By : Jannilyn Sandig MT 3A

8/14/2019 By : Jannilyn Sandig MT 3A

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By :

Jannilyn SandigMT 3A


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Definition

Malignant tumors of non epithelial,extraskeletal of the human body.

They form a diverse group ofmesenchymal malignancies, classifiedon a histologic basis according to theadult tissue they resemble or aresupposed to derive from.

represents one of the most frequentmalignant tumors of soft tissues,accounting for about 8% of all soft

tissue sarcomas
http://en.wikipedia.org/wiki/Mesenchymalhttp://en.wikipedia.org/wiki/Histologyhttp://en.wikipedia.org/wiki/Histologyhttp://en.wikipedia.org/wiki/Mesenchymal


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It is rare, it accounts 5-10 % of theapproximately 10,000 new soft

tissue sarcomas reported each year.

it makes up about 1 percent of all

cancers in adults and 15 percent ofall cancers in children.

About 50 percent of synovial

sarcomas develop in the legs,especially the knees.

The second most common location

is the arms


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Less frequently, the diseasedevelops in the trunk, head andneck region, or the abdomen.

. It is common for synovial cancerto recur (come back), usually

within the first two years aftertreatment.

it is a slow-growing tumor.

deep, painless soft-tissue massgreater than 5 cm in size issuspicious for a sarcoma


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Pathology and biology SS is clinically, morphologically

and genetically a distinctsarcoma, characterized by thespecific chromosomal

translocation t(X;18)(p11;q11).). Even if typical ofsoft tissues, SS are described

also in other sites, such as thekidney, lung, and pleura


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Signs and Symptoms

Swelling or mass (usually deep-seated)

Mass that may or may not beaccompanied by pain

Limping or difficulty using legs,arms, hands or feet.


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Gross Findings

The diameter of SSs varies from 3 to 10 centimetres(cm). Tumors tend to be multinodular and can becystic. When they grow slowly, they tend to havepushing margins and are circumscribed by a fibrous

pseudocapsule. Poorly differentiated SS growsrapidly with infiltrative margins, showinghaemorrhage and necrosis.


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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.


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Morphologically they areclassified as follow:

Biphasic SS

Monophasic SS

Monophasic epithelial SS (exceptional) Poorly differentiated SS


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Figure 1: This is a picture of a biphasic synovialsarcoma, in which we can see the coexistenceof both the epithelial glandular component and

the spindle cell component.


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Figure 2: This is a picture of a monophasic synovialsarcoma, in which only the spindle cell componentis present.
http://www.sarcomahelp.org/learning_center/figures/ss2.JPG


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Figure 3: This is a poorly differentiated synovialsarcoma, small cell type, characterized by a

prominent hemangiopericytomatous patternformed by dilated vascular structures, betweenwhich 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 geneticanalyses could be required to differentiate
http://www.sarcomahelp.org/learning_center/figures/ss3.JPG


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Extensive thigh hemangioma. This

large lesion involves much of the

medial thigh muscles (red arrows).


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Molecular Genetics

SS are characterized as a group by thepresence of a specific translocationt(X;18), that fuses the SYT gene fromchromosome 18 with SSX1 (about 2/3

of cases), SSX2 (about 1/3 of cases) orSSX4 (rare cases) gene from Xchromosome. As a consequence of the

translocation, a fusion transcript isformed at mRNA level, detectable byPCR tecniques. Cases with bothSYT/SSX1 and SYT/SSX2 fusion

transcripts have been described.


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SYT/SSX1 is reported significantly

associated with biphasic SS. Theassociation between SYT/SSX1 with

reduced metastasis-free survival in

localized tumors was not confirmed in

all series, and the prognostic relevanceof the fusion gene typing is still

uncertain.


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Clinical Features andDiagnosis

Most common feature of SS is a slowgrowing mass in the tissue of the low

extremity especially around knee and

ankle. The tumor is often near a joint,tendon orbursa. Head and neck

region, abdominal wall,

retroperitoneum, mediastinum,pleura, lungs and other organs are

less common locations.
http://en.wikipedia.org/wiki/Tendonhttp://en.wikipedia.org/wiki/Bursa_(anatomy)http://en.wikipedia.org/wiki/Retroperitoneumhttp://en.wikipedia.org/wiki/Mediastinumhttp://en.wikipedia.org/wiki/Pleurahttp://en.wikipedia.org/wiki/Pleurahttp://en.wikipedia.org/wiki/Mediastinumhttp://en.wikipedia.org/wiki/Retroperitoneumhttp://en.wikipedia.org/wiki/Bursa_(anatomy)http://en.wikipedia.org/wiki/Tendon


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Biopsy: Tissue is removed for examination

under a microscope.

Immunohistochemical analysis: Tumor tissueis 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 synovialsarcoma.


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MRI or magnetic resonance imaging

X- rays

Bone scans

CT scan or Computerized Tomography

scan


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This lesion in a 16-year old boy involving theelbow joint is hard to see on conventional

radiographs but readily imaged by MRI (red

arrow).


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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.
http://www.sarcomahelp.org/learning_center/figures/ss4.gif


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recurrence rate is high

lesion characteristically metastasizes tolymph nodes, bones, and lungs;

other sarcomas which spread via lymphnodes include: clear cell sarcoma, epithelioidsarcoma, rhabdomyosarcoma

- 5-year survival rate ranges from 25 to 55

%;

Prognosis
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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.


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Treatment

Chemotherapy

Surgery

Radiotheraphy

By : Jannilyn Sandig MT 3A

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