title: Stereotactic radiofrequency ablation for liver tumors in inherited metabolic disorders.
abstract: OBJECTIVE
  Both glycogen storage disease type Ia (GSD Ia) and tyrosinemia type I (TYR I) are inherited metabolic disorders that can be complicated by formation of liver adenomas in juvenile/young adult age and/or development of hepatocellular carcinoma. We describe the first application of stereotactic radiofrequency ablation (SRFA) in focal lesions in three patients with inherited metabolic disorders affecting the liver.
  
  
  METHODS
  SRFA was applied for removal of single large liver adenomas in a 22-year-old woman and a 20-year-old man with GSD Ia and of a suspicious lesion in a 16-year-old girl with TYR I with α-fetoprotein (AFP) elevation.
  
  
  RESULTS
  SRFA was successful. Large scars were avoided, and in the TYR I patient, elevated AFP values promptly returned to normal.
  
  
  CONCLUSIONS
  The SRFA technique is a good alternative to surgical resection of focal liver lesions and could greatly help patients with inherited metabolic disorders with liver involvement, including focal liver lesions and potential malignancy.
authors.affiliation: Department of Pediatrics I, Inherited Metabolic Disorders, Medical University of Innsbruck, Anichstrasse 35, 6020, Innsbruck, Austria, daniela.karall@i-med.ac.at.
authors.forename: Daniela
authors.initials: D
authors.lastname: Karall
authors.forename: Sabine
authors.initials: S
authors.lastname: Scholl-Bürgi
authors.forename: Gerlig
authors.initials: G
authors.lastname: Widmann
authors.forename: Ursula
authors.initials: U
authors.lastname: Albrecht
authors.forename: Katharina
authors.initials: K
authors.lastname: Niedermayr
authors.forename: Kathrin
authors.initials: K
authors.lastname: Maurer
authors.forename: Bernd
authors.initials: B
authors.lastname: Ausserer
authors.forename: Martina
authors.initials: M
authors.lastname: Huemer
authors.forename: Reto
authors.initials: R
authors.lastname: Bale
mesh_terms: D000293:Adolescent; D017115:Catheter Ablation / Q000379:methods*; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications*; D006801:Humans; D008113:Liver Neoplasms / Q000209:etiology* / Q000473:pathology / Q000601:surgery*; D008297:Male; D015641:Radiography, Interventional; D016896:Treatment Outcome; D020176:Tyrosinemias / Q000150:complications*; D055815:Young Adult; D000509:alpha-Fetoproteins / Q000032:analysis
chemical_list: D000509:alpha-Fetoproteins