Synovial sarcoma
Updates to Article Attributes
Synovial sarcomas are relatively common intermediate-to-high grade malignant soft tissue tumours, often with an initial indolent course, affecting young patients, and involving the soft tissue surrounding the knees.
Epidemiology
Synovial sarcomas typically present in adolescents and young adults (15-40 years of age). There may be a mild male predilection (M:F = 1.2:1) male predilection. Synovial sarcomas They account for 2.5-10% of all soft tissue sarcoma 2-4.
Clinical presentation
PresentationThe presentation is most often with a slowly enlarging soft tissue mass which may have been noted for a number ofsome years and gives a false impression of a benign process 4.
Pathology
Synovial sacromasarcoma derives its name from the fact that microscopically it resembles normal synovium, however, it stains for epithelial markers (e.g. epithelial membrane antigen and cytokeratin), which synovium does not. Additionally, it has been found in many locations that doesdo not have normal synovium.
The most common location for these tumours is within the soft tissues adjacent to large joints, e.g. the knee and popliteal fossa. While these tumours arise near joints, it is rare for them to arise from the joint itself and despite their name, they do not arise from synovial structures, e.g. joints, tendon sheaths and bursae.
Macroscopic appearance
Macroscopically they have non-specific appearances. They are well or poorly defined heterogeneous masses with frequentcommon areas of haemorrhage and necrosis.
Histology
They are divided histologically into four subtypes:
- biphasic: 20-30%
- monophasic fibrous: 50-60%
- monophasic epithelial: very rare
- poorly differentiated: 15-25%
Genetics
Cytogenetic aberration of the t(X;18) translocation is highly specific, seen in over 90% of cases 1-4.
Associations
Synovial sarcoma is one of the (less common) causes of cannonball metastases to the lung.
Location
- extremities: 80-95% 1
- lower limb: 60-70%
- upper limb: 15-25%
- from soft tissues: 90-95%
- from joint: 5-10%
- head and neck: 5%
- hypopharynx or parapharyngeal space
- conjunctiva 3
- CNS
- chest wall: rare 6
- viscera: rare
- lung 2, kidney, prostate, oesophagus, heart, pericardium, pleura
Radiographic features
A soft tissue mass near but not in a joint in a young patient (15-40 years old), particularly if dystrophic calcification is present, are very suggestive.
Plain radiographs
X-rays may be normal, unless the mass is large or contains dystrophic calcificationcalcifications, which are found in up to 30% of cases 1. These calcifications are non-specific within the periphery of the lesion and not usually osteoid or chondroid in appearance.
Occasionally, smooth pressure erosions may be visible on the underlying bone, which should not be mistaken for a sign of a benign process.
Alternatively, franka direct bony invasion is seen in ~30% (range 11-50%) of cases, with aggressive bonybone features (permeative, geographic, or moth-eaten appearances) 4.
Ultrasound
Non-specific findings, with a heterogeneous predominantly hypoechoic mass.
CT
CT is non-specific, similar to ultrasound. It appears as a soft tissue mass of heterogeneous density and enhancement. CT is, of course, more sensitive to calcifications that either radiographs or MRI.
MRI
MRI is the modality of choice to locally stage the tumour locally, although again imaging findings are not pathognomonic. The mass is usually large and variably well-defined (smaller lesions tend to be better circumscribed).
-
T1
- iso- (slightly hyper-) intense to muscle
- heterogeneous
-
T2
- mostly hyperintense
- markedly heterogeneous appearance of synovial cell sarcomas on fluid
sensitive-sensitive sequencesresultresults in socalled-called "triple sign" which is due to areas of necrosis and cystic degeneration with very high signal, relatively high signal soft tissue components,and areas of low signal intensity due to dystrophic calcifications and fibrotic bands - due to the high tendency of lesions to bleed, there might be areas of fluid-fluid levels known as "bowl of grapes" are seen in up to 10-25% of cases
-
T1 C + (Gd)
-
-
Bone scan
Typically synovial sarcomas have heterogeneously increased dynamic and blood pool uptake, with some increase in delayed images, but usually less intense.
Angiography/DSA
This imaging test is not usually performed. These tumours are usually hypovascular 1.
Treatment and prognosis
Treatment involves a combination of surgery and usually adjuvant radiotherapy +/- chemotherapy. Radiotherapy is particularly useful in treating tumours where an adequate clear margin cannot be achieved, and ideally, radiotherapy is administered pre-operatively.
Good prognostic variables include:
- small size
- located in the extremity
- younger age <20 years of age
- solid homogenous mass
- presence of calcification
- biphasic histology (controversial)
Poor prognostic variables include:
- large size (>5 cm): the most important factor
- located in the trunk or head and neck
- older patients
- cystic/haemorrhagic components
- marked heterogeneity
- histology
- poorly differentiated histology
- rhabdoid cells
- extensive tumour necrosis
- high nuclear grade
- p53 mutations
- high mitotic rate (>10 mitoses per 10 high-power field)
Overall 5 year-year survival is between 36-76% with both. Both local recurrence (30-50%) and distant metastases are frequent (40-70%), most commonly to the lungs (~80%), bones (~15%), regional lymph nodes (~10%), followed by chest wall / abdomen (~7.5%) 7,8.
Differential diagnosis
General imaging differential considerations include:
-<p><strong>Synovial sarcomas</strong> are relatively common intermediate-to-high grade malignant soft tissue tumours, often with an initial indolent course. </p><h4>Epidemiology</h4><p>Synovial sarcomas typically present in adolescents and young adults (15-40 years of age). There may be a mild (M:F = 1.2:1) male predilection. Synovial sarcomas account for 2.5-10% of all soft tissue sarcoma <sup>2-4</sup>.</p><h4>Clinical presentation</h4><p>Presentation is most often with a slowly enlarging soft tissue mass which may have been noted for a number of years and gives a false impression of a benign process <sup>4</sup>. </p><h4>Pathology</h4><p>Synovial sacroma derives its name from the fact that microscopically it resembles normal synovium, however it stains for epithelial markers (e.g. epithelial membrane antigen and cytokeratin) which synovium does not. Additionally it has been found in many locations that does not have normal synovium.</p><p>The most common location for these tumours is within the soft tissues adjacent to large joints, e.g. the <a title="knee" href="/articles/knee">knee</a> and <a title="Popliteal fossa" href="/articles/popliteal-fossa">popliteal fossa</a>. While these tumours arise near joints, it is rare for them to arise from the joint itself and despite their name, they do not arise from synovial structures, e.g. joints, tendon sheaths and bursae.</p><h5>Macroscopic appearance</h5><p>Macroscopically they have non-specific appearances. They are well or poorly defined heterogeneous masses with frequent areas of haemorrhage and necrosis. </p><h5>Histology</h5><p>They are divided histologically into four subtypes:</p><ul>- +<p><strong>Synovial sarcomas</strong> are relatively common intermediate-to-high grade malignant soft tissue tumours, often with an initial indolent course, affecting young patients, and involving the soft tissue surrounding the knees. </p><h4>Epidemiology</h4><p>Synovial sarcomas typically present in adolescents and young adults (15-40 years of age). There may be a mild male predilection (M:F = 1.2:1). They account for 2.5-10% of all soft tissue sarcoma <sup>2-4</sup>.</p><h4>Clinical presentation</h4><p>The presentation is most often with a slowly enlarging soft tissue mass which may have been noted for some years and gives a false impression of a benign process <sup>4</sup>. </p><h4>Pathology</h4><p>Synovial sarcoma derives its name from the fact that microscopically it resembles normal synovium, however, it stains for epithelial markers (e.g. epithelial membrane antigen and cytokeratin), which synovium does not. Additionally, it has been found in many locations that do not have normal synovium.</p><p>The most common location for these tumours is within the soft tissues adjacent to large joints, e.g. the <a href="/articles/knee">knee</a> and <a href="/articles/popliteal-fossa">popliteal fossa</a>. While these tumours arise near joints, it is rare for them to arise from the joint itself and despite their name, they do not arise from synovial structures, e.g. joints, tendon sheaths and bursae.</p><h5>Macroscopic appearance</h5><p>Macroscopically they have non-specific appearances. They are well or poorly defined heterogeneous masses with common areas of haemorrhage and necrosis. </p><h5>Histology</h5><p>They are divided histologically into four subtypes:</p><ul>
-<li>chest wall: rare 6</li>- +<li>chest wall: rare <sup>6</sup>
- +</li>
-</ul><h4>Radiographic features</h4><p>A soft tissue mass near but not in a joint in a young patient (15-40 years old), particularly if calcification is present, are very suggestive.</p><h5>Plain radiographs</h5><p>X-rays may be normal, unless the mass is large or contains dystrophic calcification, which are found in up to 30% of cases <sup>1</sup>. These calcifications are non-specific within the periphery of the lesion and not usually osteoid or chondroid in appearance. </p><p>Occasionally, smooth pressure erosions may be visible on the underlying bone, which should not be mistaken for a sign of a benign process. </p><p>Alternatively, frank bony invasion is seen in ~30% (range 11-50%) of cases, with aggressive bony features (permeative, geographic, or moth-eaten appearances) <sup>4</sup>. </p><h5>Ultrasound</h5><p>Non-specific findings, with a heterogeneous predominantly hypoechoic mass. </p><h5>CT</h5><p>CT is non-specific, similar to ultrasound. It appears as a soft tissue mass of heterogeneous density and enhancement. CT is of course more sensitive to calcifications that either radiographs or MRI. </p><h5>MRI</h5><p>MRI is the modality of choice to locally stage the tumour, although again imaging findings are not pathognomonic. The mass is usually large and variably well-defined (smaller lesions tend to be better circumscribed). </p><ul>- +</ul><h4>Radiographic features</h4><p>A soft tissue mass near but not in a joint in a young patient (15-40 years old), particularly if dystrophic calcification is present, are very suggestive.</p><h5>Plain radiographs</h5><p>X-rays may be normal unless the mass is large or contains dystrophic calcifications, which are found in up to 30% of cases <sup>1</sup>. These calcifications are non-specific within the periphery of the lesion and not usually osteoid or chondroid in appearance. </p><p>Occasionally, smooth pressure erosions may be visible on the underlying bone, which should not be mistaken for a sign of a benign process. </p><p>Alternatively, a direct bony invasion is seen in ~30% (range 11-50%) of cases, with aggressive bone features (permeative, geographic, or moth-eaten appearances) <sup>4</sup>. </p><h5>Ultrasound</h5><p>Non-specific findings, with a heterogeneous predominantly hypoechoic mass. </p><h5>CT</h5><p>CT is non-specific, similar to ultrasound. It appears as a soft tissue mass of heterogeneous density and enhancement. CT is, of course, more sensitive to calcifications that either radiographs or MRI. </p><h5>MRI</h5><p>MRI is the modality of choice to stage the tumour locally, although again imaging findings are not pathognomonic. The mass is usually large and variably well-defined (smaller lesions tend to be better circumscribed). </p><ul>
-<li>markedly heterogeneous appearance of synovial cell sarcomas on fluid sensitive sequences result in so called "<a href="/articles/triple-sign">triple sign</a>" which is due to areas of necrosis and cystic degeneration with very high signal, relatively high signal soft tissue components, and areas of low signal intensity due to dystrophic calcifications and fibrotic bands</li>-<li>due to high tendency of lesions to bleed, there might be areas of <a href="/articles/fluid-fluid-level-containing-bone-lesions-2">fluid-fluid levels</a> known as "<a href="/articles/bowel-of-grapes">bowl of grapes</a>" are seen in up to 10-25% of cases</li>- +<li>markedly heterogeneous appearance of synovial cell sarcomas on fluid-sensitive sequences results in so-called "<a href="/articles/triple-sign">triple sign</a>" which is due to areas of necrosis and cystic degeneration with very high signal, relatively high signal soft tissue components and areas of low signal intensity due to dystrophic calcifications and fibrotic bands</li>
- +<li>due to the high tendency of lesions to bleed, there might be areas of <a href="/articles/fluid-fluid-level-containing-bone-lesions-2">fluid-fluid levels</a> known as "<a href="/articles/bowel-of-grapes">bowl of grapes</a>" are seen in up to 10-25% of cases</li>
-<strong>T1 C + (Gd)</strong><ul><li>-<strong></strong>enhancement is usually prominent and can be diffuse (40%) heterogeneous (40%) or peripheral (20%) <sup>1</sup>- +<strong>T1 C + (Gd)</strong><ul><li>enhancement is usually prominent and can be diffuse (40%) heterogeneous (40%) or peripheral (20%) <sup>1</sup>
-</ul><h5>Bone scan</h5><p>Typically synovial sarcomas have heterogeneously increased dynamic and blood pool uptake, with some increase in delayed images, but usually less intense. </p><h5>Angiography/DSA</h5><p>This imaging test is not usually performed. These tumours are usually hypovascular <sup>1</sup>. </p><h4>Treatment and prognosis</h4><p>Treatment involves a combination of surgery and usually adjuvant radiotherapy +/- chemotherapy. Radiotherapy is particularly useful in treating tumours where an adequate clear margin cannot be achieved, and ideally radiotherapy is administered pre-operatively. </p><p>Good prognostic variables include:</p><ul>- +</ul><h5>Bone scan</h5><p>Typically synovial sarcomas have heterogeneously increased dynamic and blood pool uptake, with some increase in delayed images, but usually less intense. </p><h5>Angiography/DSA</h5><p>This imaging test is not usually performed. These tumours are usually hypovascular <sup>1</sup>. </p><h4>Treatment and prognosis</h4><p>Treatment involves a combination of surgery and usually adjuvant radiotherapy +/- chemotherapy. Radiotherapy is particularly useful in treating tumours where an adequate clear margin cannot be achieved, and ideally, radiotherapy is administered pre-operatively. </p><p>Good prognostic variables include:</p><ul>
-<li>located in extremity</li>- +<li>located in the extremity</li>
-<li>large size (>5 cm): most important factor</li>- +<li>large size (>5 cm): the most important factor</li>
-</ul><p>Overall 5 year survival is between 36-76% with both local recurrence (30-50%) and distant metastases are frequent (40-70%), most commonly to the lungs (~80%), bones (~15%), regional lymph nodes (~10%), followed by chest wall / abdomen (~7.5%) <sup>7,8</sup>.</p><h4>Differential diagnosis</h4><p>General imaging differential considerations include:</p><ul>- +</ul><p>Overall 5-year survival is between 36-76%. Both local recurrence (30-50%) and distant metastases are frequent (40-70%), most commonly to the lungs (~80%), bones (~15%), regional lymph nodes (~10%), followed by chest wall / abdomen (~7.5%) <sup>7,8</sup>.</p><h4>Differential diagnosis</h4><p>General imaging differential considerations include:</p><ul>
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