Massive myocardial infiltration by primary anaplastic T-cell lymphoma: a case report.

BACKGROUND: Myocardial infiltration by primary cardiac neoplasm is a rare entity, providing diagnostic and therapeutic challenges. The pathological spectrum includes more frequently benign forms. Refractory heart failure, pericardial effusion, and arrhythmias due to infiltrative mass are the most common clinical manifestations.

CASE SUMMARY: We describe the case of a 35-year-old man complaining of shortness of breath and weight loss in the last 2 months. A previous acute myeloid leukaemia treated with allogenic bone marrow transplant was reported. Transthoracic echocardiography revealed an apical thrombus in the left ventricle, with inferior and septal hypokinesia conditioning a mildly reduced ejection fraction, circumferential pericardial effusion, and abnormal right ventricular thickening. Cardiac magnetic resonance confirmed diffuse thickening of the right ventricular free wall due to myocardial infiltration. Positron emission tomography showed the presence of neoplastic tissue with increased metabolic activity. A pericardiectomy was performed showing a widespread cardiac neoplastic infiltration. Histopathological analysis done on right ventricular pathological samples obtained during cardiac surgery revealed the presence of a rare and aggressive cardiac anaplastic T-cell non-Hodgkin lymphoma. Few days after the operation, the patient developed refractory cardiogenic shock and unluckily died before initiating an adequate antineoplastic therapy.

DISCUSSION: Primary cardiac lymphoma is not frequent, and the lack of specific symptoms makes the diagnosis extremely challenging and often limited to autopsy findings. Our case highlights the importance of an appropriate diagnostic algorithm, requiring non-invasive multimodality assessment imaging and then invasive cardiac biopsy. This approach may allow an early diagnosis and an adequate therapy for this otherwise fatal pathology.