This document discusses soft tissue sarcomas. It begins by listing various histologic diagnoses of soft tissue sarcomas categorized by tissue of origin. It then discusses imaging with MRI and CT to evaluate tumors. Key points regarding surgery include performing limb-sparing over amputation when possible and obtaining negative margins of at least 2cm if no post-op radiation is planned. Adjuvant radiation improves local control but not necessarily survival. Pre-op radiation may increase wound complications compared to post-op.

1. Dr. Kanhu Charan Patro
M.D,D.N.B[RT],P.D.C.R,C.E.P.C
[EX – TATA MEMORIAL HOSPITAL]
Consultant- Radiation Oncology
MAHATMA GANDHI CANCER HOSPITAL
VISAKHAPATNAM
Email-drkcpatro@gmail.com ,M-09160470564

2. No one denies the importance of evidence.
It is a sine qua non of professional practice. But
often, there are no simple answers to
apparently simple questions: there is a role for
judgement and decision-making

3. Levels of evidence
Level-I=large double blind RCTs, or, metaanalysis of
smaller RCTs , with clinically relevant outcomes
I a=evidence from meta-analysis of RCT
I b=evidence from at least 1 RCT
Level-II=small RCTs, non-blinded RCTs
IIa=evidence from one well designed non-RCT
IIb=evidence from one well-designed quasi-
experimental study
Level-III=observational [cohort ] studies ,case-
control studies , non-RCTs
Level-IV=opinion of expert committees, or
respected authorities
Level -V=expert opinion 3

4. 4

5. 5

6. Wilms' Tumor
6%
0%
Other
7%
Leukemia
31%
Bone
5%
Eye
3%
Central
Nervous
System
18%
Germ Cell
3%
Neuroblastoma
7%
Soft Tissue
6%
Lymphoma
14%

7. Radiotherapy
X ray
Surgery
PET
MRI
Chemo
? ?
7

11. Oncologist Diagnosis
Treatment
Radiologist
Cytopathologist
Surgeon
Histopathologist
Molecular
Pathologist
Geneticist
psychiatrist
Nursing
And
Support staff
Audit

12. GOALS
High dose to tumor tissue-Tumor control
Normal tissue sparing
Minimize long and short term toxicities
Better Quality of life
10/20/12 01:12 PM 12

13. Evolution of Treatment Techniques
CONVENTIONAL RT
Collimator shapes Beam
Rectangular Treatment Field
Shaped Treatment Field
1970s and earlier
10/20/12 01:12 PM 13

14. MULTI LEAF COLLIMATOR

15. 10/20/12 01:12 PM 15

16. Radiation Chamber
ECRT

17. History
Imaging
Biopsy
Bone marrow in ewings
Immunohistochemistry-IHC study
Chromosomal study
10/20/12

18. Biopsy should be regarded as the
final diagnostic procedure, and not
a shortcut to diagnosis.
History
Imaging
Investgtn.

19. Biopsy should be regarded as the
final diagnostic procedure, and not
a shortcut to diagnosis.
“The gross anatomy, as evidenced by X-rays is a
safer guide to a correct clinical conception of the
disease than the variable and uncertain structure of
the small piece of tissue sent for microscopy”
Dr. James Ewing -1922
History
Imaging
Investgtn.

20. The biopsy should be performed by the
surgeon who will be doing the definitive
surgery

21. BIOPSY
OPEN v/s NEEDLE

22. More representative tissue
Higher accuracy of diagnosis
Correct diagnosis even with an average pathologist
Open Biopsy

23. Open Biopsy
Traumatic
Tissue contamination
Skin loss during final surgery
Hematoma, Infection
Fractures
GA required
More OT Time
Higher costs

24. Needle Biopsy
OPD procedure, LA
Can be image guided or CT guided
Practically no infection
Very little tissue contamination
Much smaller risk of causing fracture
Cores from depth of the tumor
Minimally invasive, much less traumatic

25. May not yield adequate tissue
May be difficult to make a diagnosis
Experienced pathologist required to
be able to give a diagnosis on small
quantity of tissue
Needle Biopsy

27. TREATMENT SEQUENCE

28. Typical treatment
pathways in
osteosarcoma and
Ewing’s sarcoma,
illustrating their
similarities and
differences from
diagnosis to
follow-up
PAEDIATRICS AND CHILD HEALTH 20:3

29. 29

30. 06/25/18 04:02 PM 30

31. LOTS OF BLOOD REQUIRE
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32. TABLET FORMS
06/25/18 04:02 PM 32

33. Information helps decide 
Post op radiotherapy
Radiotherapy indicated for patients with
positive margins and considered for those with
residual viable tumor
Radiation dose adjusted depending upon the
percentage necrosis of tumor and margins of
resection

34. All specialities under one roof
06/25/18 04:02 PM 34

35. 10/20/12 35
Osteosarcoma Skeletal
distribution
Most common sites are the distal FEMUR,
the proximal TIBIA, and the proximal
HUMERUS.

36. 10/20/12 36
Osteosarcoma lesions can be purely osteolytic
(30%), purely osteoblastic (45%), or a mixture of
both. (Kesselring 1982).
Lytic Sclerotic Mixed

37. 10/20/12 37
Codman triangle Sun burst

38. 10/20/12 38
CT scanning
CT scanning of the chest is more sensitive than is
plain film radiography for assessing pulmonary
metastases.
MRI
MRI of the primary lesion is the best method to assess
the extent of intramedullary disease as well as
associated soft-tissue masses and skip lesions. (Estrada
1995)
Bone Scan
A bone scan should be obtained to look for skeletal
metastases or multi focal disease.

39. 10/20/12 39
Treatment
Current standard of care
1. Radiological staging
2. Biopsy to confirm diagnosis
3. Preoperative chemotherapy
4. Repeat radiological staging
(access chemo response, finalize surgical tx plan)
5. Surgical resection with wide margin
6. Reconstruction using one of many
techniques
7. Post op chemo based on preop response

40. Tumor necrosis
10/20/12

41. 10/20/12 41
Chemotherapy
Before the era chemotherapy osteosarcoma was usually
treated with immediate wide or radical amputation on
diagnosis.
This usually treated the local disease adequately. However
80% of patients eventually died of micrometastatic
disease.
With the use of modern chemotherapy protocols, the
current 5-year survival rate for osteosarcoma is
approximately 70%.

42. 10/20/12 42
Surgery
The main goal of surgery is to safely and completely
remove the tumor.
Historically, most patients had an amputation. Over the
past 30 years, limb-sparing procedures have become the
standard, mainly due to advances in chemotherapy and
sophisticated imaging techniques (Scully 2002).
Limb salvage procedures now can provide rates of local
control and long-term survival equal to amputation.

43. 10/20/12 43
Metallic Prosthesis

44. 10/20/12 44
Radiotherapy
Radiation therapy has no major role in osteosarcoma
Radiation therapy may be useful in some cases where the
tumor cannot be completely removed by surgery. E.g. in
pelvic bones or in the bones of the face. In these
situations, As much tumor as possible is removed, and
then radiation is given to try to kill the remaining cancer
cells. Chemotherapy may be used after radiation.
Radiation can also be helpful in controlling symptoms like
pain and swelling if the cancer has come back or surgery is
not possible.

45. Ewings’ sarcoma
 ESFTs 2nd
decade of life
 Accounts for 4% of
childhood and
adolescent malignancies
 One third of primary
bone tumours
 Origin: Primordial neural
stem cell
 Slight male
preponderance
 “small round blue cell
tumors of childhood”
EFT
ETB 60% EOE PNET

47. Imaging
X-ray –Lytic
Sclerotic
Usually diaphysis
 Periosteal reaction-
onion peel .
Soft tissue mass
Sclerosis secondary to
bone reaction[not
tumor bone formation-
osteosarcoma]

48. Bx –preferably from soft tissue mass and
not from bone. If bone Bx reqd ,small
cortical window made.
Tissue for IHC ,RT-PCR.
B/L iliac for BMA and Bx.
IHC –Mic 2 [CD 99], membrane
expression,vimentin, NSE and
synaptophysin
 Neural markers - neuron-specific
enolase, Leu-7, synaptophysin,
neurofilament, and S100.

49. Management
The primary goal of treatment is local control of the
disease while, if possible, achieving salvage of the
limb and its function
Limb-salvage procedures a valid alternative method
of treatment to amputation in 80-85% of patients
with primary bone sarcomas.
Wafa H et alExpert Rev Anticancer Ther. 2006 Feb;6(2):239-48.

50. Why anterior chemotherapy
Before the era of chemotherapy, fewer
than 10% of patients with Ewing’s
sarcoma survived despite
radiosensitivity
Why systemic therapy?
Mortality within 2 yrs with distant
metastasis
Jenkin RD, Clin Radiol1966;17:97–106

51. Rx Flow ChartInduction Chemotherapy
Sx Feasible
Sx Not Feasible
CT not Effective
W/E not possible
Amputation
Definitive RTPORT
Maintenance CT

52. Information helps decide 
Post op radiotherapy
Radiotherapy indicated for patients with
positive margins and considered for those with
residual viable tumor
Radiation dose adjusted depending upon the
percentage necrosis of tumor and margins of
resection

53. Is surgery the best modality for local control at all sites ?
?

54.  Ewing’s pelvis L
 8 year old girl
 Involves ilium + upper acetabulum

55. Responded to chemotherapy

56. Responded to chemotherapy
Resection will lead to
extensive morbidity

57. METASTATECTOMY –LUNG
OLIGO METS
10/20/12

58. Soft Tissue Sarcomas
October 20, 2012

59. 10/20/12
Histologic DiagnosesHistologic Diagnoses
•Malignant Fibrous Histiocytoma (MFH)Malignant Fibrous Histiocytoma (MFH)
•Low-grade Fibromyxoid SarcomaLow-grade Fibromyxoid Sarcoma
•(Fibrosarcoma)(Fibrosarcoma)
Sarcomas of Fibrous TissueSarcomas of Fibrous Tissue
Sarcomas of Skeletal MuscleSarcomas of Skeletal Muscle
•Embryonal RhabdomyosarcomaEmbryonal Rhabdomyosarcoma
•Alveolar RhabdomyosarcomaAlveolar Rhabdomyosarcoma
•(Pleomorphic Rhabdomyosarcoma)(Pleomorphic Rhabdomyosarcoma)
•LeiomyosarcomaLeiomyosarcoma
•GIGI
•GUGU
•SkinSkin
•VesselVessel
•OtherOther
Sarcomas of Smooth MuscleSarcomas of Smooth Muscle
Sarcomas of Adipose TissueSarcomas of Adipose Tissue
•LiposarcomaLiposarcoma
•Atypical Lipomatous TumorAtypical Lipomatous Tumor
•Myxoid LiposarcomaMyxoid Liposarcoma
•Cellular Myxoid LiposarcomaCellular Myxoid Liposarcoma
•Dedifferentiated LiposarcomaDedifferentiated Liposarcoma
•Pleomorphic LiposarcomaPleomorphic Liposarcoma
Sarcomas of Blood and Lymph VesselsSarcomas of Blood and Lymph Vessels
•AngiosarcomaAngiosarcoma
•HemangiosarcomaHemangiosarcoma
•LymphangiosarcomaLymphangiosarcoma
•Epithelioid HemangioendotheliomaEpithelioid Hemangioendothelioma
•HemangiopericytomaHemangiopericytoma
•Kaposi’s SarcomaKaposi’s Sarcoma
Sarcomas of Peripheral Nervous TissueSarcomas of Peripheral Nervous Tissue
•Malignant Peripheral Nerve Sheath TumorMalignant Peripheral Nerve Sheath Tumor
•AKAAKA
•Malignant SchwannomaMalignant Schwannoma
•NeurofibrosarcomaNeurofibrosarcoma
•Neurogenic SarcomaNeurogenic Sarcoma
Sarcomas of Unknown TissueSarcomas of Unknown Tissue
•Synovial SarcomaSynovial Sarcoma
•MonophasicMonophasic
•BiphasicBiphasic
•Alveolar Soft Part SarcomaAlveolar Soft Part Sarcoma
•Epithelioid SarcomaEpithelioid Sarcoma
•Unclassified SarcomaUnclassified Sarcoma
Extraskeletal Sarcomas of BoneExtraskeletal Sarcomas of Bone
•Extraskeletal OsteosarcomaExtraskeletal Osteosarcoma
•Extraskeletal ChondrosarcomaExtraskeletal Chondrosarcoma
•Extraskeletal Ewing’s Sarcoma (PNET)Extraskeletal Ewing’s Sarcoma (PNET)
Soft-tissue Tumors of Melanocytic TissueSoft-tissue Tumors of Melanocytic Tissue
•Melanoma of Soft PartsMelanoma of Soft Parts
•AKA - Clear Cell SarcomaAKA - Clear Cell Sarcoma
•Dermatofibrosarcoma ProtruberansDermatofibrosarcoma Protruberans
•Desmoid FibromatosisDesmoid Fibromatosis
•Nodular FasciitisNodular Fasciitis
SOFT-TISSUE SARCOMASOFT-TISSUE SARCOMA

60. Introduction
Rare: only 8300 new cases annually in U.S.
3900 die annually from STS
Mesodermal origin

61. Cytokeratin
 Vimentin
 Smooth muscle actin
 Desmin
 S-100
 CD 31
 CD 34
Immunohistochemistry

63. Tumor seeding after biopsy

64. Imaging
MRI
 For extremity masses
 Gives good delineation between muscle, tumor and
blood vessels
CT for abdominal and retroperitoneal
PET
 May help determine high vs. low grade
 May be helpful in recurrences

65. Survival by stage

66. Relative risk for recurrence and survival
Age >50 years 1.6
Local recurrence at presentation 2.0
Microscopically positive margin 1.8
Size 5.0–10.0 cm 1.9
Size > 10.0 cm 1.5
High-grade 4.3
Deep location 2.5
Local recurrence 1.5

67. Surgery
Limb-sparing vs amputation
 Comparison study with post-op radiation in limb sparing
showed no difference in survival
Amputation still may be indicated for
neurovascular or bone involvement

68. Resection
Arbitrary 2 cm margin if no plan for post-op
radiotherapy
Negative margins may be adequate for post-op
radiation therapy
 Presence of positive margins increases local recurrence
by 10-15%
No need for lymph node dissection as only 2-3%
have nodal metastasis

69. Adjuvant radiotherapy
Small, low grade tumors resected with 2 cm
margins may not require radiation
Improves local control but not survival
Whether improved local control leads to
improved survival is controversial

70. Pre-op or post-op radiation?
Some avoid pre-op use because of increased
wound complications (although this is debatable)
 RCT looking at wound complication rate pre-op vs post-
op radiation showed 35% vs 17%
 Risk confined to lower extremity
 Conclusions: pre-op may be better for upper extremity
and head & neck because of equal wound complication
risk and benefit of lower radiation doses to more vital
tissues

71. Pre-op vs post-op
radiotherapy

72. Chemotherapy
Can improve local control, but not survival
Doxorubicin and iFosfamide have response rates
of 20%
Use only in advanced disease
Combination with radiation or neoadjuvant
therapy are controversial
Hypothermic isolated limb perfusion may be used
for palliation

73. Treatment of Recurrence
20-30% of STS patients will recur
More common in retroperitoneal and head &
neck high grade tumors because hard to get clear
margins
 38% for retroperitoneal
 42% for head and neck
 5-25% for extremity
After re-resection recurrence is 32% for extremity
and much higher for visceral

74. 64 y/o male with increasing abdominal girth

75. Retroperitoneal Sarcomas
15% of all sarcomas
Liposarcoma 42% and leiomyosarcoma 26%
CT scan can show cystic/solid/necrotic components and relation
to surroundings
CXR to r/o mets, chest CT if CXR abnormal
Biopsy not necessary unless suspect a lymphoma or germ cell
tumor or plan preop chemo or radiation
En bloc resection is standard treatment
 bowel prep
 assess bilateral kidney function
 50-80% need organ resection
 78% of primary lesions can be completely resected

76. Liposarcoma

77. Prognosis for retroperitoneal sarcomas
5 year survival after complete resection of 54-65%
 Drops to 10-36% if incompletely resected
Recurrence occurs in 46-59% of completely
resected tumors

78. Radiation or chemotherapy for retroperitoneal
sarcomas
Radiation
GI and neuro toxicities limit delivery of sufficient
doses
May improve local control
Recommended for use only in clinical trials given
lack of data either way
Chemotherapy
Use for recurrent, unresectable or metastatic
disease

79. 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
Gleevec (imantinib mesylate)
 c-Kit is constitutively active tyrosine kinase receptor
 Drug is tyrosine kinase inhibitor used in CML
 Initial studies showed 54% response rates
 Two RCTs currently looking at adjuvant treatment

80. GIST

81. Extremity sarcomas
MFH
Synovial sarcoma

82. Breast sarcomas
1% of all breast neoplasms
Wide excision with negative margins
No clear role for adjuvant radiotherapy

83. Sarcoma after mastectomy

84. SOFT-TISSUE SARCOMA BRACHYTHERAPY

85. ROUND CELL TUMOR
1.SMALL CELL OSTEOSARCOMA
2.PNET
3.SYNOVIAL SARCOMA
4.LYMPHOMA
5.EWING’S FAMILY OF TUMOR

86. IHC
1. CD99
2. LCA/CD45
3. NEUROFILAMENT
4. VIMENTIN

88. 88

89. OSTEOARTHRITS
89

90. BURSITIS
90

91. 91

92. ECTOPIC BONE FORMATION
92

93. HYPEROSTOSIS
10/20/12

94. HIP ARTHROPLASTY
10/20/12

95. VERTEBRAL HEMANGIOMA
95

96. ANERYSMAL BONE CYST
96

97. AMELOBLASTOMA
97

98. Sharp gun-missed target
98

100. METASTASIS
-please do not watch crying
100

101. 10/20/12 01:12 PM 101

102. METASTASIS- give a smiling
death
102

103. Palliative radiation
Skeletal X-Ray
Bone scan
MRI
PET-CT

104. Spinal metastasis
104

105. 105

106. Brain metastasis
106

107. 107

108. Whole brain radiotherapy
108

109. Choroidal metastasis
109

110. Superscan-extensive bone mets
110

111. Hemibody radiation
111

112. Prophylactic radiation
112

113. svco
113

114. CAUTION
C - Change in bowel or bladder habits
A - A sore that does not heal
U - Unusual bleeding or discharge
T - Thickening or lump in the breast or any part of the
body
I - Indigestion or difficulty swallowing
O - Obvious change in a wart or mole
N - Nagging cough or hoarseness

115. Change in bowel habits
10/20/12 01:12 PM 115

116. Change in bladder habits
10/20/12 01:12 PM 116

117. A sore that does not heal
10/20/12 01:12 PM 117

118. Unusual bleeding or discharge
10/20/12 01:12 PM 118

119. Thickening or lump in the
breast or any part of the body
10/20/12 01:12 PM 119

120. Indigestion or difficulty
swallowing
10/20/12 01:12 PM 120

121. Obvious change in a wart or
mole
10/20/12 01:12 PM 121

122. Nagging cough or hoarseness
10/20/12 01:12 PM 122

127. Prevention-passive smoking

128. Liver cancer-
hepatitis B vaccine

129. Cervix cancer vaccine
10/20/12 01:12 PM 129

130. Promise-Stop drinking alcohol
10/20/12 01:12 PM 130

131. RELAX

132. Meditation
10/20/12 01:12 PM 132

133. 133
Dietary protective factorsDietary protective factors

134. Breast feeding

135. REGULAR CHECK-UP

136. 10/20/12 01:12 PM 136

138. 138

139. Physical activity

140. 140

141. 141

142. 10/20/12 01:12 PM 142

143. 10/20/12 01:12 PM 143

144. Just “Doing It” is not good enough !
You must know “what” to do and “where” to do it !
10/20/12 01:12 PM 144

145. 10/20/12 01:12 PM 145

146. 10/20/12 01:12 PM 146

147. 10/20/12 01:12 PM 147
Whenever not
possible,
make friends

148. 14810/20/12 01:12 PM

149. 14910/20/12 01:12 PM

150. 150
?

151. 151