By: Dr. Mohammad Mujib Sakhi
Consultant General surgery
Surgical View of Sarcomas
INTRODUCTION:
Sarcomas are a heterogeneous group of tumors
 Embryonic mesoderm
 Also can originate, from the ectoderm.
 peripheral nervous system,
Types of sarcoma
According to WHO classification more than 50 histiotypes
• In children:
• Rhabdomyosarcoma
• in adults
• Pleomorphic sarcoma (MFH),
• GIST,
• liposarcoma,
• leiomyosarcoma,
• synovial sarcoma,
• malignant peripheral nerve sh
eath tumors
Most common subtypes of STS
• M ost common primary sites
• Extremities (60%),
• Trunk (19%),
• Retroperitoneum (15%)
• Head and neck (9%)
•Most common metastatic sites
• Generally : lungs
• With abdominal tumors: liver and peritoneum
• Regional lymph nod 3.7%
 Risk factor
 Genetic factors
 Environmental factors
 Prior radiation therapy
 Viral infections,
 immunodeficiency
 Sites
 Scar tissue,
 fracture sites,
 prior soft tissue trauma
• Mole cular Diagnosis of STS
• (i) specific genetic alterations
• simple karyotypes
• chromosomal translocations
• point mutations
• (ii) non-specific genetic alterations
• complex unbalanced karyotypes.
Cause unknown
Risk factor Mole cular Diagnosis of STS
 History : painless bulging/painfull
 Physical exam: 1. Size of the mass




2.Mobility
3.Superficial/deep
4.Relation to nearby NV and bony structures.
5.Regional lymph nodes.
No screening tests. diagnosis
Triple assment
annamnesis
Imaging
pathology
 MRI is the choice in extremities
 Enhances the contrast between tumor , muscles and with blood vessels.
 Follow up 3 monthly MRI are done to see recurrence
 CT may be helpful in intra abdominal and few types of sarcomas
 Abdominal- Spiral CT to know the relation with neuro-vascular structures
 CT chest and MRI brain may be required to see metastasis
 Ultrasonography if MRI is contraindicated
 An X ray may help in bone involvement
 Chest X ray: for low grade lesions <10 cm or intermediate or high grade<5cm.
Should be perform before any invasive procedure
 Biopsy Techniques –
 Fine-Needle Aspiration Biopsy
 Core Needle Biopsy (choice)
 High diagnostic accuracy,
 Ease of performance
 Low cost
 Less complications
 Incisional Biopsy
 Excisional Biopsy – small cutaneous or subcut <5cm
cont…. (pathologic examination)
 It is recommended to obtain a diagnostic biopsy
prior to definitive treatment for all soft tissue masse
• T1: <= 5 cm
• A: superficial ( to and not invading superficial fascia)
• Deep ( to or invading superficial fascia)
• T2: > 5 cm
• A: superficial ( to and not invading superficial fascia)
• Deep ( to or invading superficial fascia)
• No T3 or T4
• N1: regional LN (RARE)
• M1: distant mets
•Grading:
•G1: will Differentiated
•G2: Mederatly Differentiated
•G3 Poorly Differentiated
•G4: Un Differentiated
T1 T2 N1 M 1
G1, GX IA IB III IV
G2 IIA IIB III IV
G3 IIA III III IV
• Mainstay
• Standard primary treatment for most sarcomas
• Problems: recurrence, incomplete resection for
difficult sites
• Resect the tumor with appropriate negative margins (>1 cm)
• Close margins (<1 cm) may be necessary to preserve uninvolved critica
l neurovascular structures, bones, joints.
compartment resection is no
t routinely necessary
Surgical margin (SM) and residual (R)
• Negative SM = R0
• Adequate: >1cm
• Close: < 1cm
• Adj RT is given in close margins
• Positive SM = R1 or R2
• R1 resection - Microscopic residual disease
• R2 resection - Gross residual disease
• surgical re-resection to obtain negative margins should strongly be considered i
f it will not have a significant impact upon functionality
• Adj RT is given in microscopically positive margin (R1) on bone, major blood ve
ssels or a nerve
• Uncertain margin:
• Consult radiotherapist
Treatment of special presentation
and etities
• Limb sparing surgery (LSS) is recommended to preserve function
• Amputation
• non-functional limb
• infeasible LSS
• patient preference
• If adequate initial surgery cannot be done:
• Preoperative chemo or radio or chemoradio
• To decrease local recurrence
• Chemo or radio can be used (either pre or post)
• Negative SM is always desirable and may need re-resection
• Adjuvant RT in:
• Close SM (<1 cm; R0)
• Microscopic + SM (R1) on bone or major blood vessels
Surgery for Extremity STS
Retroperitoneal Sarcomas
• 15% of all sarcomas
• Mostly types
• Liposarcoma 43%
• Liomyosarcoma26%
• Ct scan shows in relation to surrodings
• Cystic /solid /necrotic component
• Biopsy not necessary unless suspect:
• Lymphoma
• Germ cell tumor
• Plan prop chemo or radiation
• En bloc resection is standrad treatment
• Bowl prep
• Asses bilateral kidney function
• 50-80% need organ resection
• 78% of primary lesions can be completely resected
Retroperitonial sarcoma cont..
Chemo radiation for Retroperitonial sarcoma
Radiation therapy
• GI and neurotoxicities limit delivery of sufficient doses
• May improve local control (clinical trails)
Chemotherapy
• Indication
• Recurrent
• Unresectable
• metastatic
Surgical Resection cont…
Role Of Debulking Surgery
No survival benefit for incomplete resection
In paitent with unresectable retroperitoneal STS.
Gastero intestinal stromal tumor(GIST)
Separate subtype of sarcoma defined by
• expression of c-kit(CD117)
• Surgery
• Complete resection without local or regional
lymphadenectomy
• Very resistant to traditional chemotherapy
• Imantinib mesylate (gleevec)
• C-kit is constitutively active tyrosin kinase receptor
• Initial studies showed 54% respons rates
• Two RCTs currently looking at adjuvant treatment
Criteria for unresectability
Radiographic Findings indicate unresectability
a) Extensive vascular involvement
a)Aorta
b)Vena cava
c) Illiac vessel
b) Peritonial implant
c) Distant mets
d) Involvement of the root of the mesentery (sup mesentery vessels)
e) Spinal cord involvement
Relative contraindication
Interposition grafts
Vascular sarcoma
• Most vascular types
1. Angiosarcoma
2. Hemangiosarcoma
3. Lymphangiosarcoma
4. Heangiopericytoma
• No clear role of adjuvant chemoradiation therapy
• High risk for bleeding during excision
Breast srcomas
• 1% of all breast neoplasm
• Wide excisoin with negative margin
• No clear role of adjuvant chemoradiation therapy
Prognostic factors
Increased risk of local recurrence
1. Age >30
2. Recurrent disease
3. Positive surgical margin
Increased risk of distant metastasis
I. Size >5cm
II. High grade
III. Deep location
IV. Recurrent disease
• Source:
• EBRT: conventional or IMRT
• Brachytherapy
• Timing
• Preoperative: 50 Gy
• Easier surgery
• Poor wound healing
• Boost if close or positive SM
• Postoperative
• Improve local control in high-grade extremity STS with
positive SM or higher stage (III), old age
• May be partly given immediately (Intraoperative) and
completed later
Chemotherapy or chemoradiation
• Preop chemoradiation:
• Value: increase local control, DFS and OS
• CT RT±CT Surgery ±CT
• Regimens:
• Doxorubicin (30 mg/m2/d x 3) concurrent with RT (
300 cGy x 10)
• IMAP x 2 RT±MAP on rest days (0, 21, 42) IOR
T
• MAID+RT (44 GY split) surgery MAID x 3 if S
M+
• Preop chemotherapy:
• Value: inconsistent
• CT surgery ±CT
• Regimens:
• MAID
Chemotherapy
• Postop (adjuvant) chemotherapy:
• Value: improve RFS and OS of extremity STS
• EORTC trials lack OS benefit??
• surgery CT
• Regimens:
• Doxorubicin based (doxo-ifos)
• Epirubicin based (epi-ifo)
Treatment of STS of extremities
and trunk
G Obs
erve
Preop Preo Preop RT pCT
CRT
Surg Posto
p RT
Posto
p CT
Posto
p CRT
I T1 (small, <5) 1 √ may
T2 (large, >5) 1 √ √
II T1 (small, <5) 2,3 M ay M ay √ √ M ay
T2 (large, >5) 3 M ay M ay √ √ √ M ay
III T2 (large, >5) 3 M ay M ay √ √ √ M ay
N1 M ay M ay √ + Radi
cal LND
√ M ay
IV Limited M 1
Dissemin’
d M1
May if May
Sym-
M AY May
Post op RT if : SM <1cm, non-intact fascia
l plane
Treatment of STS of retroperitoneum
or intra-abdominal
Post op RT if : SM <1cm, non-intact fascia
l plane
Obs
erve
Preop
RT
Preo
pCT
Surg Posto
p RT
Posto
p CT
Resectable M ay M ay √ ± IORT M ay
in R1
or
Boost
M ay
Unresectable √ √ √ if becomes
resectable Otherwise
as M !
IV Limited M 1
Dissemin’
d M1
May
if
Sym-
May M AY May
berifely  sarcoma

berifely sarcoma

  • 1.
    By: Dr. MohammadMujib Sakhi Consultant General surgery Surgical View of Sarcomas
  • 2.
    INTRODUCTION: Sarcomas are aheterogeneous group of tumors  Embryonic mesoderm  Also can originate, from the ectoderm.  peripheral nervous system,
  • 3.
    Types of sarcoma Accordingto WHO classification more than 50 histiotypes • In children: • Rhabdomyosarcoma • in adults • Pleomorphic sarcoma (MFH), • GIST, • liposarcoma, • leiomyosarcoma, • synovial sarcoma, • malignant peripheral nerve sh eath tumors Most common subtypes of STS
  • 4.
    • M ostcommon primary sites • Extremities (60%), • Trunk (19%), • Retroperitoneum (15%) • Head and neck (9%) •Most common metastatic sites • Generally : lungs • With abdominal tumors: liver and peritoneum • Regional lymph nod 3.7%
  • 5.
     Risk factor Genetic factors  Environmental factors  Prior radiation therapy  Viral infections,  immunodeficiency  Sites  Scar tissue,  fracture sites,  prior soft tissue trauma • Mole cular Diagnosis of STS • (i) specific genetic alterations • simple karyotypes • chromosomal translocations • point mutations • (ii) non-specific genetic alterations • complex unbalanced karyotypes. Cause unknown Risk factor Mole cular Diagnosis of STS
  • 6.
     History :painless bulging/painfull  Physical exam: 1. Size of the mass     2.Mobility 3.Superficial/deep 4.Relation to nearby NV and bony structures. 5.Regional lymph nodes. No screening tests. diagnosis Triple assment annamnesis Imaging pathology
  • 7.
     MRI isthe choice in extremities  Enhances the contrast between tumor , muscles and with blood vessels.  Follow up 3 monthly MRI are done to see recurrence  CT may be helpful in intra abdominal and few types of sarcomas  Abdominal- Spiral CT to know the relation with neuro-vascular structures  CT chest and MRI brain may be required to see metastasis  Ultrasonography if MRI is contraindicated  An X ray may help in bone involvement  Chest X ray: for low grade lesions <10 cm or intermediate or high grade<5cm. Should be perform before any invasive procedure
  • 8.
     Biopsy Techniques–  Fine-Needle Aspiration Biopsy  Core Needle Biopsy (choice)  High diagnostic accuracy,  Ease of performance  Low cost  Less complications  Incisional Biopsy  Excisional Biopsy – small cutaneous or subcut <5cm cont…. (pathologic examination)  It is recommended to obtain a diagnostic biopsy prior to definitive treatment for all soft tissue masse
  • 9.
    • T1: <=5 cm • A: superficial ( to and not invading superficial fascia) • Deep ( to or invading superficial fascia) • T2: > 5 cm • A: superficial ( to and not invading superficial fascia) • Deep ( to or invading superficial fascia) • No T3 or T4 • N1: regional LN (RARE) • M1: distant mets •Grading: •G1: will Differentiated •G2: Mederatly Differentiated •G3 Poorly Differentiated •G4: Un Differentiated T1 T2 N1 M 1 G1, GX IA IB III IV G2 IIA IIB III IV G3 IIA III III IV
  • 10.
    • Mainstay • Standardprimary treatment for most sarcomas • Problems: recurrence, incomplete resection for difficult sites
  • 11.
    • Resect thetumor with appropriate negative margins (>1 cm) • Close margins (<1 cm) may be necessary to preserve uninvolved critica l neurovascular structures, bones, joints. compartment resection is no t routinely necessary
  • 12.
    Surgical margin (SM)and residual (R) • Negative SM = R0 • Adequate: >1cm • Close: < 1cm • Adj RT is given in close margins • Positive SM = R1 or R2 • R1 resection - Microscopic residual disease • R2 resection - Gross residual disease • surgical re-resection to obtain negative margins should strongly be considered i f it will not have a significant impact upon functionality • Adj RT is given in microscopically positive margin (R1) on bone, major blood ve ssels or a nerve • Uncertain margin: • Consult radiotherapist
  • 13.
    Treatment of specialpresentation and etities
  • 14.
    • Limb sparingsurgery (LSS) is recommended to preserve function • Amputation • non-functional limb • infeasible LSS • patient preference • If adequate initial surgery cannot be done: • Preoperative chemo or radio or chemoradio • To decrease local recurrence • Chemo or radio can be used (either pre or post) • Negative SM is always desirable and may need re-resection • Adjuvant RT in: • Close SM (<1 cm; R0) • Microscopic + SM (R1) on bone or major blood vessels Surgery for Extremity STS
  • 15.
    Retroperitoneal Sarcomas • 15%of all sarcomas • Mostly types • Liposarcoma 43% • Liomyosarcoma26% • Ct scan shows in relation to surrodings • Cystic /solid /necrotic component • Biopsy not necessary unless suspect: • Lymphoma • Germ cell tumor • Plan prop chemo or radiation • En bloc resection is standrad treatment • Bowl prep • Asses bilateral kidney function • 50-80% need organ resection • 78% of primary lesions can be completely resected
  • 16.
    Retroperitonial sarcoma cont.. Chemoradiation for Retroperitonial sarcoma Radiation therapy • GI and neurotoxicities limit delivery of sufficient doses • May improve local control (clinical trails) Chemotherapy • Indication • Recurrent • Unresectable • metastatic
  • 17.
    Surgical Resection cont… RoleOf Debulking Surgery No survival benefit for incomplete resection In paitent with unresectable retroperitoneal STS.
  • 18.
    Gastero intestinal stromaltumor(GIST) Separate subtype of sarcoma defined by • expression of c-kit(CD117) • Surgery • Complete resection without local or regional lymphadenectomy • Very resistant to traditional chemotherapy • Imantinib mesylate (gleevec) • C-kit is constitutively active tyrosin kinase receptor • Initial studies showed 54% respons rates • Two RCTs currently looking at adjuvant treatment
  • 19.
    Criteria for unresectability RadiographicFindings indicate unresectability a) Extensive vascular involvement a)Aorta b)Vena cava c) Illiac vessel b) Peritonial implant c) Distant mets d) Involvement of the root of the mesentery (sup mesentery vessels) e) Spinal cord involvement Relative contraindication Interposition grafts
  • 20.
    Vascular sarcoma • Mostvascular types 1. Angiosarcoma 2. Hemangiosarcoma 3. Lymphangiosarcoma 4. Heangiopericytoma • No clear role of adjuvant chemoradiation therapy • High risk for bleeding during excision
  • 21.
    Breast srcomas • 1%of all breast neoplasm • Wide excisoin with negative margin • No clear role of adjuvant chemoradiation therapy
  • 22.
    Prognostic factors Increased riskof local recurrence 1. Age >30 2. Recurrent disease 3. Positive surgical margin Increased risk of distant metastasis I. Size >5cm II. High grade III. Deep location IV. Recurrent disease
  • 23.
    • Source: • EBRT:conventional or IMRT • Brachytherapy • Timing • Preoperative: 50 Gy • Easier surgery • Poor wound healing • Boost if close or positive SM • Postoperative • Improve local control in high-grade extremity STS with positive SM or higher stage (III), old age • May be partly given immediately (Intraoperative) and completed later
  • 24.
    Chemotherapy or chemoradiation •Preop chemoradiation: • Value: increase local control, DFS and OS • CT RT±CT Surgery ±CT • Regimens: • Doxorubicin (30 mg/m2/d x 3) concurrent with RT ( 300 cGy x 10) • IMAP x 2 RT±MAP on rest days (0, 21, 42) IOR T • MAID+RT (44 GY split) surgery MAID x 3 if S M+ • Preop chemotherapy: • Value: inconsistent • CT surgery ±CT • Regimens: • MAID
  • 25.
    Chemotherapy • Postop (adjuvant)chemotherapy: • Value: improve RFS and OS of extremity STS • EORTC trials lack OS benefit?? • surgery CT • Regimens: • Doxorubicin based (doxo-ifos) • Epirubicin based (epi-ifo)
  • 27.
    Treatment of STSof extremities and trunk G Obs erve Preop Preo Preop RT pCT CRT Surg Posto p RT Posto p CT Posto p CRT I T1 (small, <5) 1 √ may T2 (large, >5) 1 √ √ II T1 (small, <5) 2,3 M ay M ay √ √ M ay T2 (large, >5) 3 M ay M ay √ √ √ M ay III T2 (large, >5) 3 M ay M ay √ √ √ M ay N1 M ay M ay √ + Radi cal LND √ M ay IV Limited M 1 Dissemin’ d M1 May if May Sym- M AY May Post op RT if : SM <1cm, non-intact fascia l plane
  • 28.
    Treatment of STSof retroperitoneum or intra-abdominal Post op RT if : SM <1cm, non-intact fascia l plane Obs erve Preop RT Preo pCT Surg Posto p RT Posto p CT Resectable M ay M ay √ ± IORT M ay in R1 or Boost M ay Unresectable √ √ √ if becomes resectable Otherwise as M ! IV Limited M 1 Dissemin’ d M1 May if Sym- May M AY May