BASIC OVERVIEW OF Soft tissuse sarcoma MANAGEMENT.pptx

Soft tissuse sarcoma management

1. Management of soft tissue sarcomas

2. Surgery is the mainstay of treatment En bloc resection is standard treatment 50-80% need organ resection 78% of primary lesions can be completely resected Completeness of resection and grading of the tumour are the most important prognostic factor

3. Kawaguchi phenomenon Thin barrier - epineurium, periosteum, vascular sheath (2 cms margin) Thick barrier - fascia, joint capsule ( 3 cms margin) Articular cartilage - 5 cms margin A surgical margin that is outside a barrier with normal tissue in between barrier and reactive zone of the tumor is considered curative

4. ENNEKING CLASSIFICATION OF SURGICAL PROCEDURES lntralesional excision done inside pseudocapsule , very high recurrence, 100%-not used. Marginal excision -en bloc resections through the reactive zone-high recurrence rate 70% Wide excision means en bloc resection done through normal tissues beyond the reactive zone; it means if the margin is less than 5 cm; tumour is never visualised during surgery; it has local recurrence rate of 30%. Wide margin is classified as adequate if margin is at least beyond 1 cm outside the reactive zone or inadequate if margin is within 1 cm. Radical excision -if the margin is more than 5 cm outside the reactive zone. It is like compartment excision with very low recurrence rate

5. Limb sparing; function preserving; margin free wide excision is now practiced. Wide local excision with clearance of 2 cm (minimum need is 1 cm) or more with preservation of function is needed. Depth clearance is also important. 3-5 cm clearance even though was in olden days, is not necessary. Compartment resection is a radical limb saving procedure. Here muscle group of one compartment (anterior, posterior or medial) is resected entirely from its origin to insertion with the tumour. It is done only when tumour is intracompartmental. It is not suitable when tumour is extracompartmental or many compartments are involved or encased to major neurovascular bundle. Basis for compartmental excision is-STS rarely penetrate anatomical barriers unless it is very advanced. Amputation is done in large tumours of upper or lower limbs.

6. Radical amputation is done as disease has not spread systemically which should be confirmed by CT chest, abdomen and pelvis. In metastatic disease there is no need except if primary is fungating and distressing. Indications for amputation: major neurovascular encasement Bone involvement Multiple compartment involvement Limb itself like lymphoedema Recurrence with multicentricity

7. Management of Retroperitoneal STS Average size of Tumor is 15 cms. Concept of margin status Is different from extremities sarcoma because it is impractical to assess complete margin. In RPSTS - gross resection is preferred. Earlier complete compartmental resections were done. Neoadjuvant RT is better tolerated . Adjuvant RT is impractical due to bowel toxicity. Based on history, physical examination and imaging , for management, these tumours are broadly classified as rescetable or un-resectable or germ cell tumors For germ cell tumors , chemotherapy is the treatment.

8. Management of resectable RPSTS

9. Management of un-resesctable RPSTS

10. Management Gist Wide local excision with negative margin is gold standard R0 or R1 no difference in local recurrence or overall survival Lymphadenectomy is not manadatory Intra op rupture is a poor prognostic factor Liposarcoma Extremities- WLE with 1 cm margin ( limb sparing preferred) Retroperitoneum- complete gross resection > complete compartmental resection If growth of recurrence is < 0.9 cm / month then only re resection is proceeded

11. Angiosarcoma chemo and radio responsive Surgery is the primary treatment DFSP Wide local excision Neoadjuant chemotherapy with imaging MPNST Wide local excision Adj RT - if lesion in extremities/ superficial trunk lesion

12. LEIOMYOSARCOMA Surgery is gold standard Less chemo sensitive Kaposi sarcoma RT + CT ( Adriamycin, bleomycin) Lipoma Excision biospy

13. Radiotherapy Both pre op and post op RT is given ( neo adjuvant RT). Postoperative radiotherapy is commonly used because of less tumour burden and less wound problems. Titanium clips are placed during surgery at high-risk areas to identify the site to concentrate proper RT. Brachytherapy is very effective in local control of the tumour. Initially precise mapping of the area is done in the operation theatre. Loading catheters are placed in surgical field peroperatively. Later these catheters are loaded with iridium 192. Dose is 45 Gy to tumour bed for 6 days.Permanent radioactive sources can also be placed to the area. Postoperative external beam radiotherapy (EBRT)-it is quiet effective and used in high grade tumour more than 5 cm often with brachytherapy. Dose is 70 Gy-25 fractions. Palliative external radiotherapy can be given to prevent bleeding, fungation and to reduce pain in advanced cases. It is also used in secondaries in brain, bone.

14. Chemotherapy Neoadjuvant chemotherapy is only given since adjuvant chemotherapy has no role Most active chemo agents : Dacarbazine Doxorubicin ( cardio toxic) Ifosfamide (hemorrhagic cystitis, neurotoxicity, renal tubular acidosis) Chemo regimes : AIM. AD ( Adriamycin , Dacarbazine) MAID ( mesna, adriamycin , Ifosfamide, doxorubicin)

15. Chemo sensitive tumours Synovial Myxoid / round cells liposarcoma Uterine leiomyoma Ewings Tumor

16. Chemo resistant tumour Clear cell sarcoma Endometrail stromal sarcoma Alveolar soft part sarcoma Extra skeletal myxoid chondro sarcoma

17. Isolated regional perfusion It is generally used for locally advanced / multimodal / recurrent disease It is a limb sparing technique High conc of melphalan under hyperthermia

18. Poor prognostic factors in STS Size >5 cm-important factor More than one compartment involvement Neurovascular invasion Clearance margin High grade Deep tumours and multicentric Lung secondaries

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