Hyperammonemic encephalopathy in a patient with fibrolamellar hepatocellular carcinoma: Case report and literature review

Janice Cho, Joy C.Y. Chen, Jonas Paludo, Erin E. Conboy, Brendan Lanpher, Steven Robert Alberts, Thorvardur R Halfdanarson

Research output: Contribution to journalReview article

Abstract

Fibrolamellar hepatocellular carcinoma (fHCC) is a rare primary liver cancer that affects young adults with no prior liver disease. fHCC-associated hyperammonemic encephalopathy (HAE) is an uncommon and life-threatening complication. Hyperammonemia has been reported in both typical and fHCC as a result of intrahepatic shunting, side effect from immunotherapy or chemotherapy, or as a paraneoplastic phenomenon. We present a case of a 32-year-old woman with recurrent metastatic fHCC who developed HAE in the setting of steroid administration. Her hyperammonemia was exacerbated by steroid-induced protein catabolism. She was treated with ammonia scavenging medications, a low protein diet, and was placed on chronic ammonia scavenger therapy while undergoing chemotherapy. In this case report, we discuss the proposed mechanisms of HAE, and we review the literature regarding clinical presentation and treatment.

Original languageEnglish (US)
Pages (from-to)582-588
Number of pages7
JournalJournal of Gastrointestinal Oncology
Volume10
Issue number3
DOIs
StatePublished - Jan 1 2019

Fingerprint

Brain Diseases
Hyperammonemia
Ammonia
Steroids
Drug Therapy
Protein-Restricted Diet
Liver Neoplasms
Immunotherapy
Liver Diseases
Young Adult
Fibrolamellar hepatocellular carcinoma
Therapeutics
Proteins

Keywords

  • Fibrolamellar hepatocellular carcinoma (fHCC)
  • Hyperammonemia
  • Hyperammonemic encephalopathy (HAE)
  • Ornithine transcarbamylase (OTC) deficiency

ASJC Scopus subject areas

  • Oncology
  • Gastroenterology

Cite this

Hyperammonemic encephalopathy in a patient with fibrolamellar hepatocellular carcinoma : Case report and literature review. / Cho, Janice; Chen, Joy C.Y.; Paludo, Jonas; Conboy, Erin E.; Lanpher, Brendan; Alberts, Steven Robert; Halfdanarson, Thorvardur R.

In: Journal of Gastrointestinal Oncology, Vol. 10, No. 3, 01.01.2019, p. 582-588.

Research output: Contribution to journalReview article

Cho, Janice ; Chen, Joy C.Y. ; Paludo, Jonas ; Conboy, Erin E. ; Lanpher, Brendan ; Alberts, Steven Robert ; Halfdanarson, Thorvardur R. / Hyperammonemic encephalopathy in a patient with fibrolamellar hepatocellular carcinoma : Case report and literature review. In: Journal of Gastrointestinal Oncology. 2019 ; Vol. 10, No. 3. pp. 582-588.
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AB - Fibrolamellar hepatocellular carcinoma (fHCC) is a rare primary liver cancer that affects young adults with no prior liver disease. fHCC-associated hyperammonemic encephalopathy (HAE) is an uncommon and life-threatening complication. Hyperammonemia has been reported in both typical and fHCC as a result of intrahepatic shunting, side effect from immunotherapy or chemotherapy, or as a paraneoplastic phenomenon. We present a case of a 32-year-old woman with recurrent metastatic fHCC who developed HAE in the setting of steroid administration. Her hyperammonemia was exacerbated by steroid-induced protein catabolism. She was treated with ammonia scavenging medications, a low protein diet, and was placed on chronic ammonia scavenger therapy while undergoing chemotherapy. In this case report, we discuss the proposed mechanisms of HAE, and we review the literature regarding clinical presentation and treatment.

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