Soft tissue sarcomas (STS) are a diverse group of rare and aggressive malignant tumors that arise in the extremities in most cases. Extremity STS are evaluated through tissue biopsy, imaging to determine local extent and staging for metastasis. Treatment involves a multidisciplinary approach with limb-salvage surgery providing wide resection margins combined with radiation therapy. For most patients, this approach results in local tumor control while preserving a functional extremity.

1. Journal Reading Onco 2 - dr. Reza Devianto
Consultants
Prof. Dr. dr. Darmadji Ismono., Sp.B., Sp.OT(K)
dr. M. Naseh Sajadi Budi Irawan, Sp.OT(K)
dr. Herry Herman., Sp.OT.,Ph.D
dr. Bangkit Primayudha, Sp. OT

2. INTRODUCTION
• A diverse group of rare malignant tumors which arise from mesenchymal tissue
• Median age 56–65 years; peaking in the 8th
• Extremities account for 60% of cases (thigh)
• Subtypes vary based on molecular characteristics, clinical behavior & response to
treatment (nearly 100)
• UPS
• MPNST
• synovial sarcoma
• liposarcoma
• rhabdomyosarcoma
• fibrosarcoma
• leiomyosarcoma
• epithelioid sarcoma
• angiosarcoma
• dermatofibrosarcoma protuberans
• clear cell sarcoma
• alveolar soft part sarcoma (ASPS)

3. ETIOLOGY, CLINICAL PRESENTATION, &
DIAGNOSIS
• Remains unknown (certain environmental factors & genetic predispositions)
• Commonly : enlarging painless mass, can cause pain via nearby neurovascular structures
• Rapid growth  concern for a malignant
• Common in specific anatomic locations :
• Lower extremity : liposarcoma
• Upper extremity : synovial sarcoma, epithelioid sarcoma, fibrosarcoma
• <5% STS metastasize to lymph nodes, except :
• synovial sarcoma
• rhabdomyosarcoma
• epithelioid sarcoma
• clear cell sarcoma
• angiosarcoma

4. DIAGNOSIS OF A SOFT-TISSUE SARCOMA
1. ASSESMENT OF LOCAL EXTENSION
• compressing but not violate anatomic barriers (fascia or bone)
• MRI : gold standard  defining the local extent of the tumor & surrounding edema
• strongest predictor of local recurrence : positive surgical margin
• gadolinium differentiate
• cystic areas representing hemorrhage
• necrosis based on peripheral rim enhancement
• solid viable areas of tumor based on enhancement throughout the lesion

5. 2. HISTOLOGICAL FINDING
• biopsy  prior to excision in order to avoid inadequate surgery
• FNAB, CNB & open biopsy
DIAGNOSIS OF A SOFT-TISSUE SARCOMA

6. 3. STAGING
• AJCC & MSTS
• metastasize hematogenously (lungs) and 10% have
detectable at initial presentation
• bone scan  evaluate rare occurrence of metastatic
bone disease
• PET scan  staging of recurrent disease
DIAGNOSIS OF A SOFT-TISSUE SARCOMA

7. 3. STAGING
• American Joint Committee on Cancer (AJCC)
DIAGNOSIS OF A SOFT-TISSUE SARCOMA

8. 3. STAGING
• Musculoskeletal Tumor Society (MSTS)
DIAGNOSIS OF A SOFT-TISSUE SARCOMA

9. TREATMENT OF EXTREMITY STS
• “oncologic control” : minimizing each patient’s risk of local and systemic recurrence with
current treatment modalities
• adjuvant radiotherapy & cross-sectional imaging (MRI)  allowed more conservative
resection margins
• primary amputation rarely indicated except  very extensive and locally invasive disease
Ghert MA, Abudu A, Driver N, Davis AM, Griffin
AM, Pearce D, White L, O’Sullivan B, Catton CN,
Bell RS, Wunder JS (2005) The indications for
and the prognostic significance of amputation as
the primary surgical procedure for localized soft
tissue sarcoma of the extremity. Ann Surg Oncol
12(1), 10–17

10. RADIATION THERAPY
A. Preoperatively
• 50 Gy delivered in 2 Gy daily : 5 weeks
• surgery 4-6 weeks after the completion of
radiation
• encompasses the tumor & surrounding
region
• associated with a significantly higher wound
complication rate
• complicate short-term outcome  usually
resolvable & little impact on long-term
function
B. Postoperative
• begins approximately four to six weeks after
surgery
• 60–66 Gy delivered 30–33 daily fractions :
over six weeks
• likely to develop : fibrosis, lymphedema,
joint stiffness, pain
• significantly worse longterm functional
outcome, can be permanently disabling
TREATMENT OF EXTREMITY STS

11. • ability to ‘‘sculpt’’ the treatment volume 
less radiation to surrounding normal tissues
• <<< radiation dose
• <<< wound complication rate
• (-) surgical intervention for wound
complications
• (-) bone fractures
• <<< local recurrence (88% 5 year local
recurrence-free survival)
 favorable functional outcomes
IMAGE-GUIDED INTENSITY-MODULATED RADIATION THERAPY (IMRT)
GTV: red ; CTV: green; PTV: blue; radiotherapy dose
volume : yellow
TREATMENT OF EXTREMITY STS

12. TREATMENT OF EXTREMITY STS

13. SURGICAL MARGIN
• important impact  the only independent risk factor under
the surgeon’s control
• superficial STS/small and deep STS  high degree of local
control  true wide negative resection margins (1–2 cm of
surrounding normal tissue or a fascial barrier)
• Gerrand et al. classified positive margins : low & high-risk
• planned dissections critical structures + radiation : low risk
• unplanned positive margin : local recurrence > 30%
TREATMENT OF EXTREMITY STS

14. SURGICAL MARGIN
• O’Donnell et al.
• positive margin to a close dissection to spare neurovascular / bone  safe in local recurrence, but is
associated with worse cause-specific survival
• suggest that critical structures can be preserved  multidisciplinary treatment (unless
invade/encased by tumor)
• Tumor invades into bone
• resect a segment of bone  reconstruct the osseous defect
• periosteal margin  adequate local control combined with adjuvant radiation
• Fractures in the radiation
• problematic  does not reliably heal
• commonly affect the femur  combination of higher radiation dose & larger treatment field
• High risk  prophylactic internal fixation
TREATMENT OF EXTREMITY STS

15. SURGICAL MARGIN
• Fractures in the radiation
• problematic  does not reliably heal
• commonly affect the femur  combination of higher radiation dose & larger treatment field
• high risk  prophylactic internal fixation
TREATMENT OF EXTREMITY STS
Dickie CI, Parent AL, Griffin AM, Fung S, Chung PW, Catton CN, Ferguson PC, Wunder JS, Bell RS, Sharpe MB, O’Sullivan B (2009) Bone fractures
following external beam radiotherapy and limb-preservation surgery for lower extremity soft tissue sarcoma: relationship to irradiated bone length, volume,
tumor location and dose. Int J Radiat Oncol Biol Phys 75(4), 1119–1124.

16. SOFT-TISSUE RECONSTRUCTION
SKIN GRAFTING
• used in a well-vascularized wound  cover muscle and tendons with paratenon
• durable coverage of a radiated wound  enhanced with a negative pressure wound dressing
FLAPS
• used in exposed nerves, vessels, bone, tendon without paratenon or hardware
• local pedicled flaps or free flaps  anatomic location and size of the defect
• Townley et al. : preoperative radiation does not increase the rate of microvascular complications

17. FLAPS
SOFT-TISSUE RECONSTRUCTION

18. FUNCTIONAL OUTCOME
• Toronto Extremity Salvage Score (TESS)
• questionnaire which is validated to assess activity limitations
• Musculoskeletal Tumor Society (MSTS)
• MSTS-87 : physician rating based on function at specific anatomic locations (e.g. hip, knee)
• MSTS-93 : physician rating based on function of the entire extremity (upper vs. lower)
• Predictors of worse functional outcome :
• large tumor size
• high-grade tumors
• deep tumors
• resection of bone
• sacrifice of a major motor nerve

19. FUNCTIONAL OUTCOME

20. FUNCTIONAL OUTCOME

21. SUMMARY
• Extremity STS  aggressive and rare malignant tumors
• Several factors influence outcome : size, depth, grade, and tumor
• Following a tissue diagnosis & staging  multidisciplinary team approach
• Most patients are eligible  limb-salvage surgery + radiation.
• Following treatment the majority of patients : painless and functional extremity.

22. THANK YOU