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Case Reports
. 2016 Apr;21(4):514-20.
doi: 10.1634/theoncologist.2015-0267. Epub 2016 Mar 14.

Hyperammonemic Encephalopathy Associated With Fibrolamellar Hepatocellular Carcinoma: Case Report, Literature Review, and Proposed Treatment Algorithm

Claudia I Chapuy  1 , Inderneel Sahai  2 , Rohit Sharma  2 , Andrew X Zhu  2 , Olga N Kozyreva  3
Affiliations
Case Reports

Hyperammonemic Encephalopathy Associated With Fibrolamellar Hepatocellular Carcinoma: Case Report, Literature Review, and Proposed Treatment Algorithm

Claudia I Chapuy et al. Oncologist. 2016 Apr.

Abstract
in English, Chinese

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.

Implications for practice: 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.

摘要

本文报告了一例患有转移性纤维板层型肝细胞癌 （FLHCC） 的 31 岁男性患者, 在接受吉西他滨和奥沙利铂治疗后并发高氨血症性脑病, 其生化特征与鸟氨酸氨甲酰转移酶缺乏症一致。知晓 FLHCC 相关性高氨血症性脑病, 以及了解基于其病理生理学的管理方案, 能够使患者获得最佳转归并预防严重并发症。本文还对这一罕见代谢性并发症进行了相关处理的讨论、文献综述和提出了建议治疗方案。The Oncologist 2016;21:514–520

对临床实践的提示: 对癌症相关性高氨血症性脑病进行病理生理学指导的管理能够改善患者预后及预防危及生命的并发症。社区医生和专业肿瘤科医生应知晓这一严重的癌症代谢并发症并熟悉其管理措施。

Keywords: Fibrolamellar hepatocellular carcinoma; Hyperammonemia; OTC deficiency; Paraneoplastic hyperammonemia.

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Conflict of interest statement

Disclosures of potential conflicts of interest may be found at the end of this article.

Figures

Figure 1.
Figure 1.
Abdominal computed tomographic scan of the liver showing numerous adjacent extrahepatic peritoneal nodules around the left lateral and inferior border.
Figure 2.
Figure 2.
Ammonia levels during and after treatment.
Figure 3.
Figure 3.
Screening and diagnosis (A) and management of acute episodes (B) of hyperammonemia. Abbreviations: FLHCC, fibrolamellar hepatocellular carcinoma; IV, intravenous; PEG, percutaneous endoscopic gastrostomy; PO, oral.
See this image and copyright information in PMC

References

    1. Eggert T, McGlynn KA, Duffy A, et al. Fibrolamellar hepatocellular carcinoma in the USA, 2000-2010: A detailed report on frequency, treatment and outcome based on the Surveillance, Epidemiology, and End Results database. United European Gastroenterol J. 2013;1:351–357. - PMC - PubMed
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    1. El-Serag HB, Davila JA. Is fibrolamellar carcinoma different from hepatocellular carcinoma? A US population-based study. Hepatology. 2004;39:798–803. - PubMed
    1. Moreno-Luna LE, Arrieta O, García-Leiva J, et al. Clinical and pathologic factors associated with survival in young adult patients with fibrolamellar hepatocarcinoma. BMC Cancer. 2005;5:142. - PMC - PubMed
    1. Ang CS, Kelley RK, Choti MA, et al. Clinicopathologic characteristics and survival outcomes of patients with fibrolamellar carcinoma: Data from the fibrolamellar carcinoma consortium. Gastrointest Cancer Res. 2013;6:3–9. - PMC - PubMed

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