1. Soft Tissue Sarcomas
Dr.Arun Raj B
DNB General Surgery
MIMS

2. Introduction
 Soft tissue sarcomas are rare and unusual
neoplasms
- about 1% of adult human cancers
-15% of pediatric malignancies
 Most commonly occur in the extremities(50%)
 Also common in the abdominal cavity / retro-
peritoneum, trunk/thoracic region, and head
and neck

3. Definition
 Sarcomas are malignant tumors that arise from skeletal
and extra-skeletal connective tissue, mesenchymal cells
including:
 adipose tissue
 bone
 cartilage
 smooth muscle including vascular smooth muscle
 skeletal muscle

4. Etiology
 H/o Radiation therapy .
 Lymphedema – post surgery/ radiation/ filarial
 Chemical exposure
 Thorotrast, vinyl chloride, arsenic for hepatic
angiosarcoma
 Genetic syndromes
 Neurofibromatosis – nerve sheath tumors
 Li-fraumani syndrome
 Familial gastrointestinal stromal tumor syndrome
– KIT mutation

5. Classification
 Soft tissue and bone
 viscera (gastrointestinal, genitourinary, and gynecologic organs)
 nonvisceral soft tissues (muscle, tendon, adipose, pleura, and connective
tissue)
 By differentiation (usually with IHC staining)
 adipocytic tumors
 fibroblastic/myofibroblastic tumors
 fibrohistiocytic tumors
 smooth muscle tumors
 pericytic (perivascular) tumors
 primitive neuroectodermal tumors (PNETs)
 skeletal muscle tumors
 vascular tumors
 osseous tumors
 tumors of uncertain differentiation

6. Age as factor
 In childhood, embryonal rhabdomyosarcoma is most
common
 Synovial sarcoma is more likely to be seen in young
adults (<35 years old)
 An even distribution of liposarcoma and malignant
fibrous histiocytoma as the predominant types in the
older population

7.  Histopathology is determined by anatomic site
 Common subtypes in the extremity are liposarcoma and
malignant fibrous histiocytoma
 In the retroperitoneal location liposarcoma and
leiomyosarcoma are the most common histotypes
 In the visceral location gastrointestinal stromal tumors
are found almost exclusively

8. Histology
 The biologic behavior of sarcomas is extremely variable
 Histologic grade is a major prognostic determinant and is based on
degree of mitosis,
cellularity,
presence of necrosis, differentiation, and stromal content
 Low-grade sarcomas better-differentiated,
less cellular, tend to resemble the tissue of origin to some extent,
cytological abnormalities are less prominent,
mitotic rate is low,
Grow slower, low risk of metastasis.

9. Diagnosis
 Extremity sarcomas usually present with as a painless
mass
 However, pain is noted at presentation in up to one third of
patients
 Delay in diagnosis is common, with the most common
differential diagnosis for extremity and trunk lesions being
a hematoma or a "pulled“ muscle
 Physical examination
- should include assessment of the size of the
mass
- its relationship to neurovascular and bony
structures

10. INVESTIGATIONS
 To obtain a tissue diagnosis
 To determine the exact extent of the disease
 To evaluvate metastatic disease

11. Biopsy
 Core needle biopsy guided by palpation or by image
guidance if not palpable
 Excisional biopsy for superficial small lesions if needle
biopsy non-diagnostic
 Incision biopsy
 Longitudinal incision without tissue flaps with meticulous
hemostasis to prevent tumor seeding in hematomas AND NO
DRAIN AND SUTURING
 Send biopsy fresh and orientated

12. 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

13. Metastatic Workup
 Evaluation for sites of potential metastasis:
- Lymph node metastases occur in less than 3% of
adult soft tissue sarcoma.
- For extremity lesions, the lung is the principal site
for metastasis of high-grade lesions.
- For visceral lesions, the liver is the principal site.
 Low-grade lesions are assumed to have a low, <15%
risk of subsequent metastasis
 High-grade lesions have a high, >50% risk of
subsequent metastasis

14. Workup
 Low-grade extremity lesions require a chest
radiograph
 High-grade lesions require a chest CT
 Visceral lesions should have the liver imaged
as part of the initial abdominal CT or MRI.

15. Staging
 Staging systems focus on:
- Histologic grade of the tumor
- Size of the primary tumor
- Presence or absence of metastasis
 Staging systems:
- apply to risk of metastasis
- disease-specific survival
- overall survival
- almost exclusively confined to extremity
lesions

16. Staging
 AJCC/UICC Staging System for Soft Tissue Sarcomas
 T1: <5cm
 T1a: superficial to muscular fascia
 T1b: Deep to muscular fascia
 T2: >5cm
 T2a: superficial to muscular fascia
 T2b: Deep to muscular fascia
 N1: Regional nodal involvement
 Grading
 G1: Well-differentiated
 G2: Moderately differentiated
 G3: Poorly differentiated
 G4: Undifferentiated

17. Staging
Stage IA G1,2 T1a,b N0 M0
Stage IB G2 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
**Does not take into account extremity vs. visceral
Staging system predicts survival and risk of metastasis, but not local recurrence

18. Prognostic Factors
 Increase risk of local recurrence
– Age > 50
– Recurrent disease
– Positive surgical margins
– Fibrosarcoma
– MPNST
 Increase risk of distant metastasis
– Size > 5 cm
– High grade
– Deep location
– Recurrent disease
– Leiomyosarcoma

19. MANAGAMENT
 Concept of three dimensional clearance
 MULTIDISCIPLINARY APPROACH

20.  SURGICAL RESECTION
 ADJUVANT RT
 CHEMOTHERAPY

21. NCI Trial 1975- 1981,Rosenberg et al
43 pts
Amputation Vs Limb Salvage + RT
At 9 yrs follow up
Recurrence rate – 6 % Vs 19%
DFS - 71% Vs 63%
OS - 71% Vs 70%
NIH Alert 1985 – “LSS as Standard of care”

22. Read the cross sectional imaging
Plan your surgery preoperatively
Magnitude of Resection
Reconstruction
Rehabilitation
Revise the anatomy
Plastic / Vascular/Ortho help as required
Surgical Planning

23.  Three Dimensional clearance
 Frozen Section control
 Mark the bed for adjuvant RT
 Orient the specimen
 Gross the specimen
Surgical Principles

24. Gross Pathology
 Centripetal growth
 Pseudocapsule
Compressed tumor cells
Fibrovascular zone (reactive inflammatory cells)

26. QUALITY OF MARGINS
Barrier – Tissue that has resistance to tumor invasion
Thin Barrier
Membranous muscle fascia
Adult periosteum
Epineurium
Vascular sheath
Thin growth plate
Thick Barrier
Iliotibial band
Sacral fascia
Joint capsule
Periosteum of infant /
young child
Thick growth plate

27. Thin Barrier – 2 cm
Thick Barrier – 3 cm
Growth Plate – 5 cm
Normal Tissue Equivalent
Aim – Normal tissue outside the barrier

28. CURATIVE RESECTION MARGINS
Barrier +ve - Outside the barrier
Barrier –ve – > 5 cm margin

30. Adjuvant Therapy
Radiotherapy
Brachy / EBRT
Chemotherapy

31. Adjuvant radiotherapy
 Small, low grade tumors < 5cm resected with 2 cm
margins may not require radiation
 Adjuvant radiation should be added to the surgical
resection:
- If the excision margin is close
- If extra muscular involvement is present
- If a local recurrence would result in the
sacrifice of a major neurovascular bundle or
amputation
 Improves local control but not survival

32. Radiotherapy
 Can be given as brachy-therapy or EBRT or intra-
operative RT
 Brachytherapy for high grade lesions
 External-beam radiation therapy for large (>5 cm)
high- or low-grade lesions
 Intra-operative RT given in cases of retro-peritoneal
sarcomas.
 Can be given as pre-operative/ post-operative RT.
 Pre-operative preferred in head and neck
malignancy/ rest post-operative RT

33. 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
 Hyperthermic isolated limb perfusion may be
used for palliation

34. Metastatic disease
 Lung most common site of mets, but visceral
often go to liver
 Median survival from development of
metastatic disease is 8-12 months
 Resection of pulmonary mets can give 5 year
survival of 32% if all mets can be removed
 >3 mets is poor prognosticator

35.  Surgeon is an important prognostic factor
 Good preop planning (no ontable surprises)
 Resect , Reconstruct , Restore

36. Thank youThank you