Recommended
More Related Content
What's hot
What's hot (20)
Similar to Soft tissue sarcoma
Similar to Soft tissue sarcoma (20)
More from Junaid Ahmad
Recently uploaded
Recently uploaded (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