title: Hyperammonemic Encephalopathy Associated With Fibrolamellar Hepatocellular Carcinoma: Case Report, Literature Review, and Proposed Treatment Algorithm.
abstract: We report a case of a 31-year-old man with metastatic fibrolamellar hepatocellular carcinoma (FLHCC) treated with gemcitabine and oxaliplatin complicated by hyperammonemic encephalopathy biochemically consistent with acquired ornithine transcarbamylase deficiency. Awareness of FLHCC-associated hyperammonemic encephalopathy and a pathophysiology-based management approach can optimize patient outcome and prevent serious complications. A discussion of the management, literature review, and proposed treatment algorithm of this rare metabolic complication are presented.
  
  
  CONCLUSIONS
  Pathophysiology-guided management of cancer-associated hyperammonemic encephalopathy can improve patient outcome and prevent life-threatening complications. Community and academic oncologists should be aware of this serious metabolic complication of cancer and be familiar with its management.
authors.affiliation: Dana-Farber Cancer Institute at St. Elizabeth's Medical Center, Boston, Massachusetts, USA.
authors.forename: Claudia I
authors.initials: CI
authors.lastname: Chapuy
authors.affiliation: Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Inderneel
authors.initials: I
authors.lastname: Sahai
authors.affiliation: Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Rohit
authors.initials: R
authors.lastname: Sharma
authors.affiliation: Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.
authors.forename: Andrew X
authors.initials: AX
authors.lastname: Zhu
authors.affiliation: Dana-Farber Cancer Institute at St. Elizabeth's Medical Center, Boston, Massachusetts, USA Olga_Kozyreva@dfci.harvard.edu.
authors.forename: Olga N
authors.initials: ON
authors.lastname: Kozyreva
mesh_terms: D000328:Adult; D001927:Brain Diseases / Q000139:chemically induced / Q000473:pathology*; D006528:Carcinoma, Hepatocellular / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D003841:Deoxycytidine / Q000008:administration & dosage / Q000009:adverse effects / Q000031:analogs & derivatives; D006801:Humans; D022124:Hyperammonemia / Q000139:chemically induced / Q000473:pathology*; D008113:Liver Neoplasms / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D009944:Organoplatinum Compounds / Q000008:administration & dosage / Q000009:adverse effects; D020163:Ornithine Carbamoyltransferase Deficiency Disease / Q000139:chemically induced / Q000473:pathology; D000077150:Oxaliplatin; D000093542:Gemcitabine
chemical_list: D009944:Organoplatinum Compounds; D000077150:Oxaliplatin; D003841:Deoxycytidine; D000093542:Gemcitabine
keywords: Fibrolamellar hepatocellular carcinoma; Hyperammonemia; OTC deficiency; Paraneoplastic hyperammonemia