PRINCIPLES OF MANAGEMENT

OF SOFT TISSUE SARCOMA

PRESENTER - AYENI F.B
MODERATOR DR MAI A.
SURGICAL ONCOLOGIST
ABUTH , ZARIA

OBJECTIVES
Highlight the Epidemiology and risk factors
for Soft tissue sarcoma
Discuss the principles involved in :
Diagnosis
Staging
Treatment

outline
Introduction
Statement of Surgical import
Principles of Management
Diagnosis
Staging
Treatment Options
Prognosis/Outcome
Follow up

Current / Future trend

Summary

introduction
Soft tissue :
Extra skeletal connective tissue
Muscles , Tendon , Fat ,Fascia , Synovium
> 50% body weight
All from primitive mesodermal tissue

Sarcoma :
Sarkoma ( Greek fleshy growth )
Malignant soft tissue tumour

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

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

...Stewart Treves syndrome

PRINCIPLE OF MANAGEMENT
- DIAGNOSIS
CLINICAL ASSESSMENT
IMAGING
BIOPSY

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

physical examination
General Examination
Status Localis
Site
Size
Tenderness
Consistency
Mobility

Other Systems

retroperitoneal sarcoma

general principle of diagnosis

Location
Deep tends to malignant

Size
Large malignant

Growth pattern
Rapidly growing - malignant

Metastasis
malignant

when a lump is not just a lump

Painful
> 5cm in diameter
Evidently growing
Recur after previous excision
Deep to fascia

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

investigation

USS
CT Scan
MRI
Endoscopy / Endoscopic USS
Others :
Chest X-Ray
CBC
E/U/Cr

BIOPSY

biopsy technique

Core needle biopsy

Incisional biopsy
Excisional biopsy
FNAC

tru cut needle

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

STAGING / GRADING
TNM / UICC

staging
Primary Tumour :
T1 Tumour </= 5cm
T1a superficial to muscular fascia
T1b deep

T2 Tumour > 5cm

T2a superficial
T2b deep

staging
Regional Lymph Node :
Nx regional lymph node can not be assessed
N1 no regional lymph node metastasis
N2 regional lymph node metastasis

Distant Metastasis :
Mx distant metastasis can not be assessed
M1 no distant metastasis
M2 presence of distant metastasis

grading

G1:
G2:
G3:
G4:
Gx:

Well-differentiated
Moderately differentiated
Poorly differentiated
Undifferentiated
Tumour grade can not be assessed

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

TREATMENT OPTIONS
SURGERY
CHEMOTHERAPY
RADIOTHERAPY
IMMUNOTHERAPY

...multidisciplinary approach
Surgeons
Radiation oncologists
Pathologists
Radiologists
Medical oncologists
Specialist nurses

Physiotherapists

surgery
Evolution of surgical management
Up until 1950s
Amputation

1950 1970s
Radical resection

1980s
Limb-Salvage

+ Chemotherapy and Radiation

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 re resection if possible

surgical options

Enucleation
Marginal excision
wide local excision
Compartmental resection
Amputation

soft tissue reconstruction

Types :
Immediate
Late

Options :
Primary closure
Skin grating
Flaps

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

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 5 7cm
Entire surgical scar should be included
Dose About 60Gy

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

summary
Stage 1
Surgery

Stage 2 and 3
Surgery + Radiotherapy +/_ Chemotherapy

Stage 4
Palliative therapy

Chemotherapy regimen
Single agent
Combination therapy
AIM
AD
MAID

limb salvage

Oncologic aspect

Achieve adequate margin

Functional aspect

Retain adequate function of salvaged limb

prognosis

Grade
Stage
Histology
Site

recent advances
Use of Trabectedin ( Yondelis )
Isolated limb perfusion

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

references
Soft Tissue Sarcoma What a General
Surgeon Needs to Know by Frederick C. Eilber
, M.D. , Assistant Professor of Surgery ,
Division of Surgical Oncology , UCLA Sarcoma
Program
Schwartz Textbook of Surgery , 8th Edition ,
Chapter 35
Oxford Textbook of Surgery , 2nd Edition ,
Chapter 37

references
Soft tissue 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

THANK YOU ALL FOR YOUR

ATTENTION