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Unusual evolution of an Epstein–Barr
virus‐associated leiomyosarcoma occurring after liver transplantation
Author(s) -
Brichard Bénédicte,
Smets Françoise,
Sokal Etienne,
Clapuyt Philippe,
Vermylen Christiane,
Cornu Guy,
Rahier Jacques,
Otte Jean Bernard
Publication year - 2001
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1034/j.1399-3046.2001.00022.x
Subject(s) - medicine , immunosuppression , biliary atresia , leiomyosarcoma , liver transplantation , transplantation , epstein–barr virus , pathology , immune system , chemotherapy , virus , immunology
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.