Cardiac tumor recurrence after orthotopic heart transplantation in primary cardiac lymphoma

Primary cardiac lymphoma is a rare malignancy. It is difficult to diagnose, and there is no standard mode of treatment, surgical resection is rarely performed. The prognosis is poor, because of the advanced stage of myocardial involvement at the time of presentation. We report a rare case of PCL diagnosed as DLBCL treated with orthotopic heart transplantation and 4 cycles of chemotherapy with R-COP. The patient achieved a complete response (CR), but suffered from a pulmonary fungal infection. A tumor occurrence was occurred in the mediastina and the transplanted heart, due to 2 months suspension of chemotherapy for the infection. Subsequently, the patient received a mediastinal intensity-modulated radiation therapy (IMRT) of 20 Gy in 11 fractions and a second-line chemotherapy with GEMOX with a partial response (PR). However, the patient finally died of tumor progression (in the mediastina, the neck, the thorax, and the abdomen) and serious fungal infection of lungs and survived for 11 months after diagnosis.