Therapy ∗ Standard Chemotherapy. ∗ Novel Chemotherapeutic Agents. ∗ Targeted Therapies. Treatment Modalities
- 36. ∗ Limb Salvage Technique ∗ 1-2 cm margins (Dermatofibrosarcoma Protuberance require 2-4cm) ∗ Biopsy site is resected ∗ Nerve and vessels are usually preserved by narrowing the margins ∗ NV bundle if very near to tumor then epineurium and adventitia are removed ∗ Tumor encircling NV bundles should be removed ∗ Nearby veins are not be spared usually Wide Local Excision
- 37. ∗ Enucleation is discouraged and dissection must be in grossly normal planes (even with radiation) ∗ Lin and colleagues studied that in absence of frank cortical margins periosteum is the adequate surgical margin with radiation ∗ Bone involvement is poor prognostic factor ∗ NV bundles reconstructed ∗ Free flaps and tendons/muscle transferred ∗ Skin covered Wide Local Excision
- 38. ∗ Early physical therapy is essential ∗ Large sarcomas of distal parts of extremities are difficult to treat and amputations are considered Wide Local Excision
- 39. ∗ Also limb sparing surgery ∗ Differ from wide local excision that involved muscles are removed from origin to insertion Compartectomy
- 40. ∗ FNAC should be done US guided ∗ If involved selected lymphedenectomy done ∗ Proved increase survival rate ∗ Sentinel lymph node biopsy is controversial Lymphedenectomy
- 41. ∗ 5% cases of extremity sarcomas ∗ Have no survival advantage ∗ Avoids local recurrence ∗ Large unresectable tumors ∗ Reserved only for cases when Limb Salvage cannot be done Amputations
- 42. ∗ Palliative treatment ∗ Artery and Veins of region involve are dissected and connected to a pumping device ∗ Branches are ligated ∗ Perfused with TNF-alpha and Malaphalan ∗ Limb is kept warm with heater at 40C ∗ Systemic leakage checked with Tc labelled albumin ∗ Artery and vein repaired ∗ Expensive and controversial Isolated Regional Perfusion
- 43. ∗ Indications ∗ Limb conservation or limited surgery ∗ Gross residual tumor or inadequate excision margins ∗ Grade is high on histology ∗ Tumor 5 cm or more in any dimension ∗ Virtually all tumors in H&N Radiation
- 44. ∗ Smaller than 5cm tumors with clear margins can avoid radiation even if high grade ∗ Pre operative post operative and intra operative techniques have debates ∗ Metallic clips during surgery help define margins Radiation
- 45. ∗ Post operative ∗ Large doses required ∗ Reconstruction is less complicated ∗ Long time to complete ∗ Local Cure is comparable Radiation ∗ Pre operative ∗ Difficulty in pathologic assessment of margins ∗ Wound complications increased ∗ Low doses required ∗ Better long term results fibrosis ∗ Difficult reconstructions
- 46. ∗ No survival benefit ∗ Synovial and Myxoid sarcomas are most sensitive ∗ For those with significant risk of death ∗ Metastasis ∗ Non extremity tumors (unresectable) ∗ Intermediate to high grade with size larger than 5cm ∗ Docurubicine and ifosfamide are standard ∗ Hemorhagic cystitis, real tubular acidosis, neurotoxicity are side effects of ifosfamide Systemic Chemotherapy
- 47. ∗ Anti vascular endothelial growth factor is particularly effective against angiosarcoma ∗ Trabectedin for leiomayosarcoma ∗ Imitanib for GI stromal tumor ∗ Use is contorversial as many trials even failed to show benefits in disease free interval Systemic Chemotherapy
- 48. ∗ Increase wound complications ∗ Decrease the size of tumor ∗ Tumors with high pathological necrosis on CT respond better to neoadjuvant chemotherapy Neoadjuvant (Preoperative) Chemotherapy.
- 49. ∗ Theoratical benefit is short total treatment time in high risk patients Concurrent Chemoradiation Therapy
- 50. Thoracotomy and metstatectomy 3 year survival is 23 to 42 % Indicated if Primary tumor is controlled or controllable Complete resection appears to be possible No extra thoracic disease No medical complicated disease Lung Metastatic Disease
- 51. ∗ History Physical Chest CT or radiograph every 3 to 6 months ∗ Tumor site should be evaluated with MRI first at 3 months then every 6 months ∗ First 2-3 years are most important ∗ Some prefer less aggressive radiological approach for asymptomatic patients Post treatment Surveillance
- 52. ∗ Biospy of any suspicious nodule ∗ Redical excision with or without radiation is treatment for tumor Management of Recurrent Sarcoma
- 53. Thank you