Principles of Management of Soft Tissue Sarcoma

8/10/2019 Principles of Management of Soft Tissue Sarcoma

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PRINCIPLES OF MANAGEMENT

OF SOFT TISSUE SARCOMA


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OBJECTIVES

Highlight the Epidemiology and risk factors

for Soft tissue sarcoma

Discuss the principles involved in :

Diagnosis

Staging

Treatment


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outline

Introduction

Statement of Surgical import

Principles of Management

Diagnosis Staging

Treatment Options

Prognosis/Outcome

Follow up

Current / Future trend

Summary


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introduction

Soft tissue :

Extra skeletal connective tissue

Muscles , Tendon , Fat ,Fascia , Synovium

> 50% body weight

All from primitive mesodermal tissue

Sarcoma :

Sarkoma ( Greekfleshy growth )

Malignant soft tissue tumour


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introduction

Simply not grouped into Benign and Malignant

Heterogenous group of tumours

Relatively rare

Can occur throughout the body

> 50 histiotypes

Dominant pattern of metastasis is

hematogenous

Almost always arise De novo


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risk factors / aetiology

Genetic predisposition

Neurofibromatosis , Li-Fraumeni syndrome

Radiation Exposure

Poor prognosis Chronic Lymphoedema

Post surgical , irradiation , Parasitic infection

Chemical Carcinogenesis Thorotrast , polyvinyl chloride , arsenic

??? Trauma


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...Stewart Treves syndrome


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PRINCIPLE OF MANAGEMENT

- DIAGNOSIS

CLINICAL ASSESSMENT

IMAGING

BIOPSY


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history

Biodata - Age

Presenting Complaint

Mass / Lump

Other Symptoms depending on site History of Presenting Complaint

Duration , Site , Symptomatic , Progression ,

Past Medical History

Previously excised ?

Family and Social History


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physical examination

General Examination

Status Localis

Site

Size

Tenderness

Consistency

Mobility

Other Systems


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retroperitoneal sarcoma


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general principle of diagnosis

Location

Deeptends to malignant

Size

Largemalignant

Growth pattern

Rapidly growing - malignant

Metastasis

malignant


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when a lump is not just a lump

Painful

> 5cm in diameter

Evidently growing Recur after previous excision

Deep to fascia


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radiological investigation

Assist in :

Defining local extent of the tumour

Percutaneous biopsy

Staging

Diagnosis

Benign Versus Malignant

Monitoring response to treatment

Detecting recurrence after surgical resection


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investigation

USS

CT Scan

MRI

Endoscopy / Endoscopic USS

Others :

Chest X-Ray

CBC E/U/Cr

BIOPSY


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biopsy technique

Core needle biopsy

Incisional biopsy

Excisional biopsy FNAC


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tru cut needle


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biopsy

Principle :

Done in a designated centre

Surgeon to perform the definitive resection

Adequate pre operative planning

Incision centered over the mass in its most

superficial location

Incision parallel important structures Do not raise tissue flap


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STAGING / GRADINGTNM / UICC


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staging

Primary Tumour :

T1Tumour 5cm

T2asuperficial

T2bdeep


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staging

Regional Lymph Node :

Nxregional lymph node can not be assessed

N1no regional lymph node metastasis

N2regional lymph node metastasis

Distant Metastasis :

Mxdistant metastasis can not be assessed

M1no distant metastasis

M2presence of distant metastasis


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grading

G1: Well-differentiated

G2: Moderately differentiated

G3: Poorly differentiated G4: Undifferentiated

Gx: Tumour grade can not be assessed


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stage grouping

Stage IA G1,2 T1a,b N0 M0

Stage IB G2,2 T2a,b N0 M0

Stage IIA G3,4 T1a,b N0 M0

Stage IIB G3,4 T2a N0 M0

Stage III G3,4 T2b N0 M0

Stage IV Any G Any T N1 M1


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TREATMENT OPTIONS

SURGERY

CHEMOTHERAPYRADIOTHERAPY

IMMUNOTHERAPY


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...multidisciplinary approach

Surgeons

Radiation oncologists

Pathologists Radiologists

Medical oncologists

Specialist nurses Physiotherapists


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surgery

Evolution of surgical management

Up until 1950s

Amputation

19501970s

Radical resection

1980s

Limb-Salvage

+ Chemotherapy and Radiation


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surgical resection

Principle :

Thorough pre operative planning

Complete removal of the tumour

Microscopically negative margin

Preservation of maximum function

Biopsy site should be removed en bloc with

specimen Positive margin require reresection if possible


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surgical options

Enucleation

Marginal excision

wide local excision

Compartmental resection

Amputation


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soft tissue reconstruction

Types :

Immediate

Late

Options :

Primary closure

Skin grating

Flaps

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Immediate soft tissue

reconstruction

Indications :

Vital structure / organ remains exposed

Exposed major nerves , tendons , vessels , bones

, joints , replacement prosthesis

Large wound defect , wound cavity or tension at

suture line

Planned post operative radiotherapy /chemotherapy


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radiotherapy

Optimal mode and timing yet to be

determined

Pre operatively or post operatively

CT Scan is an integral part of radiation

therapy

Optimal radiation margin of 57cm

Entire surgical scar should be included

DoseAbout 60Gy


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chemotherapy

Indicated in :

Stage 4 disease

Small cell sarcoma of any size

Large (>/= 5cm ) high grade tumours Intermediate grade tumours >10cm

Agents

Doxorubicin Ifosfamide

dacarbazine


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summary

Stage 1

Surgery

Stage 2 and 3

Surgery + Radiotherapy +/_ Chemotherapy

Stage 4

Palliative therapy


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Chemotherapy regimen

Single agent

Combination therapy

AIM

AD

MAID

li b l


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limb salvage

Oncologic aspect

Functional aspect

Achieve adequate margin

Retain adequate function of salvaged limb


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prognosis

Grade

Stage

Histology

Site


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recent advances

Use of Trabectedin ( Yondelis )

Isolated limb perfusion


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conclusion

Prompt diagnosis and referral are desirable

Surgery remains the mainstay of treatment

Radiotherapy is useful in selected cases

Conventional chemotherapy has little effect

on the outcome of most tumours


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references

Soft Tissue SarcomaWhat a GeneralSurgeon Needs to Know by Frederick C. Eilber, M.D. , Assistant Professor of Surgery ,

Division of Surgical Oncology , UCLA SarcomaProgram

Schwartz Textbook of Surgery , 8thEdition ,Chapter 35

Oxford Textbook of Surgery , 2ndEdition ,Chapter 37


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references

Softtissue sarcoma of the Extremities by

Vallery Dronsky , MD . SUNY Downstate

Medical Centre , Brooklyn Veterans Hospital

Soft Tissue Sarcoma by Dr Janice N. Cormier ,MD et al , Department of Surgical Oncology

and Biostatistics , University of Texas ,

Houston , TX


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Documents

Principles of Management of Soft Tissue Sarcoma