A80-4-44 An Unusual Cause of Hemoptysis: Primary Left Atrial Spindle Cell Sarcoma With Pulmonary Vein Invasion

Introduction Primary cardiac spindle cell sarcomas are extremely rare, representing the least common cardiac tumor reported. We describe a case of a patient who presented to the hospital for hemoptysis and was found to have left atrial sarcoma with pulmonary vein extension. Case A 41-year-old male with no significant medical history presented with intermittent chest pain and 4 episodes of hemoptysis (1-2 tablespoons of fresh blood each episode) over 4 days. He denied fever, dyspnea, or recent sick contacts but reported feeling fatigued over the last 4 months. Vital signs and physical examination upon presentation were unremarkable, except for slightly elevated blood pressure at 148/80 mmHg. Laboratory workup was only relevant for an elevated D-dimer of 283 ng/ml. Chest Computed Tomography angiography (CTA) revealed a large filling defect in the left atrium, thought to represent a thrombus versus mass, with severe narrowing of the right inferior pulmonary veins, in addition to right-sided ground glass opacities (Fig.1). Given concern for an atrial thrombus, therapeutic anticoagulation with heparin was initiated with close monitoring for worsening hemoptysis. A cardiac magnetic resonance imaging (MRI) was obtained and revealed a large locally invasive left atrial mass extending into and occluding the right inferior pulmonary vein and partially invading the right superior pulmonary vein. The patient underwent a transcatheter biopsy, and the pathology was consistent with spindle cell malignancy with a differential diagnosis of intimal sarcoma. Surgical resection was deferred in favor of palliative chemotherapy. Discussion Primary cardiac tumors are exceedingly rare, with an incidence ranging from 0.017 to 0.019 percent (1). Among them, the least reported cardiac tumors are spindle cell sarcomas, which are associated with a poor prognosis (2). Because of the nonspecific symptoms and the rarity of these tumors, they are often difficult to diagnose at initial presentation. Transthoracic Echocardiogram (TTE) can be a useful screening tool, although cardiac CT and MRI provide a better assessment of tumor extent and invasion, including myocardial infiltration, compression of cardiac chambers, pericardial and great vessels involvement, and metastases (3). The prognosis of cardiac primary spindle cell sarcomas remains poor, with reported mean survival ranging from 3 months to 1 year. Management requires a multidisciplinary approach. Complete surgical resection offers the best chance of improved survival, although this is often not feasible due to extensive invasion. Adjuvant chemotherapy (often anthracycline-based) and/or radiotherapy are recommended to improve median survival, though long-term outcomes remain poor.