Malignant rhabdoid tumour of the liver in the young adult: report of first two cases.
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.
Marzano
Ettore
E
Centre de Chirurgie Viscérale et de Transplantation, Hôpital de Hautepierre, 67098 Strasbourg, France.
Lermite
Emilie
E
Nobili
Cinzia
C
Teyssedou
Carlos
C
Bachellier
Philippe
P
Arnaud
Jean-Pierre
JP
Pessaux
Patrick
P
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*