Primary Cardiac Synovial Sarcoma Research Articles

Complete resection of a giant intrapericardial cardiac synovial sarcoma

Synovial sarcoma of the heart is a rare tumor. Herein we would like to report a case of giant intrapericardial cardiac synovial sarcoma that originated from the right ventricle and grew outward near the diaphragm. After making adequate preoperative preparation, we performed the surgery as quickly as possible and resected the tumor completely. Based on the identification of the translocation on chromosome 18 rearrangement, the tumor can be diagnosed as a primary cardiac synovial sarcoma. Through this study, we aim to afford more information about cardiac synovial sarcomas as well as a reference for similar cases.

Primary cardiac synovial sarcoma: a clinicopathological analysis of five cases

Objective: To assess the clinicopathological features, immunophenotype, molecular characteristics and differential diagnosis of primary cardiac synovial sarcoma (PCSS). Methods: Five cases of PCSS were collected at Guangdong Provincial People's Hospital from 2008 to 2023, and their clinicopathological features were summarized. Immunohistochemical staining, fluorescence in-situ hybridization (FISH) and next-generation sequencing (NGS) were performed, and relevant literatures were reviewed. Results: The cases were found in four males and one female, ranging in ages from 16 to 51 years (median 30 years). Two cases were located in the pericardium, two in the right ventricle, and one in the left ventricle. Follow-up data were available in four cases. All the four patients died of disease at 3, 7, 13 and 26 months, respectively, after diagnosis. The tumor maximum diameter ranged from 6.0 to 14.0 cm in (mean 10.0 cm). Microscopically, three cases were monophasic and two cases were biphasic. Immunohistochemically, all cases were immunoreactive for EMA, vimentin, bcl-2 and CD56. The tumor cells were variably positive for pan-cytokeratin, SS18-SSX, SOX2, TLE1, CD99, synaptophysin, calretinin and calponin. FISH showed the presence of SS18 rearrangement in all the cases. NGS detected SS18-SSX gene fusion in three cases (SS18-SSX1 in one and SS18-SSX2 in two). Conclusions: PCSS is an exceedingly rare neoplasm, and should be distinguished from other various malignant epithelial and mesenchymal tumors. The clinical history, histopathological and immunohistochemical features, and molecular findings are all essential to the definitive diagnosis of PCSS.

Primary Cardiac Synovial Sarcoma Presents as a Ventricular Mass and Renal Infarction

Primary Cardiac Synovial Sarcoma Presents as a Ventricular Mass and Renal Infarction

Endobronchial Ultrasound-guided Transbronchial Needle Aspiration of Primary Cardiac Synovial Sarcoma of the Left Ventricle.

Endobronchial Ultrasound-guided Transbronchial Needle Aspiration of Primary Cardiac Synovial Sarcoma of the Left Ventricle.

Massive primary cardiac synovial sarcoma of the left atrium: a case report

BackgroundSynovial sarcomas are tumors typically located in the extremities and characterized by a t(X;18)(p11.2;q11.2) chromosomal translocation. With only around 100 cases reported in the literature, cardiac synovial sarcomas are extremely rare.Case presentationWe describe a case of a 59-year-old male who presented to his primary care physician with chest pain, palpitations, and dyspnea and was diagnosed with atrial flutter. Following atrial ablation, a transthoracic echocardiogram incidentally revealed a 5.5 × 5.0 cm heterogeneous mass. Further workup found a heterogeneous mass with mild fluorodeoxyglucose uptake that was abutting the left atrium, left ventricle, and left pulmonary veins. The tumor was resected and confirmed to be a monophasic synovial sarcoma with a SS18-SSX gene fusion. Four months post-operative, the patient had recovered well from surgery. He is currently undergoing concurrent radiation and chemotherapy.ConclusionsDue to the rarity of this tumor, guidelines on diagnosis and treatment come only from case reports. Our case describes a primary cardiac synovial sarcoma arising from the left atrium in the atrioventricular groove in which diagnosis of atrial flutter preceded detection of the mass.

A Rare Case of Primary Cardiac Synovial Sarcoma with Thromboctopenia

Primary cardiac tumors are of rare presentation. We present a case of primary cardiac synovial sarcoma of the right atrium admitted to our hospital. An initial diagnosis of right atrial myxoma or hydatid cyst was made based on echocardiographic and radiological features. Intraoperatively, an irregular mass was excised, and histopathologically, it was reported as monophasic synovial sarcoma. Immunohistochemistry was positive for TLE-1, BCL-2, and MIC-2.

Primary cardiac synovial sarcoma: a clinicopathological, immunohistochemical, and molecular genetics study of five clinical cases

BackgroundPrimary cardiac synovial sarcoma was an exceedingly rare tumor that less reported. The study investigated the clinicopathologic, immunohistochemical, and molecular features of primary cardiac synovial sarcoma. MethodsA total of five cardiac synovial sarcoma cases were assessed and reviewed using H&E, immunohistochemical and fluorescence in situ hybridization staining methods. Clinicopathological data were retrospectively analyzed and followed up. ResultsThe cases occurred in four males and one female ranging in age from 23 to 48 years (mean, 32 years). The tumors were grossly large and solid (7.4–13.7 cm; mean 8.6 cm). Microscopically, clinical cases were biphasic (n = 2) and monophasic (n = 3) types and were diffusely immunoreactive for EMA, vimentin, and BCL-2. All cases demonstrated SS18 rearrangement by fluorescence in situ hybridization staining. Clinically, three patients died within 1 year after surgery, while one patient had bone metastasis and still carried the disease. One last patient underwent a heart transplant and survived without evidence of the disease. ConclusionCardiac synovial sarcoma was an aggressive tumor whose differentiation may be a continuous and complex morphologic spectrum. SS18 rearrangement demonstration by fluorescence in situ hybridization was decisive in our study for differential diagnosis of cardiac synovial sarcoma and other tumors. Cardiac synovial sarcoma usually endured poor survival rates. Patients in advanced stages may undergo heart transplantation as a means of improving their survival rates.

Primary cardiac synovial sarcoma: A review correlating outcomes with surgery and adjuvant therapy.

Cardiac synovial sarcoma (CSS) is an extremely rare malignant tumor with a severe prognosis, due to frequent relapses and metastases. To obtain useful information for treatment protocols, we analyzed survival and therapy data from the cases reported in the literature. A search of MEDLINE was performed throughout December 2018. Using key words relating to primary CSS, we collected from the literature a total of 97 cases, mainly consisting of single case reports. To identify predictors of overall survival, statistical analyses were performed on a selected cohort of 55 patients for whom relevant clinicopathological data were available, including surgery and adjuvant therapy. The univariable analysis revealed that patients in their first three decades of life have better overall survival. The univariable analysis also showed that patients not receiving adjuvant chemotherapy are at increased risk of death. In the multivariable analysis, tumor resection and chemotherapy are factors significantly improving overall survival. The survival of patients withCSS is positively influenced by a young patient's age and greatly improved by the administration of chemotherapy, even in the absence of tumor resection.

Primary cardiac synovial sarcoma that was continuous with the mitral valve caused severe thrombocytopenia: a case report

BackgroundPrimary cardiac sarcomas are exceedingly rare, and they commonly result in nonspecific constitutional symptoms such as shortness of breath, weight loss, and anaemia-related fatigue and malaise. However, thrombocytopenia has very rarely been reported in association with cardiac tumours, either benign or malignant. We report one case of primary cardiac synovial sarcoma continuous with the mitral valve, which was accompanied by severe thrombocytopenia, and the platelet counts returned rapidly to a normal range early after tumour excision and without any special therapies.Case presentationA 52-year-old male diagnosed with atrial myxoma with severe thrombocytopenia was admitted to our hospital. Blood analysis showed severe thrombocytopenia, whereas erythrocyte and leucocyte counts were within the normal range. A 50 × 35 mm mobile mass continuous with the mitral valve was found to be present in the left atrium upon echocardiography. Bone marrow aspiration and related examinations excluded thrombocytopenia caused by haematologic malignancies. The patient received a platelet transfusion, but platelet counts decreased quickly. Glucocorticoid therapy and immunoglobulin transfusion were also used, but were ineffective. Although the operation risk was high, tumour resection was performed via a median sternotomy with a cardiopulmonary bypass system. The postoperative pathological diagnosis was biphasic cardiac synovial sarcoma. Surprisingly, the platelet counts returned rapidly to a normal range early after tumour excision without any special therapies. The disappearance of the tumour from the annular region was confirmed on transthoracic echocardiography 6 days after surgery, and an FDG-PET scan performed 8 days after surgery showed no abnormal accumulation. Unfortunately, the patient died suddenly 6 months later without unknown cause.ConclusionsWe report that a rare primary cardiac synovial sarcoma case continuous with the mitral valve caused severe thrombocytopenia; this provides further support for the awareness and diagnosis of primary cardiac synovial sarcoma. We also highlight that thrombocytopenia might be one rare symptom of a solid cardiac tumour but need more cases for support.

心臓原発滑膜肉腫の 1 例

A man in his 40’s was found to have a right atrial mass, bilateral pulmonary nodules, and pleural effusion. Pleural fluid cytology revealed clusters showing papillary or glandular structure resembling adenocarcinoma, composed of columnar to short-spindle cells. CT-guided needle lung biopsy showed dense proliferation of spindle to oval cells with acinar and glandular structures. The tumor cells were positive for CAM5.2, vimentin, and bcl-2 in immunocytochemistry and immunohistochemistry. RT-PCR detected SYT-SSX1 fusion transcripts in paraffin-embedded tissue. No primary lesions were found except for the cardiac tumor, and a diagnosis of primary cardiac synovial sarcoma was made. The epithelioid clusters in the pleural effusion are a very interesting cytologic feature of synovial sarcomas.

Primary cardiac synovial sarcoma

Approximately 10% of soft tissue sarcomas are synovial sarcomas, and 90% of these occur in the extremities. Among the primary tumors in the heart, 25% are malignant. Primary synovial sarcoma of the heart is an extremely rare entity. A myriad of investigations such as histopathology, immunohistochemistry, electron microscopy, and molecular genetic techniques are required for confirmation of the diagnosis. The tumor is nearly always lethal, but surgical resection with chemotherapy may prolong the life of the patient. We describe the case of a young patient with a primary synovial sarcoma arising from the right ventricle.

Primary Cardiac Synovial Sarcoma Arising from the Pulmonary Valve

Primary Cardiac Synovial Sarcoma Arising from the Pulmonary Valve

Primary Cardiac Synovial Sarcoma

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.

Primary cardiac synovial sarcoma demonstrated by 3D transesophageal echocardiogram

A 26-year old male patient presented with a 9 month history of progressive shortness of breath, easy fatigability, abdominal distension, and lower limb oedema. He had large bilateral pleural effusion on the chest X ray, right-axis deviation and incomplete right bundle branch block on the ECG. Transthoracic echocardiography and transoesophageal echocardiography (TEE) 2D and 3D showed …

Primary cardiac synovial sarcoma: A case report and literature review

Primary cardiac synovial sarcoma is a rare disease. A 51-year-old man visited our hospital with the chief complaint of palpitations and shortness of breath while exercising. Copious bloody pericardial effusion and a multicystic intrapericardial tumor were detected. A primary cardiac malignant tumor was suspected, an open-chest tumor resection was performed with the objectives of diagnosis and treatment. Histologically, the tumor cells were uniformly spindle-shaped with an ovoid or oval nucleus, they had proliferated in fascicular fashion. In addition myxoid degeneration, a hemangiopericytomatous vascular pattern and pseudorosette formation were seen in some areas of the tumor. Based on the histopathological and immunohistochemical findings and reverse transcription polymerase chain reaction detection of SS18-SSX1 fusion transcripts, a monophasic fibrous type synovial sarcoma was diagnosed. Postoperative radiation therapy was administered and there had been no recurrence 9 months after the surgery.

Re: Primary cardiac synovial sarcoma

Synovial sarcoma (SS) represents only a small proportion of the soft-tissue sarcomas. In the World Health Organization classifications of cardiac tumors, SS was defined as a biphasic tumor composed of spindled and epithelioid areas, characterized by X;18 chromosomal translocations [1]. More than 80% arise in the deep soft tissue of extremities, especially around the knee, and the tumor frequently arises adjacent to joints or tendon sheaths. Histologically, SS is biphasic (BSS), characterized by epithelial and spindle cell components, or monophasic (MSS), characterized by the spindle cell component. Up to 50% of SS recur, usually within 2 years, but sometimes up to 30 years after diagnosis [2]. Quite a few large-population prognostic factor studies of SS have been published, but results have varied from report to report. Cardiac sarcomas often manifest clinical features, such as dyspnea and palpitation, that are similar to myxomas [3, 4]. Some patients may experience a long term before onset of symptoms, especially when they arise from the pericardium [3]. Therefore, these sarcomas are usually larger than their superficial counterparts. Owing to the poor understanding and indefinite conclusions for them, the correct preoperative diagnoses are always difficult to make, although varieties of examinations are performed to identify the lesion. Moreover, most cardiac sarcomas can seldom be completely removed, and are thus susceptible to recur. Despite the publication of numerous case reports and several series of cardiac sarcomas with long-term follow-up, prognostic factors are still being debated [5‐7]. Primary cardiac SS (PCSS) is an extremely rare entity, which was first described in 1978 by McAllister and Fenoglio [8] in a 30-year-old woman. Since then, a series of isolated case reports have been published. An analysis of reviews shows that PCSS is calculated to account for approximately 4.2% (1 of 117 [8, 9] ,2o f 75[10] ,1o f 24[6], 1o f 14[11], 3 of 24 [7], 2 of 17 [5], 2 of 14 [12] ,a nd 1o f 2 [13], respectively; mean, 4.2%) of the primary cardiac sarcomas. In previous case reports with literature reviews, researchers have described some clinical features, although, the characterizations, especially adjunctive diagnostic devices and treatment, remain poorly acknowledged. To the best of our knowledge, there are few large population studies of cardiac sarcomas and no accumulated knowledge of PCSS to date. We present an analysis of 60 patients with PCSS derived from 54 isolated articles in an effort to establish definite clinical, pathologic, treatment and outcome patterns of PCSS and to develop a rationale for prognostication in this disease.

Primary cardiac synovial sarcoma

Synovial sarcoma is a rare entity and accounts for <1% in all primary cardiac tumors. This is typically a highly aggressive tumor and survival is usually less than ninemonths in this location, even with surgery and adjuvant chemoradiation. Primary cardiac synovial sarcoma has rarely been reported in the literature. We report a recent case from the Mayo Clinic, Rochester, Minnesota. The patient is still alive and with some recurrence of tumor but without significant symptoms 22months after primary surgery.

Primary cardiac synovial sarcoma

Synovial sarcomas are rare tumors and account for only 3% of cardiac malignancies. In this report, we present a case of a primary synovial sarcoma of the left ventricle.

Primary Cardiac Synovial Sarcoma: A Case Report and Brief Review of the Literature

Synovial sarcoma comprises approximately 10% of all soft tissue sarcoma diagnoses; a primary synovial sarcoma of the myocardium is exceedingly rare. There have been very few cases reported in the literature thus far. With the identification of the characteristic and diagnostic chromosomal abnormality t(X;18), this may become an increasingly recognized entity. Our report adds to the limited published cases of primary cardiac synovial sarcoma with the characteristic t(X;18). Further elucidation of the effects of this translocation on the cell cycle may lead to directed therapies in the future.

Primary synovial sarcoma of the mitral valve

Primary cardiac synovial sarcoma is a rare malignancy, comprising approximately 5% of cardiac sarcomas and less than 0.1% of all primary cardiac tumors. Herein, we describe a synovial sarcoma arising on the anterior mitral leaflet in a 66-year-old man. The histopathologic diagnosis was supported by the immunohistochemical profile and the presence of the SYT–SSX1 fusion transcript identified by molecular genetic studies.