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

Teng, Fei; Chen, Dong; Li, Yanwei; Fang, Wei; Yang, Shi; Shang, Jianfeng; Liu, Gonghan; Cui, Yingxue; Zhao, Yanli; Lian, Guoliang

doi:10.1016/j.carpath.2020.107286

Cited by 9 publications

(4 citation statements)

References 33 publications

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“…SS is typically located in the extremities, but it is occasionally found in the head and neck, the eye sockets, the lungs, the chest wall, and the mediastinum. Primary PSS is extremely rare, with only in a few dozen cases reported in the literature (7). PSS has a male preponderance; the median age of diagnosis is 32 years, and the mean tumor size is 10.6 cm (8).…”

Section: Discussionmentioning

confidence: 99%

Multimodal imaging features of primary pericardial synovial sarcoma: a case report

Jin

Zhang

et al. 2023

Front. Oncol.

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BackgroundPrimary pericardial synovial sarcoma is an extremely rare malignant tumor, and affected patients have a poor prognosis. Only a few cases have been reported in the literature.Case summaryA 34-year-old man was admitted to our hospital with chest tightness and a cough. An echocardiogram revealed a heterogeneous mass with a large pericardial effusion. Further computed tomography (CT) of the chest and cardiac magnetic resonance imaging (CMRI) demonstrated an irregular pericardial mass abutting the left atrium and left ventricle and invading the mediastinal structures. Pathology results showed that the tumor was a monophasic synovial sarcoma. The patient underwent chemotherapy and survived for 17 months.DiscussionMany cardiac tumors are clinically asymptomatic or nonspecific, and they are frequently detected or diagnosed at an advanced stage of the disease. Multimodal cardiac imaging facilitates the detection and assessment of cardiac tumors. In particular, CMRI is considered as a superior imaging tool, because it provides high tissue contrast and can detect invasion of the myocardium. We describe the clinical details and multimodal imaging features of a rare primary pericardial synovial sarcoma, hoping to provide guidance for the diagnosis of similar cases in the future.

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Section: Discussionmentioning

confidence: 99%

Multimodal imaging features of primary pericardial synovial sarcoma: a case report

Jin

Zhang

et al. 2023

Front. Oncol.

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“…PCSS is extremely rare, representing less than 5% of all primary cardiac sarcomas [ 3 ]. To date, around 100 cases have been reported in the literature [ 4 , 5 ]. PCSS has a 3:1 male predominance and a mean age of diagnosis in the fourth decade of life [ 3 , 4 ].…”

Section: Discussionmentioning

confidence: 99%

“…Adjunctive radiotherapy and chemotherapy are associated with greater survival, and the most commonly used chemotherapy regimen is ifosfamide and doxorubicin [ 3 , 4 ]. Teng et al [ 5 ] reported on the use of heart transplantation to treat a case of a PCSS invading the ventricular wall. However, it remains controversial whether transplantation prolongs survival in patients with cardiac sarcomas [ 7 , 8 ].…”

Section: Discussionmentioning

confidence: 99%

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

Zhou

Halub

Gross

et al. 2022

J Cardiothorac Surg

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Background Synovial 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 presentation We 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. Conclusions Due 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.

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“…Cases of intraprostatic synovial sarcoma have also been reported in the literature. 7 – 10 The overall prognosis of primary metastatic synovial sarcoma remains poor, with a metastases rate of 25%, and a 48% rate of recurrence. 11 , 12 The standard approach to treating synovial sarcoma is wide surgical excision combined with radiotherapy, with or without (neo)adjuvant chemotherapy, as appropriate.…”

Section: Discussionmentioning

confidence: 99%

A rare case of synovial sarcoma with lung, heart and adrenal gland metastasis: a caution for patients and clinicians

Jiang

Zhu

et al. 2021

J Int Med Res

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A 68-year-old man presented to the Urology Clinic, West China Hospital, Chengdu, with a suspected right adrenal gland mass that had persisted for two months. He had no associated lumbodynia, dizziness or palpitation. Abdominal computed tomography (CT) revealed an uneven density and contrast-enhanced oval-like mass with smooth edges in the right adrenal gland. Laparoscopic right adrenal gland resection followed by histopathology confirmed a diagnosis of metastatic synovial sarcoma. The patient had a history of synovial sarcoma on the right upper leg 3 years previously that was surgically treated, but he had not undergone further treatment. Approximately 1.5 years later, he had undergone surgery for heart and lung metastasis from the synovial sarcoma of the thigh. At 5 months following laparoscopic right adrenal gland resection, abdominal CT showed a significant sign of right adrenal recurrence, and targeted therapy of 12 mg oral anlotinib, daily, was initiated. This relatively rare but alarming case highlights the importance of patient understanding and compliance to treatment.

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

Cited by 9 publications

References 33 publications

Multimodal imaging features of primary pericardial synovial sarcoma: a case report

Multimodal imaging features of primary pericardial synovial sarcoma: a case report

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

A rare case of synovial sarcoma with lung, heart and adrenal gland metastasis: a caution for patients and clinicians

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