Management of recurrent left atrial leiomyosarcoma

Primary cardiac leiomyosarcoma is an extremely rare malignancy often misdiagnosed as benign atrial myxoma. A 55-year-old male presented with syncope from a recurrent left atrial mass, eight months after initial resection for a presumed myxoma at another center. Redo median sternotomy and macroscopic tumor resection confirmed leiomyosarcoma with R1 margins. Despite adjuvant ifosfamide, mesna, doxorubicin chemotherapy, early cardiac magnetic resonance imaging demonstrated local recurrence. A third surgical resection was performed, combined with left atrial cryoablation and mitral annuloplasty. At 6-month follow-up, the patient remains alive without recurrence or metastasis. Aggressive repeated resections with local cryoablation may provide viable disease control in recurrent cardiac leiomyosarcoma.