Our patient is unique with regard to the concomitant manifestation of the cerebral and cardiac leiomyosarcomas. Most likely, the cerebral manifestation should be regarded as a metastasis; the immunohistochemical profiles and the clinical history, however, also allow different interpretations.The best available therapy for undifferentiated leiomyosarcoma consists of early detection and radical surgical removal. Although the cardiac tumor in our patient showed no relapse after complete removal, the cerebral tumor recurred several times. The repeated craniotomies within short intervals can be discussed controversially. Nevertheless, because the patient was asymptomatic apart from occasional seizures, we decided to offer him these options as live-saving procedures.Concomitant cardiac and cranial leiomyosarcoma seems to be particularly aggressive, and only diligent follow-up examinations and early surgical intervention will make long-term survival possible. There is a need for more efficacious adjuvant therapy.

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