Primary cardiac sarcomas: an immunohistochemical and grading study with long‐term follow‐up of 24 cases

Donsbeck, A V; Ranchère, Dominique; Coindre, Jean‐Michel; Gall, François Le; Jf, Cordier; Loire, R

doi:10.1046/j.1365-2559.1999.00636.x

Cited by 172 publications

(145 citation statements)

References 15 publications

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“…Patients generally present with complaints of dyspnea, fatigue, chest pain, or congestive heart failure, sometimes with syncope or ischemic attacks due to tumor emboli. [9][10][11][12] Most tumors are located in the right heart (see Table 1), and males outweigh females by about 2.5 to 1. As can be seen in Table 1, patients with cardiac SS have an extremely adverse prognosis, as eight of 11 patients with known follow-up died shortly after diagnosis and therapy, and the three 'survivors' have been followed for no more than 12 months.…”

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%

“…The size of the tumors is variable and ranges from 2.9 to 15 cm 7,12 (Figures 1 and 2). Left-sided lesions are of comparatively smaller size because they tend to present early owing to their mass effect and obstruction of pulmonary veins.…”

Section: Macroscopic Featuresmentioning

confidence: 99%

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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“…[1][2][3] Synovial sarcomas predominantly occur in para-articular soft tissues of the extremities of young adults and adolescents. 4 Most cardiac synovial sarcomas are located in the right side of the heart.…”

mentioning

confidence: 99%

“…Complete resection is seldom possible and if so, frequently followed by recurrent illness within two years. 1,2 Due to the rarity of this malignancy, no standardised medical therapy exists (both older and newer regimes). Radiation therapy is known to be of value in cases where the margins are not tumour free, to reduce local recurrence rates.…”

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confidence: 99%

Primary monophasic mediastinal, cardiac and pericardial synovial sarcoma: a young man in distress

Zwaan¹,

Garsse²,

Jansen³

et al. 2007

NHJL

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A 19-year-old male was admitted because of exertional dyspnoea. The imaging studies revealed epicardial, pericardial and mediastinal masses. The tumours could not be resected through a minor thoracotomy, only biopsies could be taken. Analyses led to the final diagnosis of a monophasic synovial sarcoma. The patient preferred a conservative and palliative approach. Three months later he died at home. Autopsy demonstrated dramatic extension of the tumour masses. We conclude this report with a discussion on primary cardiac tumours. (Neth Heart J 2007;15:226-8.)Keywords: distress, sarcoma, heart I n May 2005, a 19-year-old male was admitted to our hospital suffering from exertional dyspnoea and a swollen abdomen. After a cold in January 2005 he started to lose weight and became progressively more dyspnoeic. He had not been exposed to asbestos.On physical examination, he was afebrile, sweaty, and in some distress. He had a blood pressure of 135/80 mmHg, with no pulsus paradoxus. Furthermore, he had congested jugular veins, diminished basal pulmonary breathing sounds, hepatomegaly, ascites and ankle oedema. The electrocardiogram showed a sinus rhythm, 90 beats/min, an intermediate axis and nonspecific repolarisation abnormalities.The laboratory data revealed mild liver function abnormalities (serum aspartate aminotransferase 108 U/l, alanine aminotransferase 97 U/l, lactate dehydrogenase 724 U/l, bilirubin 23 µmol/l), and a moderately elevated plasma pro-brain natriuretic peptide (95.8 pmol/l).Chest radiography demonstrated a widened cardiac shadow, bilateral pleural effusion and atelectasis of the left lung.An echocardiogram showed a normal left ventricular systolic function and a grade 3 mitral incompetence; a large amount of pericardial effusion was seen, without signs of tamponade, and a large mass within the pericardium. Furthermore, a large mass on top of the left atrium, posterior to the ascending aorta, could be observed. In addition, several small tumours were located on the epicardial surface (figure 1).A computed tomography and a magnetic resonance imaging scan (MRI) confirmed the pericardial and pleural effusions and clearly showed the epicardial, pericardial and mediastinal masses. A large mediastinal mass was present around the aorta and pulmonary artery (figure 2). These imaging studies, as well as the physical examinations, failed to reveal other masses in the trunk or extremities.The patient underwent surgery to obtain biopsies or excise the tumours. He was haemodynamically unstable and single lung ventilation was insufficient due to external compression of the right main stem bronchus. Via a minor anterior lateral thoracotomy, 1100 ml of haemorrhagic pericardial fluid was evacuated and a large pericardial window was created. A massive glassy tumour (measuring 10 cm x 10 cm x 15 cm) with extensive areas of necrosis and haemorrhage was found on the diaphragm within the thickened pericardium. The restrictive epicardium was interlaced with multiple small glassy tumours that bled easily. A lymphoid mass wa...

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Primary cardiac sarcomas: an immunohistochemical and grading study with long‐term follow‐up of 24 cases

Abstract: All types of sarcomas may be observed in the heart with a predominance of undifferentiated sarcomas. Histological grading, unlike histological type, seems to correlate with survival which remains extremely poor.

Cited by 172 publications

References 15 publications

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

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

Primary Cardiac Synovial Sarcoma

Primary monophasic mediastinal, cardiac and pericardial synovial sarcoma: a young man in distress

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