Soft tissue sarcoma ,Dr Sharew Delelegn

Soft tissue sarcoma is a rare malignant tumor of soft tissue, which is mesenchymal in origin.There are more than 100 subtypes of this condition. Because of its rarity & hetrogenity it is difficulty to have clinical datas of similar subtype. Treatment of Soft tissue sarcoma should be multidiscipilinary & centeralized so that more investigation is caried out and specific & more effective treatment modalities generated for each specific subtypes

1. Soft tissue sarcoma Dr Sharew Delelegn Orthopedics & Trauma Surgery Resident Adama Hospita Medical College July-2024

2. Outline Introduction Etiology Classification Clinical presentation Diagnosis & staging Treatment Summary

3. Introduction Soft-tissue sarcomas (STS) represent a cohort of rare and heterogeneous tumors that account for 1% of all adult malignancies. Mesenchymal in origin The condition usually presents in patients > 15 years old with a slow-growing, painless soft tissue mass.

4. Demography Incidence 12,000 new cases/year in the United States 4,700 deaths/year in the United States Demographics males > females 85% occur in patients >15 years old Anatomic location 60% occur in extremities 44% occur in the thigh

5. Etiology Genetics classic translocations t(X;18); SYT-SSX fusion  synovial sarcoma t(2:13)  rhabdomyosarcoma (alveolar) t(12;16)(q13:p11)  myxoid liposarcoma Associated conditions neurofibromatosis type-1  malignant peripheral nerve sheath tumor (MPNST) Stuart-Treves syndrome  angiosarcoma chronic lymphedema  angiosarcoma Radiation exposure Chemical exposure Chronic inflammation Previous trauma Idiopathic

6. Classification of soft tissue sarcoma Classification of STS is based on histology, with over 50 types recognized, all STS have the similar presentation, imaging, differential diagnosis, and treatment. H istologic findings and molecular signatures are used to distinguish individual types.

7. WHO Classification of STS

8. Clinical Presentation History slow growing, painless mass   incidental trauma often draws attention to mass rapid growth usually suggests a higher grade Physical exam mass may be palpable poor prognostic indicators include >5cm, deep to fascia, and immobile  

9. Most sarcomas have a greater tendency to metastasise via blood vessels to lungs rather than to lymph nodes. Only 5% of soft tissue sarcomas metastasize to lymph nodes soft tissue sarcomas that metastasize to lymph nodes (RACES to the lymph nodes) Rhabdomyosarcoma, Angiosarcoma , Clear cell sarcoma, Epithelioid sarcoma, Synovial sarcoma

10. Imaging Radiographs may show soft tissue shadow or mineralization (most commonly with synovial sarcoma) CT chest obtain to assess for metastatic disease  an exception is that a CT chest/abdomen/pelvis is indicated for myxoid liposarcoma MRI with contrast T1: low signal intensity (isointense with muscle) T2: high signal intensity IV gadolinium: peripheral enhancing zone and non-enhancing necrotic center

11. French Federation of cancer centers sarcoma g roup

12. AJCC 8 th edition

13. Treatment Surgery is the standard treatment for localized disease The surgical procedure consists of a wide excision with negative margin R adiation is an important adjunct to surgery decreasing local recurrence, regardless of the marginal status after resection Anthracyclines monotherapy is the first-line standard treatment for metastatic patients, not a candidate for local treatment and combination therapy could be considered for patients who may beneft from tumor reduction for symptom palliation or improving resectability

14. Poor prognostic factors include High-grade metastatic disease Size > 5 cm Tumor location below the deep fascia Delay in diagnosis Unplanned excision  

15. Survival with treatment low-grade disease: 90% 5-year survival stage II and III disease: 50-75% 5-year survival  stage IV: 15% 5-year survival patients with advanced disease have median survival of 12-18 months

16. Reference Upto date Orthobullet Basics of Oncology ( Frederi (Z-Library). pdf The 2020 WHO Classification of Soft Tissue Tumours : news and perspectives DOI:10.32074/1591-951X-213 SEOM Clinical Guideline of management of soft‑tissue sarcoma (2020) https://doi.org/10.1007/s12094-020-02534-0 Soft tissue sarcomas in adults and children: A comparison , Walter Lawrence, Jr., MD

17. THANK YOU !