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Background: Primary cardiac sarcomas are rare, highly aggressive tumors with nonspecific clinical presentations, making early diagnosis challenging. Intimal sarcoma, a subtype of undifferentiated sarcoma, is characterized by high metastatic potential and poor prognosis. Case Summary: A 23-year-old woman with no prior medical history presented to the emergency department with a 20-day history of progressive palpitations and dyspnea. Initial evaluation revealed tachycardia, hypotension, and a systolic mitral murmur. Laboratory tests demonstrated elevated D-dimer and high-sensitivity troponin I levels. Pulmonary thromboembolism was initially suspected; however, chest CT angiography revealed a left atrial mass, subsequently confirmed by transthoracic echocardiography as a heterogeneous mass causing dynamic mitral obstruction and moderate eccentric mitral regurgitation, with associated right ventricular dysfunction. The patient underwent emergency surgical resection, including removal of the left atrial appendage and partial atrial roof, followed by reconstruction of the interatrial septum. Histopathology confirmed high-grade intimal sarcoma with MDM2 overexpression. Postoperative management included adjuvant chemotherapy (Doxorubicin and Ifosfamide) and planned radiotherapy. Despite early intervention, follow-up MRI five months later revealed cerebral metastases, necessitating neurosurgical resection. Discussion and Conclusion: This case highlights the rapid progression and aggressive nature of cardiac intimal sarcoma, even in young patients with timely diagnosis and multidisciplinary care. Early recognition, prompt surgical intervention, and coordinated postoperative management remain critical to optimizing outcomes in this rare and high-risk disease.

Keywords: Sarcoma; Heart failure; Heart atria