Cardiac Myeloid Sarcoma: A Rare Case Report

Introduction Cardiac myeloid sarcoma occurs less than <1% of all sarcoma cases. For this reason, the literature data is limited to case reports and small retrospective studies. Clinical knowledge of this condition, its manifestations and treatment are limited. Case Presentation A 68-year-old man presented typical symptoms of unstable angina pectoris (pain in the chest area, shortness of breath, general weakness). Echocardioscopy revealed a 26-32 mm tumor in right atrial cavity. Cardiac magnetic resonance imaging (MRI) revealed solid, heterogenic, contrast-accumulating tumorous structure (87x72x74 mm) with solid and necrotic areas on the right side of the heart. Even though the endocardium biopsy results did not show any tumorous changes, tumor excision surgery and histological verification revealed an acute myeloid leukemia (AML). Histological testing confirmed myeloid sarcoma (most probably AML infiltration): polymorphic cells with big, uneven nucleus, multiple mitoses, positive LCA, CD117, CD99, CD34, bcl-2 marks, Proliferation Ki67 index was 80%. For further diagnosis, bone marrow aspiration and blood smear were taken. Both showed blasts numbers 5% and 7%. Genetic testing showed CBFB-MYH11 inv(16)(p13;q22) mutation, which is typical for AML. Patient underwent treatment with induction: Daunorubicin and Cytarabine (7+3), consolidation with high dose Cytarabine (3 courses), allogenic matched unrelated identical donor haemopoetic stem cells transplantation. Complete remission was achieved. Discussion The most common symptom of cardiac myeloid sarcoma is pericarditis, with or without leukaemic cells in the pericardial fluid. Other clinical signs may include tachycardia, chest tightness, palpitations, chest pain, arrhythmias. The diagnostic gold standard is MRI and biopsy, in some of the described clinical cases PET/CT has been used, due to the more precise location of the biopsy. Treatment is given according to the standard AML protocol. Conclusions Cardiac myeloid sarcoma is treated according to the algorithms of AML. Systemic chemotherapy and allogeneic haematopoietic stem cell transplantation significantly prolong overall survival. Patient follow-up in the posttransplantation period is important for possible recurrence of the disease or post-transplant complications. [1–4]