title: Unusual evolution of an Epstein-Barr virus-associated leiomyosarcoma occurring after liver transplantation.
abstract: We report the case of a child who developed, 2 yr after orthotopic liver transplantation (OLTx) for biliary atresia, a multi-focal hepatic tumor with lymphonodular metastases, identified as an Epstein-Barr virus (EBV)-associated leiomyosarcoma. Chemotherapy was given without tumor response. Subsequently, slow growth of the tumor was observed. Immunosuppression was tapered and stopped 9 yr after transplantation. At the present time, 12 yr after the discovery of the first hepatic lesions, the patient is alive and completely symptom-free, the abdominal masses are stable, and liver function tests are completely normal. Smooth muscle tumors are increasingly recognized in children with various immunodeficiencies occurring after organ transplantation. This unusual evolution of a clinically aggressive tumor into a stable disease after restoration of immunity confirms that the immune status of the patient is a crucial factor.
authors.affiliation: Department of Pediatric Hematology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Avenue Hippocrate 10, B-1200 Brussels, Belgium. Brichard@pedi.ucl.ac.be
authors.forename: B
authors.initials: B
authors.lastname: Brichard
authors.forename: F
authors.initials: F
authors.lastname: Smets
authors.forename: E
authors.initials: E
authors.lastname: Sokal
authors.forename: P
authors.initials: P
authors.lastname: Clapuyt
authors.forename: C
authors.initials: C
authors.lastname: Vermylen
authors.forename: G
authors.initials: G
authors.lastname: Cornu
authors.forename: J
authors.initials: J
authors.lastname: Rahier
authors.forename: J B
authors.initials: JB
authors.lastname: Otte
mesh_terms: D001656:Biliary Atresia / Q000601:surgery; D002675:Child, Preschool; D020031:Epstein-Barr Virus Infections / Q000276:immunology* / Q000473:pathology; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006801:Humans; D007890:Leiomyosarcoma / Q000276:immunology / Q000473:pathology / Q000821:virology*; D008113:Liver Neoplasms / Q000276:immunology / Q000473:pathology / Q000821:virology*; D016031:Liver Transplantation / Q000276:immunology*; D008297:Male; D011183:Postoperative Complications / Q000276:immunology*; D019562:Viral Load