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By :

Jannilyn Sandig
MT 3A
Definition
Malignant tumors of non epithelial,
extraskeletal of the human body.
They form a diverse group of
mesenchymal malignancies, classified
on a histologic basis according to the
adult tissue they resemble or are
supposed to derive from.
represents one of the most frequent
malignant tumors of soft tissues,
accounting for about 8% of all soft
tissue sarcomas
It is rare, it accounts 5-10 % of the
approximately 10,000 new soft
tissue sarcomas reported each year
.
it makes up about 1 percent of all
cancers in adults and 15 percent of
all cancers in children.
About 50 percent of synovial
sarcomas develop in the legs,
especially the knees.
The second most common location
is the arms
Less frequently, the disease
develops in the trunk, head and
neck region, or the abdomen.
. It is common for synovial cancer
to recur (come back), usually
within the first two years after
treatment.
it is a slow-growing tumor.
deep, painless soft-tissue mass
greater than 5 cm in size is
suspicious for a sarcoma
Pathology and biology
• SS is clinically, morphologically
and genetically a distinct
sarcoma, characterized by the
specific chromosomal
translocation t(X;18)
(p11;q11).). Even if typical of
soft tissues, SS are described
also in other sites, such as the
kidney, lung, and pleura
Signs and Symptoms
Swelling or mass (usually deep-
seated)
Mass that may or may not be
accompanied by pain
Limping or difficulty using legs,
arms, hands or feet.
Gross Findings
• The diameter of SSs varies from 3 to 10 centimetres
(cm). Tumors tend to be multinodular and can be
cystic. When they grow slowly, they tend to have
pushing margins and are circumscribed by a fibrous
pseudocapsule. Poorly differentiated SS grows
rapidly with infiltrative margins, showing
haemorrhage and necrosis.
Histological Findings
SSs are composed of two
morphologically and
immunophenotypically distinct cell
types: spindle cells, uniform and
relatively small, with oval nuclei and
scarce cytoplasm, forming solid
sheets, and epithelial cells,
characterized by true epithelial
differentiation.
Morphologically they are
classified as follow:
• Biphasic SS
• Monophasic SS
• Monophasic epithelial SS (exceptional)
• Poorly differentiated SS
Figure 1: This is a picture of a biphasic synovial
sarcoma, in which we can see the coexistence
of both the epithelial glandular component and
the spindle cell component.
• Figure 2: This is a picture of a monophasic synovial
sarcoma, in which only the spindle cell component
is present.
Figure 3: This is a poorly differentiated synovial
sarcoma, small cell type, characterized by a
prominent hemangiopericytomatous pattern
formed by dilated vascular structures, between
which there are cells that are roundish and small,
like those seen in a small round cell tumor, i.e.
Ewing's sarcoma. In these cases molecular genetic
analyses could be required to differentiate
• Extensive thigh hemangioma. This
large lesion involves much of the
medial thigh muscles (red arrows).
Molecular Genetics
• SS are characterized as a group by the
presence of a specific translocation
t(X;18), that fuses the SYT gene from
chromosome 18 with SSX1 (about 2/3
of cases), SSX2 (about 1/3 of cases) or
SSX4 (rare cases) gene from X
chromosome. As a consequence of the
translocation, a fusion transcript is
formed at mRNA level, detectable by
PCR tecniques. Cases with both
SYT/SSX1 and SYT/SSX2 fusion
transcripts have been described.
• SYT/SSX1 is reported significantly
associated with biphasic SS. The
association between SYT/SSX1 with
reduced metastasis-free survival in
localized tumors was not confirmed in
all series, and the prognostic relevance
of the fusion gene typing is still
uncertain.
Clinical Features and
Diagnosis
• Most common feature of SS is a slow
growing mass in the tissue of the low
extremity especially around knee and
ankle. The tumor is often near a joint,
tendon or bursa. Head and neck
region, abdominal wall,
retroperitoneum, mediastinum,
pleura, lungs and other organs are
less common locations.
Biopsy: Tissue is removed for examination
under a microscope.
Immunohistochemical analysis: Tumor tissue
is tested for certain antigen and antibody
interactions common to synovial sarcoma.
Ultrastructural findings: The tissue is examined
using an ultramicroscope and electron
microscope.
Genetic testing: Tissue is tested for a specific
chromosome abnormality common to synovial
sarcoma.
MRI or magnetic resonance imaging
X- rays
Bone scans
CT scan or Computerized Tomography
scan
• This lesion in a 16-year old boy involving the
elbow joint is hard to see on conventional
radiographs but readily imaged by MRI (red
arrow).
• Figure 4: Magnetic Resonance Imaging
(MRI) of a 17-year old boy with a synovial
sarcoma of the proximal thigh: MRI is
mandatory for the adequate assessment of
the local extension of the tumor. MRI
seems superior to CT scans in defining soft
tissue extension.
Prognosis
recurrence rate is high
lesion characteristically metastasizes to
lymph nodes, bones, and lungs;

other sarcomas which spread via lymph


nodes include: clear cell sarcoma, epithelioid
sarcoma, rhabdomyosarcoma
- 5-year survival rate ranges from 25 to 55
%;
The prognosis of SS patients is related to
the feasibility of surgical resection, tumor
size, and local invasiveness.
Patients with small tumors that can be
completely removed at diagnosis have an
excellent prognosis.
For tumors larger than 5 cm, the risk of
developing distant metastases is higher.
Treatment
Chemotherapy
Surgery
Radiotheraphy