Primary spindle cell sarcoma of the heart treated with carbon-ion radiotherapy

INTRODUCTION: Primary cardiac sarcoma is a rare malignant cardiac neoplasm with a poor prognosis. No evidence-based guidelines exist regarding surgical treatment management, although some case reports have been published. We report the outcome of a patient with spindle cell sarcoma of the heart treated postoperatively with carbon-ion radiotherapy and chemotherapy. PRESENTATION OF CASE: A 16-year-old female patient was noted to have abnormal electrocardiogram results for 2 consecutive years at a high school medical check. She was referred to our hospital and a cardiac tumor in the left atrium was suspected. The preoperative diagnosis was myxoma, but abnormalities were also observed macroscopically during surgery, and a diagnosis of spindle cell sarcoma was made by rapid intraoperative pathological analysis. We resected the tumor as much as possible with surgery, but the tumor in the left lower pulmonary vein could not be respected. We performed carbon-ion radiotherapy postoperatively. She experienced recurrence 1 year later and tumor growth 2 years later. We considered additional surgical treatment, but eventually selected chemotherapy. She died 4 years and 7 months after initial surgery. CONCLUSION: Spindle cell sarcoma is a rare disease with a poor prognosis. Although no optimal treatment method has been established, in the present case, incomplete surgical resection and postoperative carbon-ion radiotherapy were performed, yielding a relatively long survival period.