Intimal sarcoma of the left atrium presenting with transient ischaemic attack — A case report and review of the literature

Abstract

A 31-year-old female with a history of polycystic ovary syndrome and two recent miscarriages presented with symptoms of a transient ischemic attack. Echocardiography to assess for possible embolic source identified a 4.8cm left atrial mass, presumed to be an atrial myxoma.At surgery the tumor was suspicious of malignancy. Histopathology revealed a heterogeneous hyper- and hypo-cellular spindle cell tumor showing mild atypia. Atrial myxoma markers were negative. The Ki67 proliferation factor was 30% and fluorescence in situ hybridization (FISH) analysis showed MDM2 amplification. Expert review confirmed the morphological, immunohistochemical, and FISH features to be of a cardiac intimal sarcoma.Recent improvements in imaging, surgery, and molecular testing have increased diagnoses of primary cardiac intimal sarcomas. Here we discuss the pathological and clinical implications of these rare atrial myxoma mimics.<Learning objective: Intimal sarcomas are rare, aggressive tumors typically associated with great vessels, however, molecular diagnoses of cardiac intimal sarcomas are increasing. Presentation is varied. Cardiac symptoms and emboli in young patients should prompt investigation with high levels of suspicion. Intimal sarcomas show MDM2 amplification with genetic aberration. Careful pre-operative planning to achieve clear surgical margins almost doubles life expectancy. Chemoradiotherapy may be beneficial. MDM2 and PDGFRa inhibitors are in development.>