Soft tissue sarcomas (STS) are rare tumors accounting for 1% of adult malignancies, primarily affecting patients over 15 years old with a slow-growing mass. Treatment typically involves surgery and may include radiation, with prognosis varying significantly based on tumor grade and size. Factors such as high-grade disease and size over 5 cm indicate poorer outcomes, with 5-year survival rates ranging from 90% for low-grade tumors to 15% for advanced disease.

1. Soft tissue sarcoma
DrSharew Delelegn
Orthopedics&TraumaSurgery Resident
AdamaHospitaMedicalCollege
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

6. 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

7. 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.
Histologic findings and molecular signatures are used to distinguish
individual types.

8. WHO Classification of STS

11. 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

14. 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

15. 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

17. French Federation of cancer centers sarcoma group

18. AJCC 8th edition

19. Treatment
Surgery is the standard treatment for localized disease
 The surgical procedure consists of a wide excision with negative
margin
Radiation 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

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

22. 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

23. 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

24. THANK YOU !