Soft tissue sarcoma-What is the role of Radiation

MANAGEMENT OF SOFT TISSUE
SARCOMA
Dr Sanudev SadanandanV P
Senior Registrar
Dept of Radiation Oncology
Baby Memorial Hospital

Contents
 Introduction
 Etiology & Presentation
 Investigation,Staging,Pathological classification
 Management
-Surgery
-Radiotherapy
-Chemotherapy
 Retroperitoneal sarcoma
 Follow up
 Prognostic factors
 Summary

Introduction
 mesenchymal neoplasms
 Adult <1%
 paediatric neoplasms 15%
 Incidence in United states 11280 cases in 2012

Introduction
 Can occur at any site
Extrimities 43%
Visceral 19%
Retroperitoneal 15%
Trunk/thoracic 10%
Other 13%
 Lower extremity > trunk > upper extremity >
H&N .

Etiological factors
 Genetic mutations-NF-1, RB, Gardner’s syndrome, LF
syndrome
 Radiation exposure (osteosarcoma, angiosarcoma)
 Chronic lymphoedema- Angiosarcoma
 Chemical exposure eg. arsenic, polyvinyl chloride (hepatic
angiosarcoma)
 Infections eg. HHV-8: Kaposi’s Sarcoma

WHO PATHOLOGICAL CLASSICATION 2002
Adipocytic tumors-Liposarcoma Chondro osseous tumor-CS/OS
Fibroblasic/myofibroblastic tumor Undifferentiated tr
Fibrohystiocytic tr-MFH 1.Synovial
2.Epitheloid
3.Alveolar soft part sa
4.Clear cell sarcoma
5.Extraskeletal myxoid chondrosarcoma
6.Extraskeletal Ewings/PNET
7.Desmoplastic small round cell tr
8.Extrarenal rhabdoid tr
9.Undifferentiated sarcoma/NOS
Smooth muscle -Leiomyosarcoma
Skeletal muscle-RMS
Vascular-AS
Periferal n-Malignan peripheral nerve
sheath tr

Presentation
 Asymptomatic mass-MC
 Pain - 33%
 Paraneoplastic symptoms eg. Fever
 Nodal swellings -Rare
 Features of Metastasis –Cough, Dyspnoea,
Hemoptysis (10%)

INVESTIGATION
 ESSENTIAL
Routine inv
MRI Scan +/- CT Scan
CT Chest
True cut Biopsy/Incision Biopsy
 OTHER USEFUL INV IN SELECTED SITUATIONS
Abdominopelvic CT Scan-AS,LMS,ES,M/RC-LS
MRI Total spine-M/RC LS
MRI/CT Brain- AS,Alveolar softpart
PET CT

MANAGEMENT
ONCO PATHOLOGIST
IMAGEOLOGIST
SURGICAL ONCOLOGIST
ORTHOPEDICONCOLOGIST
RADIATION ONCOLOGIST MEDICAL ONCOLOGIST
MULTIDISCIPLINARY
TEAM

STAGE WISE MANAGEMENT
IA LG SX--FOLLOW UP
IB LG SX ONLY if margin adequate
SX –PORT if close/positive margin
IIA Resectable without functional loss SX—PORT
IIB,III Resectable without fuctional loss SX ---PORT

Synchronous Stage IV
Single organ ,limited tumor bulk,
amenable to complete resection
Disseminated
Primary tr mx
+Metastatectomy/SBRT +/- CT
+/-RT
Palliation + BSC

SURGERY
 First intervention
 Most effective treatment to ensure cure.
 Function preservation
 Adequate Oncologic clearance

SURGERY
BIOPSY
-TRUCUT/OPEN
-EXPERIENCEDSURGEON
-NOVIOLATIONOF FASCIAL PLANES
-NEEDLETRACK & SCARTO BE EXCISEDATTHETIMEOF
DEFINITIVE SURGERY
*WIDE EXCISION
WITH NEGATIVE MARGINS
*LIMB SALVAGE SURGERY
Minimum margin 1 cm
Close <1 cm margin
AMPUTATION -GROSSTOTAL RESECTION IS EXPECTEDTO RENDER LIMB NON
FUNCTIONAL
-AT PATIENT PREFERENCE

PATHOLOGIC ASSESSMENT
HISTOPATHOLOGY REPORT
Organ/site/Sx Status of Margin
Primary Diagnosis-WHO
Depth of tumor
Status of LN
Size of tumor Other studies
IHC/EM
Molecular genetics
Grade-NCI-Histo/Loc/Necrosis
-FNCLCC-diff/mito count/necro
Additional
Mitotic rate
Vascular invasion
inflamatory infiltrate
Necrosis +/-,Type,% TNM Stage.

INDICATIONS-PORT
1.All deep seated tumors
2.All high grade tumors ,size>5cm
3.Intermediate grade tumor size >5cm
4.Repeated margin positivity
5.Tumor >5cm superficial
6.Close margin Intermediate/High grade

INDICATIONS-PREOP RT
 Unresectable disease
 Resectable disease but resection will lead to
significant functional loss

PREOP RT PORT
Treatment volume smaller-No need to
cover operated field
Treatment volume larger
Reduce seeding during surgery More seeding
Tumor regression and better resectability
Decreased risk of recurrence
Less toxic More toxic
No hypoxia-Blood supply uninterrupted Hypoxia in tumor bed may adversely affect
oucome
Disadv-Poor wound healing
-No complete HPR
Adv-Complete HPR available

RADIOTHERAPY PLANNING
 Positioning & Immobilisation
 Planning CT Scan 3-5 mm cuts with iv contrast
 Co register Preoperative MRI /CT
 3DCRT/IMRT preferred

RADIOTHERAPY PLANNING
 GTV –contour tumor in preop MRI –T1C
 CTV
---Initial GTV + Margin to encompass microscopic
spread
---Surgical Scar/Drain sites/Surgical clips
---GTV to CTV Margin 3 cm longitudinally (RTOG)
1.5 cm laterally

CTV-PTV Margin 5-10 mm
 OAR & Constraints—Depends on primary
site.

Example Case
 55-year-old male with a large high-grade round
cell liposarcoma in right distal thigh. Clinical
stage (AJCC 7th edition) IIIT2bN0M0G3.
 The MRI of right distal thigh showed a large well
circumscribed heterogeneous, multiloculated mass
located within the posterior thigh.

 The tumor measured 14.8 cm in craniocaudal
dimension, 7.8 cm in AP dimension, and 11.3 cm in
maximal medial-lateral dimension.
 Simulation CT images were fused with those
from the diagnostic thigh MRI

Brachytherapy
 Surgical resection + Frozen section
 Place tumor bed clips
 HDR tube placed at 1cm spacing
 Target vol –Tumor + 2cm margin
 Planning-Paris system
 Load on POD4/D5

IORT
 Surgical resection + Frozen section
 TV=Tumor with 2 cm margin
 Electrons/photons
 Single sitting

Trials
Trial Results
Pisters et al 160 extremity & trunk Randomized
to BT/Obs
42–45 Gy over 4–6d)
BT-LC for high-grade lesions
(65–90%), but not for LG.
No difference in DSS /DM.
NCI
(Yang et al. 1998):
rct
140 ,extremity sarcoma, WLE.
LG to obs vs. PORT
HG post-opCT vs.
post-op chemo-RT.
RT = large field to 45 Gy → boost to
63 Gy.
RT increased LC for low-grade
(60% vs. 95%) and high-grade
(75% vs. 100%).
No difference in OS /DMFS
NCI
(Rosenberg et al.
1982):
43 , HG STS ,extremity
WLE + PORT vs. amputation alone.
RT = 45–50 Gy to compartment
with boost to 60–70 Gy.
No difference in LC, OS, or
DFS.
Chemo decreased LR and
increased DFS (60% vs. 90%)
and OS (75% vs. 95%).

Pre-op or Post-op RT
NCIC (O’Sullivan et al.
2002; Davis et al. 2005):
190 patients
with extremity STS
randomized pre-op RT (50
Gy) vs. post-op
RT (66 Gy). If +margins,
pre-op got 16 Gy boost..
No difference LC /DM /PFS
Preop-woundhealing
PORT-late fibrosis
Pollack et al. (1998): post-op RT
(60–66 Gy) Vs pre-op RT
(50 Gy) before excision or
reexcision.
.
No difference in LC
presenting with gross
disease, best LC with pre-
op RT (88% vs.
67%)
presenting after excision -
immediate reexcision and
post-op RT (LC 91% vs.
72%).
wound-healing --pre-op

IORT
Oertel et al. (2006): n=153
primary or recurrent
extremity STS
limb-sparing surgery + IORT
10–20 Gy → post-op EBRT
36–50 Gy.
Five-year OS 77%, DMFS
48%, and LC 78%. IORT
dose >15 Gy improved
LC, but EBRT <45 or 45
Gy not significant for LC.
Acute wound-healing
toxicity.-High
NCI
(Sindelar et al. 1993):
N=35 ,resectable
retroperitoneal STS
randomized to surgery +
IORT 20 Gy → post-op 35–40
Gy vs. surgery → post-op 50–
55 Gy.
No difference in 5-year
OS (35%), nonsignificant
increase in LC ,IORT
increased neuropathy if
>15 Gy.
Alektiar et al. (2000): primary or recurrent
retroperitoneal STS
surgery + IORT 12–15 Gy →
post-op EBRT 45–50 Gy.
5-year OS 55%, DMFS
80%, LC 62%, 10%
neuropathy

BENEFIT OF PORT
 Enucleation-80% recurrence
 WE with negative margin- 30%
 WE with negative margin and PORT-5%
 Adjuvant radiotherapy improves local control
without benefit in Overall survival

Toxicities
 Hair loss
 Skin telangictasia
 Skin fibrosis
 Lymphoedema
 ORN
 Pathological #
 Radiation induced 2nd malignancies

CHEMOTHERAPY
 Indications
1. Adjuvant
HG extrimity sarcoma
Tumor size >5 cm
2.Neoadjuvant
Unresectable disease
3.Palliative
To palliate symptomatic mets

 Regimen
ADR 60mg/m2 IV D1
IFOSFAMIDE 1.3gm/m2 IV D1-D3
q3weekly x 6 cycle
 2nd line
Doce+Gem,Pazopanib

Benefit in adjuvant setting
 Metaanalysis ( Pervaiz et al. 2008):
 N=1,953 resectable STS
 WLE ± RT ---observation vs. adjuvant doxorubicin-based
chemo.
 Chemo improved LC (absolute 4%), DMFS (9%), RFS (10%),
and OS (6%).
 Specifically doxorubicin/ifosfamide improved LC (absolute 5%,
not significant), DMFS (10%), RFS (12%), and OS (11%).
 No trial of pre-op vs. post-op chemo.

MANAGEMENT OF
RETROPERITONEAL SARCOMA

 34% of all STS
 MC- liposarcoma (40%), leiomyosarcoma
(25%), malignant peripheral nerve sheath
tumour and fibrosarcoma
 MC visceral STS -GIST, leiomyosarcoma and
desmoid tumour

Presentation
 Asymptomatic mass
 Pain
 Gastrointestinal bleeding
 Incomplete obstruction
 Neurological symptoms due to invasion of
neurovascular structures

Imaging
 CT-abdomen
 Also allows evaluation of the liver, the most
common site of metastasis
 Staging
 No official staging system
 The same grading system applies as for
extremity STS

Diagnosis
 Laparotomy with open biopsy
 CT guided biopsy has a limited role only
 Only if:
- unresectable tumour
- doubtful diagnosis
- neoadjuvent chemotherapy considered

Treatment
 Surgery -The mainstay of treatment
 Chemotherapy principles are the same as for
extremity STS
 Radiotherapy
-High morbidity and mortality due to
radiosensitivity of surrounding organs
-Intensity-modulated radiation showing
promising results

prognosis
 Adverse factors for local recurrence:
+ margins
>50 years age
deep location
fibrosarcoma type including desmoid, malignant
peripheral nerve sheath tumors.
 Adverse factors for distant metastasis:
high-grade (at 5 years, <10% for low-grade, 50% for high grade)
increasing size
deep location,
leiomyosarcoma or malignant peripheral nerve sheath
tumor
high Ki-67.

Follow up
 H&P
 CXR/CT Chest 3-6 mon x 2-3 yr
6monthly x 2yr
Annual
 Baseline and periodic imaging of primary site

Recurrence
 Local relaspse-Work up
 Metastasis
Single organ &Limited tumor bulk-
Metastatectomy+/-CT/RT
Ablation
Isolated Node-Nodal dissection+/- RT/CT
Disseminated mets-Palliation

Survival
StageI extrimity 5-year LC 90–100%, OS 90%
II–III extremity ~5-year LC 90%, OS 80% for stage II,
60% for stage III.
For recurrence, amputation salvages
~75%
Stage IV EXTRIMITY Limited mets~5-year OS ~25%.
Disseminated ~5-year OS 10%
Retroperitoneal ~5-year LC 50%, DM
20–30%, OS 50%

Take home message
 STS are hterogeneous neoplasms
 Management of STS requires multidisciplinary
tumor boards and close collaboration between
specialists.
 Surgery is the most important form of treatment
 Radiotherapy helps to improve local control.
 Chemotherapy can be utilised in selected situations
in adjuvant/Neoadjuvant treatment.

References
 NCCN V2.2014
 Textbook of Radiotherapy planning –Dobbs
 Textbook of Radiation Oncology-Leibel
Philips,Perez
 RTOG Sarcoma contouring guideline
 Online resources

Soft tissue sarcoma-What is the role of Radiation

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