Primary high-grade myofibroblastic sarcoma arising from the pericardium
Seiji Koga1, Satoshi Ikeda, Jungo Urata
1Second Department of InternalMedicine, Nagasaki University School of Medicine, 1-7-1 Sakamoto,Nagasaki 852-8501, Japan.
Insights
This study reports a rare case of primary high-grade myofibroblastic sarcoma of the pericardium in a 62-year-old man. The patient successfully responded to surgical excision, chemotherapy, and radiotherapy, with no signs of recurrence or metastasis.
Area of Science:
- Cardiovascular Pathology
- Surgical Oncology
- Medical Oncology
Background:
- Primary pericardial sarcomas are exceptionally rare malignancies.
- Cardiac tamponade is a critical presentation requiring urgent intervention.
Observation:
- A 62-year-old male presented with cardiac tamponade due to a large pericardial tumor.
- Imaging revealed massive pericardial effusion and a tumor attached to the left ventricle.
- Histopathology confirmed high-grade myofibroblastic sarcoma.
Findings:
- The patient underwent surgical tumor excision.
- Adjuvant therapy included doxorubicin, ifosfamide, and radiotherapy due to local recurrence.
- At 6 months post-treatment, the patient showed no disease progression or distant metastases.
Implications:
- This case may represent the first documented instance of primary high-grade myofibroblastic sarcoma of the pericardium.
- Multimodal treatment involving surgery, chemotherapy, and radiotherapy can be effective.
- Further research into rare pericardial tumors is warranted.
Abstract:
Primary pericardial sarcomas are very rare. A 62-year-old Japanese man presented with cardiac tamponade. Echocardiography, computed tomography and magnetic resonance imaging revealed massive pericardial effusion and a large tumor in the pericardial cavity, attached to the pericardium of the left ventricular posterolateral free wall. Surgical excision of the tumor was performed and histopathological and immunohistochemical examinations identified high-grade myofibroblastic sarcoma. Because of local recurrence soon after surgery, the patient received adjuvant chemotherapy, including doxorubicin and ifosfamide, and subsequent radiotherapy. As of 6 months after completing radiotherapy, the patient was alive and no disease progression or distant metastases were evident. This may be the first report of primary high-grade myofibroblastic sarcoma arising from the pericardium.
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