Cardiac leiomyosarcoma

Abstract

Leiomyosarcoma is an aggressive mesenchymal tumor derived from smooth muscle cells accounting for up to 30% of all sarcomas. Cardiac impairment is often a character of its metastatic involvement or, less likely, the heart can be the only organ targeted by this rare tumor disease. Patients with established diagnosis of cardiac leiomyosarcoma present with various symptoms relating to the tumor disease itself or may have symptoms associated with the location and extent of the tumor mass in the heart. Imaging modalities used in routine practice are of great importance in establishing diagnosis, assessing tumor disease staging and in evaluating the response to the selected therapeutic modality. Unfortunately, the long asymptomatic course of cardiac leiomyosarcoma and the aggressive behavior of this tumor have made available therapeutic interventions at presentation of limited efficacy. While the role of chemotherapy is not well established and considering that leiomyosarcoma is known to have a low radiosensitivity, surgical resection of the tumor mass is the treatment option with proven positive impact on patient's survival, symptoms and quality of life. In general, leiomyosarcoma is difficult to treat and has a poor prognosis. In this article we review cardiac leiomyosarcoma, its prevalence, clinical manifestation, the diagnostic modalities used for establishing the diagnosis of leiomyosarcoma, current treatment options available for the management of this disease and patient prognosis and outcomes.