Abstract
AIM: Primary cardiac intimal sarcoma is a rare entity with a poor prognosis, often requiring distinction from other undifferentiated cardiac sarcomas. CASE PRESENTATION: A 55-year-old female presented with symptoms of heart failure. Imaging identified dual left atrial masses compromising mitral inflow and pulmonary venous return. Surgical excision was performed, and morphological analysis revealed a high-grade spindle cell sarcoma. Crucially, the diagnostic dilemma was resolved through molecular testing; immunohistochemistry showed diffuse overexpression of MDM2 and CDK4, and fluorescence in situ hybridization (FISH) confirmed the amplification of the MDM2 and CDK4 loci. CONCLUSION: This case illustrates the diagnostic utility of MDM2 and CDK4 as specific biomarkers for cardiac intimal sarcoma. It reinforces the necessity of a multidisciplinary approach involving advanced molecular pathology to ensure accurate classification and appropriate management of rare cardiac malignancies.