Fiche publication
Date publication
janvier 2009
Auteurs
Membres identifiés du Cancéropôle Est :
Pr BACHELLIER Philippe
,
Pr PESSAUX Patrick
Tous les auteurs :
Marzano E, Lermite E, Nobili C, Teyssedou C, Bachellier P, Arnaud JP, Pessaux P
Lien Pubmed
Résumé
Few cases of malignant rhabdoid tumour (MRT) of the liver are reported in literature and always in paediatric patients. We report the first two cases of young adults submitted to hepatic resection for MRT of the liver. A major liver resection was performed in both cases. The histology showed round or fusiform, loosely cohesive cells. The cytoplasm contained abundant eosinophilic inclusions, which caused the nuclei to be located in eccentric locations, giving the characteristic rhabdoid appearance. The immunohistochemical study was performed, and characteristic lack of nuclear INI1 protein expression was found. In a case surgery was associated to chemoradiotherapy. One patient died at 48 months followup for tumour recurrence. The other is still alive at 25 months followup. MRTs are rare tumours of pediatric age with poor prognosis. Hypothetical less malignant behaviour in the young adults could be supposed. Therefore an aggressive surgical and oncological treatment seems justified.
Référence
HPB Surg. 2009;2009:628206
