Abstract

BackgroundUndifferentiated pleomorphic sarcoma is a very rare and aggressive type of primary cardiac tumors. Most cardiac sarcomas result in rapid growth and quick death. According to different sources the median survival is typically 6 to 12 months. We are presenting a case of primary cardiac sarcoma with 26 months disease free survival following cytoreductive surgery and chemotherapy.Case presentationA 48-year-old woman with progressing symptoms of dyspnea and palpitations for over 2 months was referred to a cardiologist. With the help of echocardiography and cardiovascular magnetic resonance cardiac sarcoma was suspected. Open biopsy and cytoreductive surgery were performed, complete resection of the tumor was not possible. Histology revealed undifferentiated pleomorphic sarcoma. Seven cycles of chemotherapy with Doxorubicine and Ifosfamide were completed. Cardiovascular magnetic resonance revealed a complete response – only signs of fibrosis without any signs of tumor were visible. Follow ups with echocardiography, cardiovascular magnetic resonance and chest, abdomen and pelvic computed tomography is performed every 3 months. Twenty-six months from initial diagnosis the patient is still free of recurrence of tumor with no compromises of the quality of life.ConclusionStandard chemotherapy together with cytoreductive surgery can have a complete response effect in undifferentiated pleomorphic sarcoma with unusual long-term survival.

Highlights

  • Undifferentiated pleomorphic sarcoma is a very rare and aggressive type of primary cardiac tumors

  • According to a study conducted in Cleveland Clinic Undifferentiated pleomorphic sarcoma (UPS) were diagnosed in 12% of all cardiac sarcoma cases [3]

  • Echocardiography and Cardiovascular magnetic resonance (CMR) were performed to assess the results of chemotherapy treatment and revealed a complete response – only signs of fibrosis without any signs of tumor were visible in CMR images (Fig. 4)

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Summary

Conclusion

Standard chemotherapy together with cytoreductive surgery can have a complete response effect in undifferentiated pleomorphic sarcoma with unusual long-term survival.

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