SYNOVIAL SARCOMA (SS) REPORT OF 11 CASES - PowerPoint Presentation

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SYNOVIAL SARCOMA (SS) REPORT OF 11 CASESW. Turki, M. Chelli Bouaziz, J. Ben Nasr, M.F. Ladeb.Service de Radiologie Institut MT Kassab d’orthopédie 2010 Ksar Said TunisieMK 215th PAN ARAB CONGRESS OF RADIOLOGY APRIL 2012

IMAGING OF

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IntroductionSynovial Sarcoma (SS) is a mesenchymal malignant tumor It represent 5 to 10% of soft tissues tumorsTwo 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 sizeImaging has an important role in the assessment of this tumor although appearance is not specificWe present 11 observations of soft tissue Synovial Sarcoma of limbs, emphasising their imaging characteristics with review of the literature

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Patients and MethodsSince 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.

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6 women and 5 men were included in the studyThe were aged between 13 to 64 years with a mean of 36 yearsLocation : 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

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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

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RadiographsFig.1 and 2: Juxta-articular calcified soft tissue mass.Fig. 3: Juxta-articular soft tissue mass with osteolysis and periosteal new bone formation of radius.Fig. 1Fig. 2Fig. 3 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

Results : Radiographs

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Fig. 4 and Fig. 5: Ultrasound of the kneeheterogenous solid mass of the infrapatellar knee region with increased Doppler Signal.Fig. 4Fig. 5Ultrasound showed echoic and heterogenous appearance, sometimes with calcifications with increased Doppler signal.Results : Ultrasound

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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. Fig. 6 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.Results : CT Scan

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On MRI, the tumor had well defined margins in all casesIn 8 patients, MRI signal intensity was intermediate to low compared to muscle on T1, high on T2, with heterogenous enhancement after IV Gadolinium injectionIn 4 patients, the signal was high and heterogenous on T2 with a "triple signal" appearanceIn one case, there was a multiloculated cystic appearance: Low intensity signal on T1, high intensity signal on T2 with internal septa enhancement after injectionIn 4 patients an associated bone lesion was revealed by MRIResults : MRI

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Axial T1-weighted imageSagittal T2-weighted imageFig. 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. 7Fig. 8

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Fig. 9 and Fig. 10: Sagittal T1-weighted image before and after IV Gadolinium injectionSoft 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. 11: Axial T2-weighted imageFig. 9Fig. 10Fig. 11

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Fig. 12: Sagittal T2-weighted image, "Triple signal" appearance Fig. 12

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Fig. 13: Sagittal T1-weighted imageFig. 14: Sagittal T2-weighted imageSynovial Sarcoma of the knee with Cystic appearance on MRI : low T1 (Fig. 13) and high T2 signal (Fig. 14)Fig. 13Fig. 14

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4th most frequent soft tissue neoplasms representing 2.5 to 10% [1,2]Occurs mainly in young adults : 15 – 40 years old80%–95% of synovial sarcomas occur in the limbs.60%–71% lower limb16%–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 : Epidemiology

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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 sarcomaNot found in other tumorsDiscussion : Pathology

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Radiographs are normal in 50% of casesOtherwise they typically show nonspecific, round to oval juxta-articular soft-tissue massesCalcifications 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 : Radiographs

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The US appearance of synovial sarcoma:In 66% of cases: focal, nodular, round or lobulated, solid hypoechoic soft-tissue massIn 14%: prominent heterogeneity with irregular margins20% of cases: complex sonographic appearance Doppler US demonstrates vascularity in the areas of viable tumor (1,11)Discussion : Ultrasound

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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 : CT

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Is the method of choice in the assessment of soft tissue tumorsOn 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 lesionsDiscussion : MRI

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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 muscleDiscussion : MRI

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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 : MRI

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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 : PET and SCINTIGRAPHY

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Statistically significant imaging features that favored a highGrade synovial sarcomaAbsence of calcificationPresence of cystic componentsPresence of hemorrhagePresence of the triple signImaging findings that were seen only with high-gradelesions : Cystic componentsHemorrhage Fluid levelsThe triple sign [1]Discussion :

Criteria for imaging grading

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The current treatment of choice is wide local excision : The surgical margins should be closely evaluated to determine the need for adjuvant therapyAmputation 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 tumoursInitiated 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 : Treatment

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Increasing signal intensity on T2-weighted MR images may be seen within the synovial sarcoma after chemotherapy or radiation therapy but does not mean recurrenceTumor size may also show a reduction in response to this therapyOedema surrounding the tumour, typically not a significant feature before therapy, may also develop subsequent to adjuvant treatmentDiscussion : Post treatment Imaging

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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 treatmentLate metastases may occur up to 26 years after the initial diagnosis, which reduces the 10-year versus 5-year survival ratesDiscussion : Local recurrence and Metastasis

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Clinical and pathologic factors having prognostic significance : Age: < 15–20 years is also associated with a better long-term prognosisLocation 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).Discussion : Prognosis factors

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CONCLUSIONSynovial sarcoma is the fourth most common malignant primary soft-tissue neoplasmAlthough 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 diagnosisMRI is the method of choice for staging extent and surgical planningLocal recurrence and metastatic disease are common and prognosis is guarded

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