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Mediastinal monophasic synovial sarcoma with pericardial extension causing hemodynamic instability
Habib R Khan1, Mohammad I Ansari1, Andrew P Thain2
1Nottingham City Hospital, Nottingham, UK.
Oxford Medical Case Reports
|May 17, 2018
Summary
A rare cardiac tumor, primary monophasic synovial sarcoma, presented as a mediastinal mass causing cardiac tamponade. Despite treatment, the patient experienced a poor prognosis, highlighting the aggressive nature of this rare condition.
Area of Science:
- Cardiology
- Oncology
- Thoracic Surgery
Background:
- Primary cardiac tumors are rare, with synovial sarcoma being an exceptionally uncommon diagnosis.
- Mediastinal masses can present with vague symptoms, leading to delayed diagnosis.
- Cardiac tamponade is a life-threatening condition caused by fluid accumulation around the heart.
Observation:
- A 46-year-old male presented with dyspnea, cough, and weight loss.
- Imaging revealed a large extra-cardiac mass compressing the right ventricular outflow tract, causing cardiac tamponade.
- A mediastinal mass invading the pericardium was identified on CT scan.
Findings:
- Biopsy confirmed primary monophasic synovial sarcoma.
- Initial response to chemotherapy, radiotherapy, and anti-inflammatories was brief.
- Tumor progression and refractoriness to treatment were observed.
Implications:
- This case highlights the aggressive nature of primary mediastinal synovial sarcoma.
- Challenges in surgical intervention due to tumor location and pericardial involvement are noted.
- The limited treatment response underscores the need for novel therapeutic strategies for this rare malignancy.
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