title: Malignant rhabdoid tumour of the liver in the young adult: report of first two cases.
abstract: 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.
authors.affiliation: Centre de Chirurgie Viscérale et de Transplantation, Hôpital de Hautepierre, 67098 Strasbourg, France.
authors.forename: Ettore
authors.initials: E
authors.lastname: Marzano
authors.forename: Emilie
authors.initials: E
authors.lastname: Lermite
authors.forename: Cinzia
authors.initials: C
authors.lastname: Nobili
authors.forename: Carlos
authors.initials: C
authors.lastname: Teyssedou
authors.forename: Philippe
authors.initials: P
authors.lastname: Bachellier
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Arnaud
authors.forename: Patrick
authors.initials: P
authors.lastname: Pessaux
mesh_terms: D000293:Adolescent; D000328:Adult; D003952:Diagnostic Imaging; D017809:Fatal Outcome; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D008297:Male; D018335:Rhabdoid Tumor / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*