Abstract
INTRODUCTION: Primary cardiac intimal sarcoma (CIS) is a rare and highly malignant soft tissue tumor associated with an extremely poor prognosis. To date, there are no established treatment guidelines for CIS in clinical practice. CASE PRESENTATION: A 44-year-old man was diagnosed with left atrial intimal sarcoma. Forty-two days after radical surgical resection, he developed local recurrence accompanied by brain and gluteus medius metastases. The patient first received emergency radiotherapy for intracranial metastases, followed by six cycles of systemic chemotherapy with ifosfamide and epirubicin, combined with targeted therapy using anlotinib. Ultimately, he achieved an overall survival (OS) of 17 months. CONCLUSION: To our knowledge, this is the first report describing the therapeutic efficacy of anlotinib in CIS. This case highlights the potential role of anlotinib as a promising therapeutic option for this rare and aggressive malignancy.