Soft Tissue Sarcomas

DARWITO SUB BAG BEDAH ONKOLOGI

FK UNDIP-RSDK
 Sarcomas from the embryonic mesoderm.
most commonly as an asymptomatic mass
originating :
in an extremity
can occur anywhere in the body, particularly the
trunk, retroperitoneum, or the head and neck.
 Pretreatment radiologic imaging is critical
for defining the local extent of a tumor,
staging the disease, guiding biopsies, and
aiding in diagnosis.
Core-needle biopsy is the preferred
biopsy technique for diagnosing soft tissue
sarcomas.
 (AJCC) staging system for soft tissue
sarcomas is based on :
histologic grade,
the tumor size and depth,
and the presence of distant or nodal metastases.
Despite improvements in local control
rates with wide local resections and
radiation therapy
 metastasis and death remain a significant
problem in 50% of patients who present
with high-risk soft tissue sarcomas.
The most common site of metastasis is
the:
lungs, and metastasis generally occurs within two
to three years after the completion of therapy.
Incidence

In 2004, approximately 8,680 new cases

are expected to be diagnosed in the
United States,
and 3,660 deaths from soft tissue
sarcomas are predicted, accounting for
0.63% of all cases and 1.15% of deaths
from cancer.
 Soft tissue sarcomas can occur anywhere
in the body, but most originate in:
extremity (59%),
the trunk (19%),
the retroperitoneum (15%),
or the head and neck (9%).
 Currently, more than 50 histologic types of soft tissue
sarcoma have been identified (Table 1),
but the most common are malignant fibrous
histiocytoma (28%),
leiomyosarcoma (12%),
liposarcoma (15%),
synovial sarcoma (10%),
and malignant peripheral nerve sheath tumors (6%).3
Rhabdomyosarcoma is the most common soft tissue
sarcoma of childhood.
 Epidemiology
The dominant pattern of metastasis is
hematogenous.
Lymph node metastasis is rare (less than
5%),
except for a few histologic subtypes such as
epithelioid sarcoma, synovial sarcoma,
rhabdomyosarcoma, clear-cell sarcoma, and
angiosarcoma.
 External radiation therapy is a well-established
risk factor for soft tissue sarcoma

Other risk factors include occupational exposure

to certain chemicals, :
including herbicides such as phenoxyacetic acids and
wood preservatives containing chlorophenols.
 Chronic lymphedema following axillary
dissection is another risk factor;
the subsequent lymphangiosarcoma is
known as Stewart-Treves syndrome.
Genetics

The oncogenes (ie, genes that can

induce malignant transformation and tend
to drive cells toward proliferation) that
have been implicated in the development
of soft tissue sarcomas
include MDM2, N-myc, c-erbB2, and
members of the ras family.
 The best characterized gene
rearrangements have been found in
Ewings sarcoma (EWSFLI-1 fusion),
clear-cell sarcoma (EWSATF1 fusion),
myxoid liposarcoma (TLSCHOP fusion),
alveolar rhabdomyosarcoma (PAX3FHKR fusion),
desmoplastic small round-cell tumor (EWSWT1
fusion),
and synovial sarcoma (SSXSYT fusion).
 Tumor suppressor genes play a critical role
in cell growth inhibition and can suppress
the growth of cancer cells.
However, these genes can be inactivated
by hereditary or sporadic mechanisms.
 Two such genes that are particularly
relevant to soft tissue tumors are the
1.retinoblastoma (Rb)gene
2.and the p53 tumor suppressor
gene.
 Mutations or deletions in the
Rb gene can lead to the development of
retinoblastomas and sarcomas of the soft
tissue and bone.
 mutations in the p53 tumor suppressor
gene are the most common mutations in
human solid tumors,
They have also been observed in 30% to
60% of soft tissue sarcomas.
Clinical Presentation

The size at presentation usually depends

on the location of the tumor.
Tumors in the distal extremities are often
small when discovered, whereas tumors
in the proximal extremities and
retroperitoneum can become quite large
before they are apparent.
 Soft tissue sarcomas grow in a centrifugal
fashion and compress surrounding normal
structures, but rarely does impingement
on
bone or neurovascular bundles produce
pain,edema, and swelling.
 The differential diagnosis of a soft tissue
mass includes benign lesions, including
lipomas, lymphangiomas, leiomyomas,
and neuromas.
Besides sarcomas, other malignant
lesions, such as primary or metastatic
carcinoma, melanoma, or lymphoma, must
also be considered. structures, and tumor.
 Biopsy should be considered in patients
with all other types of tumors to establish
a definitive diagnosis.
Biopsy Techniques:
1.Fine-needle Aspiration Biopsy
2.Core-needle Biopsy
3.Incisional Biopsy
4.Excisional Biopsy
1.Fine-needle Aspiration Biopsy

Fine-needle aspiration biopsy is an

acceptable method for the diagnosis of
most soft tissue sarcomas, particularly
when it is performed in conjunction with
clinical and imaging studies.
 Fine-needle aspiration biopsy is the
procedure of choice to confirm or rule out
a metastatic focus or local recurrence.
If tumor grading is essential for treatment
planning, fine-needle aspiration biopsy
has limitations.
The diagnostic accuracy of fine-needle
aspiration biopsy-based findings in
patients with primary tumors ranges from
60% to 96%.
 Fine Needle Aspiration (FNA): A
procedure in which a thin needle is
inserted to remove, for microscopic
evaluation, fluid from a cyst or cells from a
tumor.
 FNA
2123 gauge needle
Sensitivity: 68100%
Biopsy Options

Specificity: 82100%
Guidance
Free hand
Stereotactic
Ultrasound
Jackson VP, Bassett, LW
Stereotactic fine-needle aspiration biopsy for
nonpalpable breast lesions.
AJR AM J Roentgenol 1990; 154:1196-97
2.Core-needle Biopsy
Core-needle biopsy is a safe, accurate, and
economical procedure for diagnosing soft tissue
sarcomas.
In addition, enough tissue is usually
obtained for use in several diagnostic tests, such
as electron microscopy, cytogenetic analysis,
and flow cytometry.
 Core Needle Biopsy: (Also known as
Core-Cutting Needle Biopsy; see also
Needle Biopsy) Removal of breast
tissue samples using a large-diameter
needle. The samples are examined by a
pathologist who determines whether they
are benign or malignant.
 Core Needle Biopsy
Can be performed in an
outpatient setting or
doctors office
Biopsy Options

Local anesthesia
No sutures
4 6 needle insertions to
collect a sufficient amount
of breast tissue for an
accurate diagnosis
Unable to mark biopsy
site
 Complications occur in less than 1% of
patients who undergo core needle biopsy.
The use of CT or sonography to guide a
core-needle biopsy can increase the yield
of tumor tissue by more accurately
pinpointing the location of the tumor.
The diagnostic accuracy of core-needle
biopsy-based findings is reported to be
93%.19
3.Incisional Biopsy
Incisional biopsies are usually performed
only when fine-needle aspiration biopsy or
coreneedle biopsy specimens yield
nondiagnostic findings.
 Excisional Biopsy: A surgical procedure to
remove, for evaluation, all suspicious breast
tissue or tumor.