Successful management of recurrent Epstein-Barr virus-associated multilocular leiomyosarcoma after cardiac transplantation.

Abstract Background In contrast to Epstein-Barr virus (EBV)-associated posttransplant lymphoproliferative disorders (PTLD), EBV-associated leiomyomatous tumors have thus far only rarely been described. Case report Two years after heart transplantation with ATG induction, cyclosporine (CsA; trough levels of 250 ng/mL)–based triple drug immunosuppression), a 23-year-old patient developed a small round lesion within the left lateral liver segment. The patient underwent ultrasound-guided biopsy followed by liver resection. Histological and immunohistological examination showed a leiomyosarcoma. In situ hybridization using EBV-specific EB endoplasmic reticulum-RNA showed an intensive signal in almost all tumor cells. The tumor stained for EB nuclear antigen (EBNA)-2-protein. Immunosuppression was drastically reduced, namely, CsA levels Conclusion Long-term survival in patients with posttransplant EBV-associated leiomyosarcoma can be achieved by combined surgical intervention, reduction of immunosuppression, switch to Sirolimus, and antiviral chemotherapy.