Soft tissue sarcomas account for less than 1% of malignant tumors but around 40% of patients die from the disease. While most soft tissue sarcomas have no clear cause, some risk factors include genetic predispositions, prior radiation therapy, and chemical exposure. A recent study found that over 50% of soft tissue sarcoma patients have a germline genetic variant contributing to tumor development. Diagnosis involves biopsy or imaging like CT or MRI. Treatment depends on location and grade, with surgery to remove the tumor with margins. High-risk cases may also receive radiation therapy or chemotherapy.

2. SOFT TISSUE SARCOMA
 Soft tissue sarcomas account for <1% of
the overall human burden of malignant
tumors but remain life
 threatening, and approximately 40% of
patients with newly diagnosed soft tissue
sarcoma die of the disease
 incidence of soft tissue sarcoma is
estimated to be between 1.4 and 5.0
cases per 100,000

3. Etiology and Risk Factors
Most soft tissue sarcomas are believed
to be sporadic and have no clearly
defined cause. In a small proportion of
cases, researchers have identified
predisposing or associated factors,
including genetic factors, lymphedema,
prior radiation therapy, and carcinogens
.

4.  In fact, a recent study of 1,162
patients presenting with soft tissue sarcoma
demonstrated that known or predicted
deleterious germline variants
may contribute to tumorigenesis in over 50% of
patients
 Desmoid tumors in adenomatous
Polyposis, germline mutations in the APC
gene
 Malignant peripheral nerve sheath tumors by
mutations in the NF1 gene.

5.  Heritable retinoblastoma gene (RB1)
mutations are associated with an increased
risk of bone and soft
tissue sarcoma. For instance, patients with
RB1 mutations have a 36% cumulative
incidence over 50 years of
P53 mutation in Li-Fraumenii Syndrom

6.  Lymphedema
 Radiation (The most common histologic
types of radiation-associated
sarcomas were pleomorphic malignant
histiocytoma (PMFH); also known as
undifferentiated pleomorphic sarcoma
[UPS]; 26%), angiosarcoma (21%),
fibrosarcoma (12%), LMS (12%), and
MPNST (9%).
Trauma
Chemical Agents

7. Anatomic and Age
Distribution
 A total of 45% are located in the extremities,
with 30% of
all lesions occurring in the lower limb (most
commonly in the thigh); 38% are intra-
abdominal, divided between
visceral (21%) and retroperitoneal (17%);
10% are truncal; and 5% are head and neck
 the median age of onset tends to be 20 to 50
years for translocation-associated sarcomas
and 50 to 70 years for complex sarcoma
types

10. Diagnosis
 Incision Bx or Core needle
 The most useful immunohistochemical
markers are the intermediate filaments (e.g.,
vimentin, keratin, desmin), leukocyte
common antigen, and S-100
 FISH is now feasible for routine diagnostic
testing for specific chromosomal
abnormalities. FISH is also useful to identify
supernumerary ring chromosomes, seen in
mesenchymal neoplasms of low or borderline
malignancy, such as DFSP

11. Imaging
 A Radiology Diagnostic Oncology Group study
comparing these modalities showed no benefit
of MRI over CT but For the
primary sarcomas in the abdomen, chest, or
retroperitoneum, a spiral CT scan is preferable
because air–tissue interface and motion artifacts
often degrade MRI quality
 Positron emission tomography (PET) has a
number of potential uses in sarcoma
management, although it has yet to gain
universal acceptance.

12. American Joint Committee on
Cancer (AJCC).
 The current (eighth edition, 2017) AJCC
TNM
(tumor-node-metastasis) system accounts
for histologic type, histologic grade, tumor
size, regional
lymph node involvement, and distant
metastasis.

13. Changes :
 it places greater emphasis on the anatomic primary site,
distinguishing sarcomas of head and neck,
extremity and trunk, visceral, and retroperitoneal locations.
 Second, for head and neck tumors, it uses smaller
primary tumor size criteria to define prognostic stage groups.
 Third, it adds a new size category,T4, for primary
tumors >15 cm for sarcomas in the extremity and trunk,
visceral, and retroperitoneal location and eliminates the
distinction of superficial versus deep
 . Fourth, it reclassifies N1 disease from stage III to stageIV.
Fifth, it provides guidance for unique histologic types.

14. Staging

15.  Unfortunately, this system still fails to
adequately account for the influence of
specific histologic types on overall survival
and the timing and pattern of local and
distant recurrence. There is as yet no
adequate staging system for retroperitoneal,
intra-abdominal, and visceral lesions.

16. Management of Extremity and Truncal Sarcoma
Surgical Management of Primary Localized Disease
A prospective, randomized trial with well over 10
years of follow-up found that although local
recurrence is greater in those undergoing
limb-sparing operation plus irradiation than in
those undergoing amputation, disease-free
survival is not different. Moreover, the level of
handicap can be significantly lower in patients
treated with limb-sparing surgery.

17.  Aiming to obtain a 1-cm margin of uninvolved
tissue in all directions. Two-centimeter margins
are employed for histologic subtypes with
infiltrative borders (e.g., DFSP or
myxofibrosarcoma).
For certain lowgrade histologic types, however,
even1-cm margins are not required for excellent
local control. For example, WDLSs of the
extremities require only complete excision
with a minimal surrounding margin because the
majority of these tumors will not recur, even after
a limited or microscopically positive margin
excision, as long as the excision is complete
.

18. Indications for Adjuvant Therapy
 As detailed in the next section, radiation
therapy should be added to limb sparing
surgery for some high-risk patients.
. Neoadjuvant chemotherapy or
investigational approaches should also be
considered for patients with high-grade
lesions >10 cm and for those with synovial
sarcoma, myxoid–round cell liposarcoma or
pleomorphic liposarcoma >5 cm
(subtypes highly responsive to chemotherapy)

21. Thank you and good luck