Soft tissue sarcoma is a rare and heterogeneous group of tumors that arise from mesenchymal tissues. It accounts for less than 1% of all cancers. The extremities are the most common primary site. Complete surgical excision with negative margins is the standard treatment, but adjuvant radiation and/or chemotherapy may be used depending on factors like tumor size, grade, and margins. Prognosis depends on tumor size, grade, depth, and whether there is local or distant spread.
2. Introduction
Rare and heterogeneous
group of tumors
Mesenchymal origin
<1% of all cancers
Dozens of subtypes
Classified as per the
purported cell of origin or
resemblance
Vodanovich DA, Choong PF. Soft-tissue sarcomas. Indian journal of orthopaedics. 2018 Feb;52:35-44.
3. STS predominate over bone sarcoma
4:1
Male preponderance for the incidence
1.4:1
Extremities are the most common site (40%)
Lower limb being more common (28%)
44% in thigh
Vodanovich DA, Choong PF. Soft-tissue sarcomas. Indian journal of orthopaedics. 2018 Feb;52:35-44.
6. History
Parameter Benign Malignant Comments
Pain Rare Rare Uncommon in the
absence of nerve
or bone invasion.
Size <5cm >5cm
Location Superficial Deep to fascia 1/3 of STS are
superficial
Mobility Mobile Fixed
Consistency Soft Firm
Growth Stable Enlarging Synovial sarcoma
and clear cell
sarcoma grown
very slow
Intramuscular hemangioma presents with pain.
Benign peripheral nerve sheath tumors are tethered to the never so medial
lateral mobility present
7. Age wise
In childhood
Embryonal rhabdomyosarcoma is the most common
Young adults
Synovial sarcoma (<35 years)
Old age
Evenly distributed
Liposarcoma
Undifferentiated pleomorphic sarcoma
11. CT
CT chest for staging
Bony involvement
Contraindication of MRI
Pattern of mineralization
12. MRI
Imaging modality of choice in the diagnosis and therapeutic
planning
To determine size, site, anatomic relationship to
neurovascular bundle
Preoperative radiation or chemotherapy responses and for
detecting local recurrence
Most tumor demonstrate
Dark signal on T1
Bright signal on T2
Donot follow this usual signaling pattern have a narrow
differential
High T1 lipoma, low T1, low T2 fibromatosis
13. CT vs MRI – Cortical involvemet is seen better in CT
14. A typical MRI appearance of a soft tissue sarcoma in the arm
17. Helpful hints
Any deep heterogeneous mass or mass >5cm regardless
of location is a soft tissue sarcoma unless proven
otherwise
Cysts do not enhance centrally with gadolinium
True lipomas are homogenous on all sequences
Well differentiated to moderately differentiated
liposarcomas have areas of signal consistency with
normal fat but admixed with non-fatty signal
18. Biopsy
Essential part of both
diagnosis and
management
Core needle biopsy or
open biopsy
Fine needle biopsy is not
sufficent to provide
information
19. Whoops
Unplanned biopsy or
excision lead to
Increased mortality
Increased complex
surgery
Increased local
recurrence
Increased metastatic
disease
Increased amputation
Vodanovich DA, Choong PF. Soft-tissue sarcomas. Indian journal of orthopaedics. 2018 Feb;52:35-44.
20. Histology
Histological grade is a major prognostic determinant
Based upon
Degree of mitosis
Cellularity
Presence of necrosis
Differentiation
Stromal content
22. American joint committee on
cancer (AJCC) staging
Stage Size Depth Grade Metastases
I Any Any Low No
II <5cm, any
depth or
>5cm
superficial
High No
III >5cm Deep High No
IV Any Any Any yes
23.
24. Surgical procedure based on
Tumor location
Tumor size
Involvement of adjacent
anatomical structures
Patient preference
Response to neo-adjuvant
therapies
25. Surgery
Primary treatment
modality
Aim is to excise the
whole tumor with tumor
negative margins
26. Prognostic Factors
Increased risk of local recurrence
Age>50 years
Recurrent disease
Positive surgical margins
fibrosarcoma
Increased risk of distant metastasis
Size >5cms
High grade
Deep locations
Recurrent disease
Leiomyosarcoma
27. Metastatic potential of
histological subtypes
Low Intermediate High
Well differentiated
liposarcoma
Inflammatory myofibroblastic tumor Undifferentiated pleomorphic
sarcoma
Haemangipericytoma Dedifferentiated liposarcoma
Haemangioendothelioma Leiomyosarcoma
Solitary fibrous tumor Round cell liposarcoma
angiosarcoma
Synovial sarcoma
Rhabdomyosarcoma
Extraskeletal Ewings sarcoma
Epitheloid sarcoma
Alveolar soft part sarcoma
Malignant peripheral Nerve
Sheath tumor
Roland CL. Soft Tissue Tumors of the Extremity. Surgical Clinics. 2020 Jun 1;100(3):669-80.
28. Lymph Node spread
An infrequent event
Of total 10,731 2.1% had LN metastatis
Poorer survival rate with LN spread
Several variables
Histologic subtypes
Tumor grade
Patient age
Primary tumor size
1. Fong Y, Coit DG, Woodruff JM, Brennan MF. Lymph node metastasis from soft tissue sarcoma in adults. Analysis of data from a
prospective database of 1772 sarcoma patients. Annals of surgery. 1993 Jan;217(1):72.
2. Miccio JA, Jairam V, Gao S, Augustyn A, Oladeru OT, Onderdonk BE, Chowdhary M, Han D, Khan S, Friedlaender G, Lindskog DM.
Predictors of Lymph Node Involvement by Soft Tissue Sarcoma of the Trunk and Extremity: An Analysis of the National Cancer Database.
Cureus. 2019 Oct;11(10).
29. Histological subtypes with increased Lymph node
metastases
Rhabdomyosarcoma
Synovial sarcoma
Undifferentiated pleomorphic sarcoma
Epitheloid sarcoma
Angiosarcoma
Clear cell sarcoma
Neurofibrosarcoma
Leiomyosarcoma
fibrosarcoma
Nodal disease has prognostic value
Regional lymphadenectomy to improve survival rate
Miccio JA, Jairam V, Gao S, Augustyn A, Oladeru OT, Onderdonk BE, Chowdhary M, Han D, Khan S, Friedlaender G, Lindskog DM.
Predictors of Lymph Node Involvement by Soft Tissue Sarcoma of the Trunk and Extremity: An Analysis of the National Cancer Database.
Cureus. 2019 Oct;11(10).
30. Is resection enough?
Half of patients with adequate local control of sarcoma
develop distant mets and die from disease
Low cure rate
Metastatic STS are rarely cured
Systemic chemotherapy is the standard care in
pediatric sarcomas
Chemotherapy in adult is controversial
Whether or not there are small population of patients
truly benefit from adjuvant chemotherapy
Bajpai J, Susan D. Adjuvant chemotherapy in soft tissue sarcomas…Conflicts, consensus, and controversies. South Asian J Cancer.
2016;5(1):15-19. doi:10.4103/2278-330X.179687
31. Radiotherapy (RT)
Small, low grade tumors <5 cms with 2 cm negative
margins may not require
Should be added to
If margin is close
If extra muscular involvement is present
If local recurrence would result neurovascular
involvement or amputation
Improves local control but not survival
Tiong SS, Dickie C, Haas RL, O’Sullivan B. The role of radiotherapy in the management of localized soft tissue sarcomas. Cancer biology &
medicine. 2016 Sep;13(3):373.
32. Role of RT
Downsizing prior to surgery
Local control if surgery is impossible
Due to medical comorbidities, surgery is
contraindicated
Burden of metastatic disase
Care must be taken to appropriately design fields to
exclude sensitive structures.
Tiong SS, Dickie C, Haas RL, O’Sullivan B. The role of radiotherapy in the management of localized soft tissue sarcomas. Cancer biology &
medicine. 2016 Sep;13(3):373.
33. Chemotherapy
Cannot be used as a blanket therapy
Can be used in size >5cm, high grade STS
Chemo-sensitive STS
Synovial sarcoma
Leiomyosarcoma
Angiosarcoma
34. Role of chemotherapy
Downsizing
Increase the rate of limb salvage surgery
Acts as a surrogate marker of chemosensitivity of
disease
Palliative care for metastatic STS
Uncertain due to the limited randomized controlled trial
35. controversies
Can radiotherapy be avoided?
The appropriated time of radiotherapy in relation to
surgery
The appropriate time of chemotherapy in relation to
surgery
36. Take home message
Any lump >5cm should be treated as soft tissue
sarcoma unless proven otherwise
Biopsy is the most to confirm the diagnosis
Surgical resection is the standard treament modality
with negative surgical margins
Adjuvant chemotherapy or radiotherapy can be given
for local control