Transcript Diapositive 1

IMAGING OF
Introduction
 Synovial Sarcoma (SS) is a mesenchymal malignant tumor
 It represent 5 to 10% of soft tissues tumors
 Two features associated with synovial sarcoma that may lead to
an initial mistaken diagnosis of a benign indolent process are
slow growth (average time to diagnosis, 2–4 years) and small size
 Imaging has an important role in the assessment of this tumor
although appearance is not specific
 We present 11 observations of soft tissue Synovial Sarcoma of
limbs, emphasising their imaging characteristics with review of
the literature
Patients and Methods

Since 1995, 11 observations of soft tissue Synovial Sarcoma of
limbs have been detected.

All patients had radiographs, 2 were examined by high
resolution ultrasound, 4 by CT and 9 by MRI.
Results

6 women and 5 men were included in the study

The were aged between 13 to 64 years with a mean of 36 years

Location :

The lower limbs were involved in 9 cases: knee (n=5) , 1 in
thigh (n= 1) leg (n= 1), ankle (n= 1) and foot (n= 1)

2 tumors were located in the upper limb: forearm (n= 1),
shoulder (n= 1).
Results

9 Synovial Sarcoma had a juxta-articular location.

2 were located in soft tissues of leg and in that of thigh, away
from the joint.

The size of the tumors ranged from 4 to 14 cm with an average
of 7.8 cm.
Results : Radiographs
Radiographs showed a soft tissue mass, associated with
calcifications in 3 cases, osteolysis in 2 cases, unilamellar
periosteal reaction in one case, and bone condensation facing
the mass in one case
Fig. 1
Fig. 2
Radiographs
Fig.1 and 2: Juxta-articular calcified soft tissue mass.
Fig. 3
Fig. 3: Juxta-articular soft tissue mass with osteolysis
and periosteal new bone formation of radius.
Results : Ultrasound
Ultrasound showed echoic and heterogenous appearance,
sometimes with calcifications with increased Doppler
Fig. 5
signal.
Fig. 4
Fig. 4 and Fig. 5: Ultrasound of the knee
heterogenous solid mass of the infrapatellar knee region with increased
Doppler Signal.
Results : CT Scan
CT confirmed showed a solid soft tissue mass in three cases
and a multiloculated cystic mass in one case. Calcifications
within lesions were observed in two patients and a bone
lesion in 3 patients.
Fig. 6
Fig. 6 (Same case as Fig and 5) transversal
CT view of the knee in soft tissue algorithm:
solid infrapatellar soft tissue mass
containing coarse calcifications, and
infiltrating the subcutaneous tissue.
Results : MRI

On MRI, the tumor had well defined margins in all cases

In 8 patients, MRI signal intensity was intermediate to low
compared to muscle on T1, high on T2, with heterogenous
enhancement after IV Gadolinium injection

In 4 patients, the signal was high and heterogenous on T2 with a
"triple signal" appearance

In one case, there was a multiloculated cystic appearance: Low
intensity signal on T1, high intensity signal on T2 with internal septa
enhancement after injection

In 4 patients an associated bone lesion was revealed by MRI
Fig. 7
Fig. 8
Axial T1-weighted image
Sagittal T2-weighted image
Fig. 7 and Fig. 8: Well defined mass with a low signal on T1 weighted images and a
heterogenous high signal on T2 weighted images.
Fig. 11
Fig. 9
Fig. 10
Fig. 11: Axial T2-weighted image
Fig. 9 and Fig. 10: Sagittal T1-weighted
image before and after IV Gadolinium
injection
Soft tissue mass having an intermediate signal (similar to muscle) on T1 (Fig. 9),
heterogenous high signal on T2 (Fig. 11) and a heterogenous enhancement after
IV Gadolinium injection (Fig. 10)
Fig. 12
Fig. 12: Sagittal T2-weighted image, "Triple signal" appearance
Fig. 13
Fig. 13: Sagittal T1-weighted image
Fig. 14
Fig. 14: Sagittal T2-weighted image
Synovial Sarcoma of the knee with Cystic appearance on MRI : low T1 (Fig.
13) and high T2 signal (Fig. 14)
Discussion : Epidemiology
 4th most frequent soft tissue neoplasms representing 2.5 to
10% [1,2]
 Occurs mainly in young adults : 15 – 40 years old
 80%–95% of synovial sarcomas occur in the limbs.
 60%–71% lower limb
 16%–25% upper limb [1,4,5,6,7]
 Despite its name, less than 10% of SS arise within a joint
 Mostly adjacent (40%–50% ) or nearby (60%–75%)a joint
space, with a mean diatance of 5 cm.
 Intra-articular involvement is more commonly due to the
extension of a juxta-articular neoplasm [1-2-3].
Discussion : Pathology
 Synovial sarcoma is an intermediate to high grade tumor.
 There are three main histologic subtypes of synovial sarcoma:
biphasic (20%-30%), monophasic(50-60%), and poorly differentiated
(15-25%) [1,8].
 Grading of synovial sarcoma is achieved by applying the grading
scheme for all sarcomas by the FNCLCC (French Federation Nationale
des Centres de Lutte Contre le Cancer) : degree of differentiation,
mitotic activity, and necrosis.
 The cytogenetic aberration of the t(X;18) translocation is highly
specific for synovial sarcoma [1,23]. :
 Observed in 90% of synovial sarcoma
 Not found in other tumors
Discussion : Radiographs
 Radiographs are normal in 50% of cases
 Otherwise they typically show nonspecific, round to oval
juxta-articular soft-tissue masses
 Calcifications are identified in up to 30% of synovial
sarcomas. These are often eccentric or peripheral within
and nonspecific in appearance [1,9]
 Extrinsic bone erosions or periosteal new bone formation
have been reported in 11%–20% of cases
 Aggressive bone invasion is far less common (5% of cases)
Discussion : Ultrasound
The US appearance of synovial sarcoma:
 In 66% of cases: focal, nodular, round or lobulated, solid
hypoechoic soft-tissue mass
 In 14%: prominent heterogeneity with irregular margins
 20% of cases: complex sonographic appearance
 Doppler US demonstrates vascularity in the areas of viable
tumor (1,11)
Discussion : CT
 Is not a good imaging modality for the assessment of soft tissue
tumors.
 Non specific appearance : heterogenous multinodular soft-tissue mass
 Areas of lower attenuation representing necrosis or hemorrhage are
also common (1,9,11)
 Interesting to show calcifications
( 27%–41%) and adjacent bone
abnormalities ( nearly 25% of lesions)
 A cystic form had been described in a minority of cases (12,24)
Discussion : MRI
 Is the method of choice in the assessment of soft tissue tumors
 On T1-weighted MR images: heterogeneous multilobulated soft tissue
mass with intermediate to low signal

On T2-weighted MR images:
heterogeneity with predominant high
signal intensity (1,22), sometimes a multilobulated mass with intervening
septa.
 The enhancement is more commonly heterogeneous (83%–100%) than
homogeneous (0%– 17%).
 It may be diffuse, Peripheral, nodular , with or without thick septa in
largely necrotic lesions
Discussion : MRI
 Areas of haemorrhage, are seen as fluid levels or foci of high signal
intensity on T1- and T2-weighted MR images (47%)
 The triple sign: Described by Jones et al (see Fig 12)
- Is the result of intermixed areas of low (calcified or fibrotic
collagenized regions), intermediate (solid cellular elements), and
high signal intensity on T2 (hemorrhage or necrosis)
- It has been described as occurring in 35%–57% of cases but also
seen in other soft-tissue neoplasms (1,21,22)
 Bone involvement, manifested either by cortical erosion or invasion of
the marrow space (21% )
 Frequently invade adjacent muscle
Discussion : MRI
 Diffusion shows an increased signal in benign soft tissue masses
compared to their malignant counterparts, whereas the ADC
values between these groups are not significantly different. This
difference can be explained by the contribution of perfusion to
the ADC values [26].
 The dynamic contrast-enhanced MR imaging appearance of
synovial sarcoma may be a rapid progressive linear increase in
signal intensity followed by washout or plateau (60%), or a late
sustained increase in enhancement after the initial rapid
enhancement (40%) [1]
Discussion :
PET and SCINTIGRAPHY
 Scintigraphic evaluation reveal prominent increased
uptake. It has an important role in the detection of possible
metastatic disease and in monitoring response to therapy
[1,25]
 Positron emission tomography demonstrates marked
increased tracer accumulation [1,10]
Discussion :
Criteria for imaging grading
Statistically significant imaging features that favored a high
Grade synovial sarcoma
 Absence of calcification
 Presence of cystic components
 Presence of hemorrhage
 Presence of the triple sign
Imaging findings that were seen only with high-grade
lesions :
 Cystic components
 Hemorrhage
 Fluid levels
 The triple sign [1]
Discussion : Treatment
 The current treatment of choice is wide local excision :
 The surgical margins should be closely evaluated to
determine the need for adjuvant therapy
 Amputation should be reserved for those cases in which
gross resection of the tumour and preservation of a
functional limb is not possible [1]
 Radiation therapy plays an important role in the treatment of
marginally resected tumours
 Initiated preoperatively if the surgeon believes that the
surgical margins will be positive or close
 If the margins are microscopically positive, radiation
should be given postoperatively [1,14,15]
Discussion :
Post treatment Imaging
 Increasing signal intensity on T2-weighted MR images may
be seen within the synovial sarcoma after chemotherapy or
radiation therapy but does not mean recurrence
 Tumor size may also show a reduction in response to this
therapy
 Oedema surrounding the tumour, typically not a
significant feature before therapy, may also develop
subsequent to adjuvant treatment
Discussion :
Local recurrence and Metastasis
 The clinical course of synovial sarcoma is characterized by a high rate of
local recurrence and metastatic disease.
 Local recurrence following resection occurs in 30%– 50% of patients, and
distant metastasis in 41% [16]
 The most frequent metastatic site is the lung (94%), followed by lymph
nodes (4%–18%) and bone (8%–11%)
 Metastases are present in 16%–25% of patients at their initial presentation
[17]
 The majority of metastases occur within the first 2–5 years after treatment
 Late metastases may occur up to 26 years after the initial diagnosis, which
reduces the 10-year versus 5-year survival rates
Discussion : Prognosis factors
Clinical and pathologic factors having prognostic significance :
 Age: < 15–20 years is also associated with a better long-term prognosis
 Location in the extremities: more favourable prognosis
 Tumour size: greater than 5 cm at presentation has the greatest impact
(64% vs 26% 5 years survival rates)
 The presence of extensive calcifications: suggests improved long-term
survival, with 5-year survival rates of 82% and decreased rates of local
recurrence (32%) and metastatic disease (29%) (1,16,18,19)
 Other factors:
 Degree of differenciation
 high nuclear grade
 More recently, the gene fusion type SYT-SSX2 (more common in
monophasic lesions) has been associated with an improved
prognosis (compared with that for SYT-SSX1, and an 89%
metastasis-free survival).
CONCLUSION
 Synovial sarcoma is the fourth most common malignant primary
soft-tissue neoplasm
 Although the radiographic and ultrasonographic signs of
synovial sarcoma are not specific, the presence of a soft-tissue
mass, nearby a joint in a young patient is very suggestive of this
diagnosis
 MRI is the method of choice for staging extent and surgical
planning
 Local recurrence and metastatic disease are common and
prognosis is guarded
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