Primary cardiac lymphoma

Primary cardiac lymphoma is a very rare tumor, with no typical clinical presentation, making early diagnosis very challenging. The best diagnostic strategy, pathological characteristics, optimal treatments, and prognostic factors are not well defined. Mortality rate is very high if the disease is not promptly recognized and treated. Tissue characterization is pivotal for diagnosis and treatment strategy. At present, the most effective treatment is anthracycline-based chemotherapy. Since well-defined guidelines on the management of primary cardiac lymphoma are still lacking, future prospective investigations for better defining the optimal approach of patients with this rare disease are strongly required.