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Updated: Sep 9, 2025

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Published on: September 15, 2017
Primary Cardiac Sarcoma: A Rare Case With Superior Vena Cava Syndrome
Ci Xin Ong1, Zi Hui Tan2, Samuel Sherng Young Wang3
1Duke-NUS Medical School, Singapore, Singapore; ong.ci.xin@u.duke.nus.edu.
Primary cardiac sarcomas are rare tumors. This case highlights a pericardial spindle cell sarcoma presenting with superior vena cava syndrome, emphasizing the poor prognosis despite multimodal treatment.
Area of Science:
- Cardiovascular Pathology
- Oncology
- Thoracic Surgery
Background:
- Primary cardiac tumors, especially sarcomas, are exceedingly rare.
- Limited data exists on the diagnosis and management of these tumors.
- Early stages are often asymptomatic, with later presentation of diverse symptoms.
Purpose of the Study:
- To report a rare case of primary cardiac spindle cell sarcoma.
- To illustrate the diagnostic challenges and clinical presentation.
- To discuss the management and prognosis of this rare malignancy.
Main Methods:
- A case report of a 59-year-old patient with a cardiac mass.
- Clinical presentation included symptoms of superior vena cava syndrome.
- Diagnostic investigations confirmed a primary pericardial spindle cell sarcoma.
Main Results:
- The patient presented with odynophagia, dysphagia, hoarseness, and constitutional symptoms.
- Imaging and pathological examination identified a malignant spindle cell sarcoma of the pericardium.
- Despite aggressive treatment, the tumor exhibited poor prognosis.
Conclusions:
- Primary cardiac spindle cell sarcoma is a rare and aggressive malignancy.
- Multimodal treatment including chemotherapy, radiotherapy, and surgery offers limited survival benefit.
- Further research is needed to improve outcomes for these rare tumors.
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