Primary pericardial synovial sarcoma: A case report and literature review

Al-Rajhi, Nasser; Husain, Siraj; Coupland, Robert; McNamee, Ciaran; Jha, Naresh

doi:10.1002/(sici)1096-9098(199903)70:3<194::aid-jso10>3.0.co;2-8

Cited by 48 publications

(32 citation statements)

References 11 publications

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“…23,24 In two papers, the SYT-SSX fusion gene has been presented by RT-PCR. 25,26 This leaves restricted data for comparison of the cardiac cases in particular. The detection and description of the complex karyotype was facilitated by the use of multicolor FISH.…”

Section: Discussionmentioning

confidence: 99%

Primary synovial sarcoma of the heart: a cytogenetic and molecular genetic analysis combining RT-PCR and COBRA-FISH of a case with a complex karyotype

et al. 2004

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Synovial sarcomas usually occur in the soft tissues of the extremities of adolescents and middle-aged patients, in the vicinity of large joints. We present a patient with a synovial sarcoma of the left atrium and ventricle, which is an extremely rare location. Diagnosis was confirmed by reverse transcriptase polymerase chain reaction (RT-PCR), showing the t(X;18) fusion transcript. With a multicolor COmbined Binary RAtio labeling Fluorescence In Situ Hybridization (COBRA-FISH) technique, a complex karyotype evolved with identification of derivative chromosomes with multiplex rearrangements. This underscores the importance of molecular analyis of spindle cell tumors in unusual locations. Moreover, it shows that the presumed diagnostic translocation t(X;18) can be embedded in a sequence of other chromosomal rearrangements of which the function is as yet unknown.

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Section: Discussionmentioning

confidence: 99%

Primary synovial sarcoma of the heart: a cytogenetic and molecular genetic analysis combining RT-PCR and COBRA-FISH of a case with a complex karyotype

et al. 2004

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“…In the heart, SSs tend to arise from the heart but may also arise from the pericardium. [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23] Cases that arise from the heart locate predominantly to the right side-particularly the right atrium, with a right to left ratio of 2:1. Of the 26 cases (with information available) reported, 21 have originated in the heart (14 in the right atrium, 5 in left atrium, and 2 in left ventricle).…”

Section: Locationmentioning

confidence: 99%

“…Like other cardiac sarcomas, which are usually diagnosed at advanced stage, most synovial sarcomas tend to involve more than 1 anatomic compartment within the heart by the time they are discovered. [14][15][16][17][18][19][20][21][22][23] This may be explained by direct extension due to close proximity of the 4 chambers.…”

Section: Locationmentioning

confidence: 99%

“…23 Of the total cases reported in the English literature so far, only a few mentioned the race and ethnicity of patients. 4,[13][14][15] Since there are only a handful of cases reported so far, the prevalence of synovial sarcoma in 1 ethnic group compared to other groups cannot be established with certainty. Also in soft tissue SS, there appears to be no ethnic or racial predilection.…”

Section: Demographicsmentioning

confidence: 99%

“…Two other patients who were disease-free at 13 months and 12 months posttherapy had tumors that were pericardial in origin. 15,22 Since cardiac SSs are rare, prognostic factors are hard to ascertain, but younger age at diagnosis, absence of complex chromosomal abnormalities, and origin of tumor from the pericardium seem to be favorable factors.…”

Section: Prognosismentioning

confidence: 99%

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Primary Cardiac Synovial Sarcoma

Varma¹,

Adegboyega²

2012

Archives of Pathology &Amp; Laboratory Medicine

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Primary cardiac synovial sarcoma is an uncommon malignant neoplasm, with only a handful of cases reported in the English literature to date. Synovial sarcomas have also been described at other unusual sites, such as the heart, pleuropulmonary region, kidney, prostate, liver, mediastinum, retroperitoneum, gastrointestinal tract, and peripheral nerve. For synovial sarcomas that arise at these unusual locations, definitive diagnosis is challenging and requires use of ancillary diagnostic procedures, such as immunohistochemistry, electron microscopy, and molecular genetic techniques, for confirmation of diagnosis. The nonrandom occurrence of t(X;18) has been found consistently in synovial sarcomas. It has also been found as a sole cytogenetic abnormality in some cases, suggesting it as a key molecular event in tumor development. This review highlights salient features of primary cardiac synovial sarcoma and the associated diagnostic challenges.

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