Unusual evolution of an Epstein-Barr virus-associated leiomyosarcoma occurring after liver transplantation

We report the case of a child who developed, 2 yr after orthotopic liver tr ansplantation (OLTx) for biliary atresia, a multi-focal hepatic tumor with lymphonodular metastases, identified as an Epstein-Barr virus (EBV)-associa ted leiomyosarcoma. Chemotherapy was given without tumor response. Subseque ntly, slow growth of the tumor was observed. Immunosuppression was tapered and stopped 9 yr after transplantation. At the present time, 12 yr after th e discovery of the first hepatic lesions, the patient is alive and complete ly symptom-free, the abdominal masses are stable, and liver function tests are completely normal. Smooth muscle tumors are increasingly recognized in children with various immuno deficiencies occurring after organ transplanta tion. This unusual evolution of a clinically aggressive tumor into a stable disease after restoration of immunity confirms that the immune status of t he patient is a crucial factor.