title: Liver Transplant for Children With Hepatocellular Carcinoma and Hereditary Tyrosinemia Type 1.
abstract: OBJECTIVE
  This study sought to determine the prevalence of hepatocellular carcinoma and other premalignant lesions in children with hereditary tyrosinemia type 1 who had undergone an orthotopic liver transplant at the Shiraz Transplant Center, in Shiraz, Iran.
  
  
  METHODS
  Between September 2006, and June 2011, thirty-six patients with hereditary tyrosinemia type 1 received a liver transplant from a deceased (whole or split) or a living-related donor. Clinical records and pathologic specimens, before and after surgery, for each case were reviewed. In addition, ultrasound, abdominal computed tomographic imaging scan findings, and levels of alpha-fetoprotein were recorded.
  
  
  RESULTS
  Twenty-two patients with hepatic nodules larger than 10 mm underwent a Tru-Cut needle biopsy before their liver transplant. In 2 patients, a diagnosis of hepatocellular carcinoma was made by pathologic examination; in the other 20, cirrhosis was confirmed with no evidence of malignancy. After pathologic examination of the explanted livers, the largest nodules in the 36 patients were 35 mm. Five cases had at least 1 nodule of hepatocellular carcinoma. Three of the other patients had small cell dysplasia in some of nodules. All 5 cases with hepatocellular carcinoma were patients older than 2 years of age (19 patients were older than 2 years of age). All patients with hepatocellular carcinoma received pretransplant nitisinone treatment. All patients with hepatocellular carcinoma after their liver transplant are alive at the time of this writing.
  
  
  CONCLUSIONS
  The prevalence of cell dysplasia and hepatocellular carcinoma in children with hereditary tyrosinemia type 1 in our study is not as high as that reported previously, so it appears that patients older than 2 years of age require a liver transplant.
authors.affiliation: Department of Surgery, Transplant Ward, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Ali
authors.initials: A
authors.lastname: Bahador
authors.forename: Seyed Mohsen
authors.initials: SM
authors.lastname: Dehghani
authors.forename: Bita
authors.initials: B
authors.lastname: Geramizadeh
authors.forename: Saman
authors.initials: S
authors.lastname: Nikeghbalian
authors.forename: Mohsen
authors.initials: M
authors.lastname: Bahador
authors.forename: Seyed Ali
authors.initials: SA
authors.lastname: Malekhosseini
authors.forename: Kurosh
authors.initials: K
authors.lastname: Kazemi
authors.forename: Heshmatolah
authors.initials: H
authors.lastname: Salahi
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001706:Biopsy; D006528:Carcinoma, Hepatocellular / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007492:Iran / Q000453:epidemiology; D008113:Liver Neoplasms / Q000097:blood / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D015995:Prevalence; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D020176:Tyrosinemias / Q000175:diagnosis / Q000453:epidemiology / Q000601:surgery*; D000509:alpha-Fetoproteins / Q000032:analysis
chemical_list: C097353:AFP protein, human; D000509:alpha-Fetoproteins