Glycogenosis storage type I diseases and evolutive adenomatosis: an indication for liver transplantation.
We report on two cases of type I glycogen storage disease (GSD) complicated by malignant tumors. A 23-year-old man had GSD Ia with adenomatosis. He underwent transplantation for rapidly growing and radiologically changing adenomata. At histological examination, one adenoma had become a hepatocellular carcinoma. A 22-year-old, HBV-infected woman had GSD type Ib with adenomatosis. At follow-up, several tumors showed changing morphological characteristics. Pre-transplant laparotomy confirmed the presence of a metastatic cholangiocarcinoma. Liver transplantation should be considered in GSD type I patients with adenomatosis, especially when tumor characteristics change. Regular detailed Doppler ultrasound and magnetic nuclear resonance screening during childhood and adolescence are, therefore, mandatory in order for the timing of transplantation to be optimized.
Lerut
Jan P
JP
Cliniques Universitaires St. Luc/1400, Université catholique de Louvain (UCL), Avenue Hippocrate 10, 1200 Brussels, Belgium. lerut@chir.ucl.ac.be
Ciccarelli
Olga
O
Sempoux
Christine
C
Danse
Etienne
E
deFlandre
Jacques
J
Horsmans
Yves
Y
Sokal
Etienne
E
Otte
Jean-Bernard
JB
D018248:Adenoma, Liver Cell / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D000328:Adult; D018281:Cholangiocarcinoma / Q000150:complications / Q000473:pathology / Q000601:surgery; D017809:Fatal Outcome; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications*; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male