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Synovial Sarcoma: a Clinical Review

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Synovial Sarcoma: a Clinical Review Review Synovial Sarcoma: A Clinical Review Aaron M. Gazendam 1,*, Snezana Popovic 2, Sohaib Munir 3, Naveen Parasu 3, David Wilson 1 and Michelle Ghert 1 1 Department of Surgery, Division of Orthopaedic Surgery, McMaster University, Hamilton, ON L8V 1C3, Canada; [email protected] (D.W.); [email protected] (M.G.) 2 Department of Pathology and Molecular Medicine, McMaster University, Hamilton, ON L8S 4L8, Canada; [email protected] 3 Department of Radiology, McMaster University, Hamilton, ON L8V 1C3, Canada; [email protected] (S.M.); [email protected] (N.P.) * Correspondence: [email protected] Abstract: Synovial sarcomas (SS) represent a unique subset of soft tissue sarcomas (STS) and account for 5–10% of all STS. Synovial sarcoma differs from other STS by the relatively young age at diagnosis and clinical presentation. Synovial sarcomas have unique genomic characteristics and are driven by a pathognomonic t(X;18) chromosomal translocation and subsequent formation of the SS18:SSX fusion oncogenes. Similar to other STS, diagnosis can be obtained from a combination of history, physical examination, magnetic resonance imaging, biopsy and subsequent pathology, immunohistochemistry and molecular analysis. Increasing size, age and tumor grade have been demonstrated to be negative predictive factors for both local disease recurrence and metastasis. Wide surgical excision remains the standard of care for definitive treatment with adjuvant radiation utilized for larger and deeper lesions. There remains controversy surrounding the role of chemotherapy in the treatment of SS and there appears to be survival benefit in certain populations. As the understanding of the molecular and immunologic characteristics of SS evolve, several potential systematic therapies have been proposed. Keywords: synovial sarcoma; soft tissue sarcoma; clinical review; current concepts Citation: Gazendam, A.M.; Popovic, S.; Munir, S.; Parasu, N.; Wilson, D.; Ghert, M. Synovial Sarcoma: A 1. Introduction Clinical Review. Curr. Oncol. 2021, 28, 1909–1920. https://doi.org/ Synovial sarcoma is a relatively rare malignancy representing a soft tissue sarcoma 10.3390/curroncol28030177 (STS) of uncertain differentiation. It accounts for 5–10% of all STS [1–3]. The age-adjusted incidence is 0.81/1,000,000 in children and 1.42/1,000,000 in adults with approximately Received: 23 April 2021 1000 patients diagnosed with synovial sarcoma in the United States each year [4]. Synovial Accepted: 17 May 2021 sarcoma is unique from other STS as it presents at a younger mean age of onset and com- Published: 19 May 2021 monly occurs in adolescents and young adults (mean age of 39 years at diagnosis) and af- fects both sexes equally [5]. Synovial sarcoma is the most common non-rhabdomyosarcoma Publisher’s Note: MDPI stays neutral STS in children, representing 30% of STS diagnosed in childhood [4,6]. with regard to jurisdictional claims in published maps and institutional affil- 2. Clinical Presentation iations. 2.1. Location Although many synovial sarcomas originate near articular structures, the name syn- ovial sarcoma is a misnomer in that these lesions do not originate from intra-articular synovium, but from primitive mesenchymal cells [7]. Synovial sarcomas most commonly Copyright: © 2021 by the authors. present as soft tissue masses but cases of primary synovial sarcoma of bone have been Licensee MDPI, Basel, Switzerland. reported [8]. These lesions can occur anywhere in the body, with the majority arising in This article is an open access article the extremities, particularly in the lower extremity in the anatomic structures adjacent distributed under the terms and to the knee joint [4,9]. Synovial sarcomas are considered the most common STS of the conditions of the Creative Commons foot [6,10,11]. Attribution (CC BY) license (https:// creativecommons.org/licenses/by/ 4.0/). Curr. Oncol. 2021, 28, 1909–1920. https://doi.org/10.3390/curroncol28030177 https://www.mdpi.com/journal/curroncol Curr. Oncol. 2021, 28, 2 Curr. Oncol. 2021, 28 1910 2.2.2.2. Signs and Symptoms SynovialSynovial sarcomas often do notnot presentpresent withwith thethe typicaltypical STSSTS presentationpresentation ofof aa largelarge andand quickly growinggrowing painlesspainless massmass [[9].9]. Instead, thethe majoritymajority ofof synovial sarcomassarcomas areare slowslow growinggrowing andand the the mean mean duration duration of of symptoms symptoms before before diagnosis diagnosis is approximately is approximately 2 years 2 years [12]. In[12]. comparison In comparison to other to STS,other the STS, duration the durati of symptomson of symptoms are long andare patientslong and may patients have painmay orhave joint pain contractures or joint contractures that precede that swelling precede [13 sw]. Onlyelling half [13]. of Only patients half haveof patients clinical have findings clin- consistentical findings with consistent STS according with STS to according the National to the Institute National for Institute Health and for Health Care Excellence and Care (NICE)Excellence guidelines (NICE) guidelines [12,13]. Given [12,13]. the Given insidious the insidious onset, younger onset, younger age at presentation age at presenta- and atypicaltion and presenting atypical presenting symptoms, symptoms, these patients these may patients be initially may be clinically initially misdiagnosed clinically misdiag- with benignnosed with processes benign including processes myositis, including synovitis, myositis, bursitis synovitis, or tendonitis. bursitis or tendonitis. 3.3. Imaging 3.1.3.1. Plain Radiographs PlainPlain radiographsradiographs areare notnot requiredrequired forfor diagnosisdiagnosis butbut areare typicallytypically performedperformed asas partpart ofof thethe initialinitial workup and can identify adjacent bony remodeling, bone invasion or calcifi-calcifi- cationcation ofof thethe softsoft tissuetissue massmass (Figure(Figure1 1))[ [14–16]14–16].. Typically, synovialsynovial sarcomasarcoma presentspresents asas aa well-definedwell-defined oror lobulatedlobulated softsoft tissuetissue massmass onon plainplain radiographs.radiographs. PunctatePunctate calcifications,calcifications, particularlyparticularly aroundaround thethe peripheryperiphery ofof thethe lesion,lesion, are visualized in one third of patients [[17].17]. Occasionally,Occasionally, moremore extensiveextensive calcificationcalcification cancan bebe visualizedvisualized andand cancan mimicmimic bonebone formingforming tumors including osteosarcoma and myositis ossificans [17]. tumors including osteosarcoma and myositis ossificans [17]. FigureFigure 1.1. AP ((AA)) andand laterallateral ((BB)) radiographsradiographs of of the the right right (R) (R) knee knee and and lower lower leg leg in in a 31-year-olda 31-year-old male male demonstrate coarse calcifications within the soft tissues adjacent to the posteromedial tibial demonstrate coarse calcifications within the soft tissues adjacent to the posteromedial tibial plateau, plateau, corresponding to a biopsy proven synovial sarcoma. No significant articular abnormality. corresponding to a biopsy proven synovial sarcoma. No significant articular abnormality. Adjacent Adjacent bony structures appear unremarkable. bony structures appear unremarkable. 3.2.3.2. Cross-Sectional ImagingImaging SimilarSimilar toto otherother STS,STS, magneticmagnetic resonanceresonance imagingimaging (MRI)(MRI) withwith andand withoutwithout contrastcontrast isis the gold standard standard for for diagnostic diagnostic imaging imaging for for synovial synovial sarcoma sarcoma (Figure (Figure 2)2 [17].)[ 17 MRI]. MRI de- defines the local extent of the soft tissue mass and surrounding edema and provides Curr. Oncol. 2021, 28, 3 Curr. Oncol. 2021, 28 1911 fines the local extent of the soft tissue mass and surrounding edema and provides excel- lentexcellent visualization visualization of the ofmass the with mass respect with respect to the surrounding to the surrounding anatomy, anatomy, which is which critical is forcritical preoperative for preoperative planning. planning. The utilization The utilization of gadolinium of gadolinium contrast contrast can differentiate can differentiate be- tweenbetween hemorrhagic hemorrhagic or ornecrotic necrotic areas areas and and areas areas of of solid solid viable viable tumor. tumor. As As with with most most STS, STS, synovialsynovial sarcomas sarcomas are are typically typically heterogenous heterogenous with with low low intensity intensity on on T1 T1 and and high high intensity intensity onon T2-weighted T2-weighted images images with with post-gadolinium post-gadolinium enhancement enhancement [17]. [17]. FigureFigure 2. SagittalSagittal MR images inin thethe samesame patient patient demonstrate demonstrate a a periarticular periarticular soft soft tissue tissue mass mass situated situated posteromedial posteromedial to theto theproximal proximal tibia, tibia, in closein close relation relation to to the the pes pes anserine anserine tendons. tendons. The The mass mass demonstrates demonstrates intermediateintermediate signal on T1 weighted weighted imagesimages ( (AA),), and and heterogeneously heterogeneously high high signal signal on on T2 T2 weighted weighted fat-saturated fat-saturated images images ( (BB).). T1 T1 weighted weighted fat-saturated fat-saturated after after gadoliniumgadolinium administration administration ( (CC),), demonstrates demonstrates heterogeneousl heterogeneouslyy avid avid enhancement. enhancement. Known Known areas areas of of calcification
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