Primary cardiac MYC/BCL6 double hit non-Hodgkin lymphoma.

Cardiac and pericardial involvement by malignant lymphoma is a rare condition. The present case report describes a case of primary cardiac MYC/BCL6 double hit non-Hodgkin lymphoma in the pericardium, and highlights the importance of a prompt diagnosis and aggressive pharmacologic treatment of this disease. In a symptomatic patient, a minimally invasive 3 cm sub-xiphoidal incision was performed under deep sedation with spontaneous ventilation to perform a pericardial biopsy. A 5 cm × 3 cm portion of pericardium was removed from above the right ventricle, thus ameliorating the extrinsic compression on the right chambers. The patient received 6 cycles of immuno-chemotherapy (rituximab plus cyclophosphamide, vincristine, and methylprednisolone), with no complications, achieving complete remission with no symptoms. Malignancies must be excluded in every case of acute pericardial disease with imaging techniques, and lymphomas should be always considered in the differential diagnosis of cardiac tumors. Complete surgical removal of the tumor is not necessary to achieve complete remission, and minimally invasive surgical approaches are an effective tool to confirm diagnosis and allow a precise histologic characterization. <Learning objective: Primary MYC/BCL6 double hit non-Hodgkin lymphoma is a rare tumor of the pericardium, which requires prompt diagnosis and treatment. Minimally invasive surgical approaches are an effective tool to confirm diagnosis and allow a precise histologic characterization. Pericardial tumors should always be considered in the differential diagnosis of acute pericardial disease presenting with pericardial effusion. Double hit diffuse large B cell-lymphoma has a poor prognosis with standard chemotherapy and the treatment should be tailored according to the patient's comorbidities and performance status.>.