SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Primary cardiac myxofibrosarcoma is a rare malignancy of mesenchymal origin which is associated with poor prognosis. Patients are generally asymptomatic in the earlier stages, however can present with life threatening hemodynamic instability. CASE PRESENTATION: Patient is a 25-year-old Caucasian male with history of asthma who presented with sudden onset of unresponsiveness, refractory shock and extensive bilateral pulmonary infiltrates. He was treated for bronchitis and pneumonia as outpatient on and off for two months prior to presentation. A transthoracic echocardiography showed large rounded mobile mass (5.5 cm by 4.5 cm) in left atrial cavity which was attached to anterior mitral leaflet causing severe mitral stenosis. CT showed extensive bilateral pulmonary consolidations. The patient was not deemed a candidate for surgical resection of the atrial mass. In the light of grave prognosis and failed aggressive medical therapy, family opted for comfort care measures. Autopsy of the left atrial mass showed low grade primary cardiac atrial myxofibrosarcoma. DISCUSSION: Myxofibrosarcoma most commonly occurs in the extremities but rarely can be seen in the heart. Primary cardiac myxofibrosarcoma is seen in the left atrium about 75% of the time [1]. Prognosis is poor because of its invasive nature and poor response to therapy including radiation, resection and chemotherapy [2]. Patients tend to be asymptomatic in the earlier stages. Clinical presentation is usually due to mass effect from the tumor. Tumor specifically located in the left atrium can cause obstruction of the mitral valve which manifests as symptoms of functional mitral stenosis leading to symptoms associated with heart failure. It can produce “tumor plop” (sound from tumor bounding around in the heart) and murmur similar to the mid-diastolic rumble of mitral stenosis [3]. This case is significant not only because of rarity of primary myxofibrosarcoma but also the clinical presentation and acute decompensation. Per our literature review, there are no reports of primary cardiac myxofibrosarcoma that presented with acute respiratory distress syndrome (ARDS). Our patient’s acute decompensation is likely synergistic result of cardiogenic shock from functional mitral stenosis (tumor mass effect) as well as underlying septic shock from pneumonia. CONCLUSIONS: Primary cardiac myxofibrosarcoma is a rare tumor which is usually detected when there are apparent hemodynamic changes form the mass effect, at which time treatment is limited given its extensive spread. Palliative care is generally the final outcome. Reference #1: Ujihira, Kosuke et al. “A case report of primary cardiac myxofibrosarcoma presenting with severe congestive heart failure” Journal of cardiothoracic surgery vol. 11,1 95. 7 Jul. 2016. Reference #2: Lazaros GA, et al. Primary myxofibrosarcoma of the left atrium: case report and review of the literature. Angiology. 2008;59(5):632–635. Reference #3: Vaideeswar, P. Butany, JW. (Feb 2008). "Benign cardiac tumors of the pluripotent mesenchyme". Semin Diagn Pathol. 25 (1): 20–8. DISCLOSURES: No relevant relationships by Bishal Bhandari, source=Web Response No relevant relationships by Cameron Koester, source=Web Response No relevant relationships by Abhishek Kalidas Kulkarni, source=Web Response No relevant relationships by Manjari Regmi, source=Web Response
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