Cancer Res Treat.  2021 Jan;53(1):283-288. 10.4143/crt.2020.575.

Hyperammonemic Encephalopathy Mimicking Ornithine Transcarbamylase Deficiency in Fibrolamellar Hepatocellular Carcinoma: Successful Treatment with Continuous Venovenous Hemofiltration and Ammonia Scavengers

• Lee JS
• Jin HY
• Ko JM
• Kim SH
• Han N
• Park BK
• Park M
• Park HJ
• Lee JA

• KMID: 2510671
• DOI: http://doi.org/10.4143/crt.2020.575

Abstract

Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare liver cancer affecting adolescents and young adults without any pre existing liver disease. Hyperammonemic encephalopathy (HAE) is a serious paraneoplastic syndrome, and several cases of HAE have been reported in patients with FLHCC. This condition is rare; hence, there are currently no management guidelines for cancer-related HAE. Herein, we report a case of an 18-year-old man with advanced FLHCC who developed HAE during the first course of chemotherapy consisting of cisplatin, doxorubicin, 5-fluorouracil, and interferon-α. He was successfully treated with continuous venovenous hemofiltration, sodium benzoate, sodium phenylbutyrate, and amino acid supplementation for HAE. After the second course of chemotherapy, he underwent surgery, and thereafter, his ammonia levels were normal without any ammonia scavenger therapy. Treatments for HAE described here will be helpful for this rare, but serious metabolic complication of FLHCC and could partially applied to HAE related to any malignancies.

Keyword

Fibrolamellar hepatocellular carcinoma; Hyperammonemia; Encephalopathy; OTC deficiency

Figure

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  Fig. 1 Dynamic liver computed tomography at the time of diagnosis of fibrolamellar hepatocellular carcinoma (coronal view, portal phase).

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  Fig. 2 Photographs of the fine needle biopsy and surgical resection specimen. (A) Histologic features of fibrolamellar hepatocellular carcinoma (FLHCC). Large, polygonal tumor cells with abundant oncocytic cytoplasm in background of dense collagen bundles (H&E, ×100). Cytokeratin 7 (B) and CD68 (C) protein expression were noted in FLHCC evaluated by immunohistochemistry (×100). (D) Representative image of surgical specimen. Bulging, large mass with well-circumscribed border was observed. Fibrous bands and central stellate scar are noted.

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  Fig. 3 Change of the serum ammonia levels with ammonia scavengers and hemofiltration. HF, hemofiltration; IV, intravenously; PO, orally.

Reference

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