EXTREMITY STS
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EXTREMITY STS
Dr. S. SRIVATSAN
Dr. M. ANTO, Dr. THIRUMURUGANAND
Prof. K.S.RAVISHANKAR UNIT
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50 % of STS- Extremity STS
Extremity sarcomas represent almost half of all patientswith STS.
Much of the treatment of sarcomas at non-extremity siteshas been extrapolated from evidence in clinical trials of patients with extremity sarcomas.
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STAGING STS EXCEPTIONS Sixth edition of AJCC staging system applies toall soft tissue sarcomas with the exception of
Malignant mesenchymoma Angiosarcoma, Infantile fibrosarcoma Dermatofibrosarcoma.
Mn emo nic: MAID
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STAGING KEY POINTS
STAGE 1- All low grade lesions
STAGE 2- High grade, small, large &superficial lesionsSTAGE 3- High grade , large & deep lesionsSTAGE 4- Any nodal or distant Mets
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N1 is Stage IV - CARESSarcoma Type Nodal Metastases (%)Clear cell sarcoma 25-50
Angio sarcoma 11-40
Rhabdomyosarcoma 11-36
Epithelioid sarcoma 17-80
Synovial sarcoma 2-17
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Grade of the tumor
T he best indicator of a tumor's biologicaggressiveness and metastatic potential is itsgrade, regardless of - Histological type
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Grade of the tumor
Cellularity Differentiation Pleomorphism Necrosis (most important) Mitosis
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TUMOUR GRADE VITAL The central importance of histologic grade in thestaging system is unique to STS.
Histologic grade, tumor size and depth are theprimary determinants of AJCC stage.
Histologic grade is the most important factor inpredicting the risk for d is ta n t meta s ta sis andtumor-related death
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TUMOUR SIZE 5 cm a- Superficial to Deep fascia b- Deep to or involves Deep fascia.
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Prognosis
Proximal vs Distal Deep vs Superficial More than 5 cm vs Less than 5 cm
High grade vs Low grade
POOR GOOD
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HISTOLOGY OF EXTREMITYSTS
PROXIMAL EXTREMITY Malignant fibrousHistiocytomas( MFH)
Liposarcomas Leiomyosarcomas
DISTAL EXTREMITY Synovial sarcomas Epithelioid sarcomas Clear cell sarcomas
HAND
Epithelioid sarcoma
FEETSynovial sarcoma
Clear cell sarcoma
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METASTASIS
The most common site of metastases for extremity sarcomas is the lu ng .
Other sites of metastases include thelymph nodes and bone.
Epithelioid and clear cell Sarcoma Lymphnode Mets
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PROGNOSIS
Most important factor in predicting localrecurrence is the presence of po si t iv e mar gins o n s ur gic al ex cisi o n.
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L IPOSARCOMA OF RIGHT ARM
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LIPOSARCOMA OF RIGHTTHIGH
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CLINICAL PRESENTATION
Most common presentation of a soft tissuesarcoma is a p ainless mass .
Most commonly occur proximally in the hipand shoulder regions
The patient may first notice the mass whenan unrelated injury occurs to the affectedextremity.
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Pre operative MRI vs CT
MRI may be most useful for determiningthe extent of soft tissue and neurovascular invasion.
CT can be useful for determining the extent
of local invasion as well as the extent anddegree of any bone invasion
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Liposarcoma Thigh addductorcompartment
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MFH of Popliteal region
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DIAGNOSIS AND EVALUATION
Pretreatment biopsy Helpful not useful always
Pretreatment biopsy is mandatory when thetumor seems to involve critical structures or whenneoadjuvant therapy is considered
Most commonly, c ore n eedle bi op sy is used andprovides adequate tissue diagnosis
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DIAGNOSIS AND EVALUATION
Principles of biopsy Assess extent of local disease Assess presence or absence of distantmets
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PRINCIPLES OF BIOPSY
Incision to be placed longitudinally Avoid rising flaps Perfect haemostasis to be achieved Avoid using drains while taking biopsy Biopsy site must be included in thedefinitive surgery.
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Biopsy tract - cells
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METASTATIC WORK-UP
CXR or CT CHEST PET Scan- Not performed routinely
Current Trends:FDG-PET + CT To predict disease free
survival in pts receiving neo-adjuvanttreatment
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SURGERY FOR STS AMPUTATION
COMPARTMENTAL EXCISION
WIDE LOCAL EXCISION WITH NEGATIVEMARGINS
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Limb function preserving Surgery-PRINCIPLES
Surgical resection with 2cm margin
The resected tissue should include theunviolated tumor, pseudocapsule, and reactivezone with a wide margin
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Any previous biopsy site or scars should becontained within the final specimen.
Incisions should be placed longitudinally tofacilitate resection with minimal violation
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A barrier to tumor infiltration can include tissuessuch as fascia, joint capsule, tendon, epineurium,and the vascular sheath.
The guidelines dictate that if a barrier to spreadexists, the tumor should be removed outside of that barrier (ie, without violation).
Limb function preserving Surgery-PRINCIPLES
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If there is no barrier to spread, the tumor isremoved with a broad margin.
Drain sites should be placed in proximity tothe surgical incision to facilitate the safe
inclusion in radiotherapy field.
Dont see the tumour
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Adductor Muscle GroupExcision
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If a positive margin is obtained at the time of
resection, a re-resection should be performed withthe goal of obtaining a negative margin.
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Limb-sparing resection Contraindications
1.Groin involvement
. .
2. Extracompartmental extension .3. Intrapelvic extension .4. Superficial femoral artery or common femoral
artery involvement.5. Femur involvement .6. Palliation .
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RECONSTRUCTION Vascular reconstruction to salvage limb? Graft or flap for wound closure? Pre-op vs post- op RT?
Vascular reconstruction tosalvage limb confers no added advantage , though it increasesthe morbidity of the procedure .
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Graft/ flap cover planned simultaneously
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RADIOTHERAPY External beam radiation is delivered Mondaythrough Friday over the course of 6 to 7 weeks.
Brachytherapy is usually employedpostoperatively with treatment delivered over several hours or days.
Wide excision of STS followed by adjuvantradiotherapy results in local control rates inexcess of 80%
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RADIOTHERAPY Use of radiotherapy is associated with animprovement in local control after surgicalresection w i thout a ny inf lue nc e o no v erall s ur viv al or d is ta n t meta s ta s e s .
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BENEFITS OF POST-OP RTLess risk of surgical wound complicationsAllows selection of patients at the highestrisk for recurrence based on surgicalpathology.
PRE-OP vs POST OP RT
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In patients with large, marginally resectablelesions, a preoperat iv e approa c h istypically preferred to improve the likelihoodof a margin-negative resection and to allowa greater likelihood of function preservation
PRE-OP vs POST OP RTVERDICT
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For patients in whom the risk of wound com p lications isp rohibitively high , specially in smaller more readilyrespectable lesions, a p osto p erative a pp roach may bepreferred to decrease this risk while accepting an increasedrisk of late toxicity.
Small, su p erficial, or low-grade lesions or those with a
questionable pathologic diagnosis, a postoperativeapproach may be preferred to allow a determination of theappropriateness of adjuvant radiotherapy.
PRE-OP vs POST OP RTVERDICT
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BRACHYTHERAPY ON POD 5
For delivering radiation at the time of surgery(intraoperatively) or shortly thereafter
Involves placing catheters within the tumor bed atthe time of surgery
Radioactive sources can then be placed in thecatheters to deliver radiation to the tissues
surrounding the resection cavity, typically after postoperative day 5 to minimize woundcomplications
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RADIOTHERAPY- FINAL WORD
Radiation can enhance local control after surgical resectionR ad i otherap y doe s n ot c ompe ns ate f or s u b opt i mal re s e c t i o n
Use of adjuvant radiotherapy should not beseen as the alternative to a margin-negative resection, even if re-resection isrequired.
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Special considerations: distal
extremities
Distal extremity sarcomas present a uniquechallenge based on the anatomic andfunctional constraints.
Lesions involving the wrist, hand, ankle, or feet more frequently are in proximity to or involve vital neurovascular structures or muscles, joints, and tendons critical tofunction.
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DISTAL EXTREMITY-
CONSERVATIVE SURGERY VSAMPUTATION
Use of surgery alone for patients with distalextremity lesions results in a higher rate of local recurrence
Use of limited surgery and radiotherapy for sarcomas of the hand-wrist and ankle-footcomplex to obtain local control whilemaintaining a functional distal extremity
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DISTAL EXTREMITY-
CONSERVATIVE SURGERY VSAMPUTATION
When do you prefer Amputation for distal extremity STS?
1. For patients who decline conservative surgery and radiotherapy
2. Instances in which negative margins cannot be obtained
3. When amputation is expected to have minimal functionalconsequences (ie, ray amputation)
4. Limited surgery with reconstruction may lead to inferior functional
outcomes when compared with amputation with prosthesis
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ADJUVANT CHEMO At this time, the role of chemotherapy for resectedextremity soft tissue sarcomas is uncertain.
Patients with a high risk of metastatic diseaseappear to benefit from chemotherapy.
These patients include those with large, highgrade, and deep lesions.
The recommendation to deliver chemotherapymust be individualized based on the risk of distantfailure balanced with the risks of chemotherapy.
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ADJUVANT CHEMO- STUDIES There was a significant improvement indistant rela p se-free interval and in overall recurrence- free survival with the additionof Adriamycin-based adjuvantchemotherapy;
However, there was n o b e n e fi t in o v erall s ur viv al
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Currently, the neoadjuvant approachremains investigational based on the lackof randomized data supporting its use andthe significant toxicity that results from thisapproach
NEOADJUVANT THERAPY
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Recurrence depends on
High grade tumors Tumor Size > 5 cm Violation of tumor capsule Positive margins
Early recurrence is Grade &Late recurrence is Size
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Recurrent tumors Popliteal region
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RECURRENT DISEASE Isolated local recurrence- evaluted andtreated as a new primary
Isolated metastasis- metastatectomy
Widely disseminated disease- palliativetherapies, including surgery,chemotherapy, radiotherapy, embolization,and ablation procedures
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FOLLOW UP-NCCN Guidelines Patients should be followed up with a history andphysical examination
Every 3 to 6 months for 2 to 3 years, Every 6 months for the next 2 years, and thenannually
For stage I tumors, chest imaging (radiography or CT) should be performed every 6 to 12 months;
For stage II and III tumors, chest imaging shouldbe performed more frequently, every 3 to 6months for 5 years and then annually.
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FOLLOW UP NCCN GUIDELINESPeriodic imaging with MRI or CT of the p rimary site
should be considered if the combination of factorsplaces the patient at increased risk for locoregional recurrence, especially if the locationor depth of the lesion makes physicalexamination unreliable for this determination.
Ultrasound, instead of MRI and CT, can also beconsidered as the mode of surveillance in thesecircumstances.
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FOLLOW UP NCCN GUIDELINES
After 10 years, the chance of local recurrenceif the patient remains disease free becomesmuch smaller, and the requirement for surveillance imaging after this time pointshould be individualized.
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