P1697 A rare case: primary cardiac sarcoma in the left and right atrium presenting with dispnea due to mitral valvular obstruction
Authors: Akturk S , Tan T S , Mammadov M , Dincer I , Erol C 
Published: January 2020

Introduction Primary cardiac tumors are extremely rare; the incidence is approximately 0.001% to 0.03%. Malignant tumors account for 25% of primary cardiac tumors, and among those, sarcomas are the most prevalent. We report a case of primary cardiac sarcoma presenting with dyspnea due to mitral valvuler obstruction.

Case Report A 41-year-old woman was admitted to the hospital with dyspnea for 8 months without any obvious causes. She was healthy previous and without family history. Physical examination was normal. Thorax computed tomography (CT) scan was performed with the doubt of pulmonary disease. A 1x1 cm hypodense nodule was detected in superior segment of the right lower lobe lung. Positron emission tomography-computed tomography (PET-CT) revealed a 1*1 cm nodule in the lung. And also increased 18F-fluorodeoxyglucose uptake was observed in the left scapula, left iliac wing and right 4th rib, likely due to metastasis. Biopsy from the lesion of iliac wing was performed. Pathologic examination was primarily compatible with the malign mesenchymal tumor and sarcoma infiltration; but malignant epithelial tumor and metastasis of carcinoma could not be ruled out. Magnetic resonance imaging of the lung was performed to find the origin of the metastatic tumor. A 8,6 x 5,3 x 5,1 cm mass filling the right and left atria was detected.It was extending from the right atrium to the superior vena cava and also from the left atrium to the right inferior and superior pulmonary venules. The image of mass was compatible with sarcoma. Transthoracic echocardiography was performed. A mass in the left and right atrium was detected. It was filling the left atrium. Due to the mass, the maximum mitral gradient was 21 mm Hg and the mean mitral gradient was 10 mm Hg, as if there was mitral stenosis.Systolic pulmonary artery pressure was 40 mmHg. Mild mitral insufficiency was detected. The patient was evaluated with the department of oncology and cardiovascular surgery. It was decided that the mass was inoperable. So transesophageal echocardiography was not performed. It was thought that, the mass was primary cardiac sarcoma, pulmonary nodule and bone lesions were metastasis of this primary cardiac sarcoma. So chemotherapy protocol (ifosfamide,adriamycin,mesna) was started.

Conclusion Cardiac tumors have many clinical presentations. Early stages of the disease are often asymptomatic. In advanced stages, patients present with symptoms of the classic triad (intracardiac obstruction, systemic embolization, constitutional symptoms) Symptoms are often non-spesific; so diagnostic suspicion is very important. Although cardiac sarcoma therapy includes complete surgical excision, followed by radiotherapy and chemotherapy, prognosis is still poor. Surgery can offer dramatic palliation of symptoms in cases of valvular obstruction. In conclusion, it remains clear that early diagnosis and treatment are extremely important because of their prognostic and therapeutic value.

Abstract P1697 Figure.