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Combined treatment for a large primary cardiac sarcoma: a case report
Shinji Akishima1, Akito Imai2, Yoshiharu Enomoto2
1Department of Cardiovascular Surgery, Ibaraki Prefectural Central Hospital, 6528 Koibuchi, Kasama-shi, Ibaraki, 309-1793, Japan. akishima-ths@umin.ac.jp.
Surgical Case Reports
|June 19, 2019
Summary
A rare cardiac undifferentiated pleomorphic sarcoma (UPS) case in a shock patient showed 2-year survival after emergency surgery and combined therapy. This highlights potential treatment strategies for this aggressive primary cardiac tumor.
Area of Science:
- Cardiology
- Oncology
- Surgical Pathology
Background:
- Primary cardiac tumors are rare, with undifferentiated pleomorphic sarcoma (UPS) presenting a particularly poor prognosis due to high recurrence and invasion rates.
- Undifferentiated pleomorphic sarcoma (UPS) of the heart is exceptionally uncommon, often associated with a grave outlook.
Purpose of the Study:
- To report a rare case of primary cardiac undifferentiated pleomorphic sarcoma (UPS) causing shock.
- To describe the successful emergency surgical management and subsequent combined therapy for a patient with a left atrial UPS.
Main Methods:
- A 41-year-old male presented with acute respiratory distress and hemoptysis, diagnosed with left heart failure due to a large left atrial mass.
- Emergency open-heart surgery was performed for tumor resection, with pathological diagnosis of undifferentiated pleomorphic sarcoma (UPS) invading the left atrium.
- The patient received adjuvant combined therapy, including proton beam radiotherapy and chemotherapy with molecularly targeted drugs.
Main Results:
- The patient, diagnosed with left atrial undifferentiated pleomorphic sarcoma (UPS), underwent emergency tumor resection.
- Following combined therapy, the patient experienced 2 years of disease-free survival, returning to work without active local recurrence or distant metastasis.
Conclusions:
- This case demonstrates that emergency surgery followed by combined therapy can improve outcomes for patients with primary cardiac undifferentiated pleomorphic sarcoma (UPS) presenting in shock.
- Aggressive surgical intervention and multimodal treatment, including radiotherapy and targeted chemotherapy, may offer a survival benefit for this rare and aggressive cardiac malignancy.
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