Hyperammonemic encephalopathy: An unusual presentation of fibrolamellar hepatocellular carcinoma

Thakral, Nimish; Simonetto, Douglas A.

doi:10.3350/cmh.2018.0042

Cited by 12 publications

(8 citation statements)

References 10 publications

(14 reference statements)

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“…As another HCC subtype rich in stromal components, scirrhous carcinoma shared almost the same macroscopical clinical characteristics and prognoses with classic HCC, which was also supported by other researchers (19,21,(31)(32)(33). Interestingly, both the two rare subtypes might present unusual paraneoplastic manifestations: hyperammonemic encephalopathy (HAE) occurred in patients with fibrolamellar carcinoma, and hypercalcemia was found in scirrhous carcinoma sufferers (34)(35)(36)(37). However, for most patients, vague symptoms and nonspecific imaging features presented challenges in differential diagnoses of scirrhous carcinoma, especially from fibrolamellar carcinoma and cholangiocarcinoma.…”

Section: Discussionsupporting

confidence: 70%

Epidemiological and Clinical Characteristics of Five Rare Pathological Subtypes of Hepatocellular Carcinoma

Chen

Shi

et al. 2022

Front. Oncol.

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BackgroundHepatocellular carcinoma (HCC) is a highly heterogeneous tumor with several rare pathological subtypes and which is still poorly understood. This study aimed to describe the epidemiological and clinical spectrum of five rare HCC subtypes and develop a competing risk nomogram for cancer-specific survival prediction.MethodsThe study cohort was recruited from the Surveillance, Epidemiology, and End Results database. The clinicopathological data of 50,218 patients histologically diagnosed with classic HCC and five rare subtypes (ICD-O-3 Histology Code = 8170/3-8175/3) between 2004 and 2018 were reviewed. The annual percent change (APC) was calculated utilizing Joinpoint regression. The nomogram was developed based on multivariable competing risk survival analyses. Akaike information criterion, Bayesian information criterion, C-index, calibration curve, and area under the receiver operating characteristic curve were obtained to evaluate the prognostic performance. A decision curve analysis was introduced to examine the clinical value of the models.ResultsDespite scirrhous carcinoma, which showed a decreasing trend (APC = -6.8%, P = 0.025), the morbidity of other rare subtypes remained stable from 2004 to 2018. The incidence-based mortality was plateau in all subtypes during the period. Clear cell carcinoma is the most common subtype (n = 551, 1.1%), followed by subtypes of fibrolamellar (n = 241, 0.5%), scirrhous (n = 82, 0.2%), spindle cell (n = 61, 0.1%), and pleomorphic (n = 17, ~0%). The patients with fibrolamellar carcinoma were younger and more likely to have a non-cirrhotic liver and better prognoses. Scirrhous carcinoma shared almost the same macro-clinical characteristics and outcomes as the classic HCC. Clear cell carcinoma tended to occur in the Asia-Pacific elderly male population, and more than half of them were large HCC (Size>5cm). Sarcomatoid (including spindle cell and pleomorphic) carcinoma was associated with a larger tumor size, poorer differentiation, and more dismal prognoses. The pathological subtype, T stage, M stage, surgery, alpha-fetoprotein, and cancer history were confirmed as the independent predictors in patients with rare subtypes. The nomogram showed good calibration, discrimination, and net benefits in clinical practice.ConclusionThe rare subtypes had unique clinicopathological features and biological behaviors compared with the classic HCC. Our findings could provide a valuable reference for clinicians. The constructed nomogram could predict the prognoses with good performance, which is meaningful to individualized management.

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Section: Discussionsupporting

confidence: 70%

Epidemiological and Clinical Characteristics of Five Rare Pathological Subtypes of Hepatocellular Carcinoma

Chen

Shi

et al. 2022

Front. Oncol.

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“…We recently had the opportunity ro read the brilliant paper by Thakral and Simonetto "Hyperammonemic encephalopathy: An unusual presentation of fibrolamellar hepatocellular carcinoma" in the Clinical and Molecular Hepatology. 1 We would appreciate to share some insights about this interesting theme.…”

Section: Dear Editormentioning

confidence: 96%

“…The importance of this finding was, once understanding that an urea cycle disorder due to metabolites consumption was responsible for the hyperammonemia, to guide the developement of new medical treatment options based on a combination of ammonia scavenger drugs (sodium benzoate and phenylbutyrate) with amino acids supplementation (citrulline, ornithine and arginine), thus reducing the mortality of FLHCC-related HAE and allowing complete clinical recovery from HAE. 1,6,8 "What is the cause of acute hepatic encephalopathy in a young patient?" regarding an 18-year-old patient with a large FLHCC has been answered, including its precise pathophysiology.…”

Section: Dear Editormentioning

confidence: 99%

Fibrolamellar hepatocellular carcinoma-related hyperammonemic encephalopathy: Up to now and next steps

et al. 2020

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“…Case reports have described hyperammonemia in a narrow spectrum of tumor types (e.g. GIST, myeloma, neuroendocrine tumors, hepatocellular carcinoma, renal cell carcinoma, and colorectal carcinoma) [3][4][5][6][7][8][9][10][11][12][13][14]. GIST in particular is well known for complications related to endophytic growth leading to GI bleeding, obstruction, and perforation.…”

Section: Discussionmentioning

confidence: 99%

A Case of Non-cirrhotic Hyperammonemic Encephalopathy in a Patient With Metastatic Gastrointestinal Stromal Tumor

Jeong¹,

Abiri²,

Cai³

et al. 2023

Cureus

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Acute toxic encephalopathy (ATE) is a widely recognized medical emergency with an expansive differential. One particular known etiology for ATE is elevated ammonia, a powerful neurotoxin that often presents with clinical findings of confusion, disorientation, tremors, and in severe cases, coma and death. Hyperammonemia is most commonly associated with liver disease and presents as hepatic encephalopathy in the setting of decompensated cirrhosis; however, in rare cases, a patient may suffer from non-cirrhotic hyperammonemic encephalopathy. We describe the case of a 61-year-old male with metastatic gastrointestinal stromal tumor who was diagnosed with non-cirrhotic hyperammonemic encephalopathy, and briefly explore the literature describing its mechanisms.

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Hyperammonemic encephalopathy: An unusual presentation of fibrolamellar hepatocellular carcinoma

Cited by 12 publications

References 10 publications

Epidemiological and Clinical Characteristics of Five Rare Pathological Subtypes of Hepatocellular Carcinoma

Epidemiological and Clinical Characteristics of Five Rare Pathological Subtypes of Hepatocellular Carcinoma

Fibrolamellar hepatocellular carcinoma-related hyperammonemic encephalopathy: Up to now and next steps

A Case of Non-cirrhotic Hyperammonemic Encephalopathy in a Patient With Metastatic Gastrointestinal Stromal Tumor

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