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

Surjan, Rodrigo C.; Santos, Elizabeth Santana Dos; Silveira, Sergio do Prado; Makdissi, Fábio F.; Machado, Marcel Autran

doi:10.3350/cmh.2019.0084

Cited by 3 publications

(3 citation statements)

References 12 publications

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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: 67%

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: 67%

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

Chen

Shi

et al. 2022

Front. Oncol.

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“…10,11 Further, these changes cause dysfunction of c-Myc and ornithine decarboxylase, resulting in the depletion of amino acids crucial to the urea cycle. 12 Dysfunction of the urea cycle in turn causes accumulation of ammonia and hyperammonemia. However, when these symptoms are treated with routine therapies for cirrhotic patients with hepatic encephalopathy, these patients tend to not respond.…”

Section: Introductionmentioning

confidence: 99%

Current Advances in the Treatment of Fibrolamellar Carcinoma of Liver

Gummadi,

Wang,

Xie

2023

JHC

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Fibrolamellar carcinoma (FLC) of the liver is a rare type of liver cancer that is prevalent in children and young adults, often less than 40 years old. The etiology is unclear. It presents without underlying liver disease with distinctive histological features such as fibrous collagen bands surrounding the tumor cells. Fusion protein DNAJB1-PRKACA is found in most of the cases. The prognosis of FLC is poor. Even though curative treatment option is surgery for a certain patient population, other treatment modalities including radiation, chemotherapy are currently being used without significant improvement of overall survival. Recently, targeted therapy and immunotherapy have been studied which may provide survival advantage in the future. This review sought to compile data from clinical trials and case reports/series to outline the current state of FLC treatment.

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“…The late stage of HCC is often associated with complications such as gastrointestinal bleeding, infection, hepatic and renal syndrome, and hepatic encephalopathy. When complicated by hepatic encephalopathy, clinical manifestations may include behavioral abnormalities, cognitive impairment, altered consciousness, and even coma, which are often combined with liver dysfunction making the treatment more difficult and the patient's prognosis poor ( 4 , 5 ). However, no extensive study has been conducted to determine whether other factors may contribute to cognitive impairment in patients with HCC.…”

Section: Introductionmentioning

confidence: 99%

Metabolomic analysis of vascular cognitive impairment due to hepatocellular carcinoma

Zhu

Zhu²,

Liu³

et al. 2023

Front. Neurol.

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IntroductionScreening for metabolically relevant differentially expressed genes (DEGs) shared by hepatocellular carcinoma (HCC) and vascular cognitive impairment (VCI) to explore the possible mechanisms of HCC-induced VCI.MethodsBased on metabolomic and gene expression data for HCC and VCI, 14 genes were identified as being associated with changes in HCC metabolites, and 71 genes were associated with changes in VCI metabolites. Multi-omics analysis was used to screen 360 DEGs associated with HCC metabolism and 63 DEGs associated with VCI metabolism.ResultsAccording to the Cancer Genome Atlas (TCGA) database, 882 HCC-associated DEGs were identified and 343 VCI-associated DEGs were identified. Eight genes were found at the intersection of these two gene sets: NNMT, PHGDH, NR1I2, CYP2J2, PON1, APOC2, CCL2, and SOCS3. The HCC metabolomics prognostic model was constructed and proved to have a good prognostic effect. The HCC metabolomics prognostic model was constructed and proved to have a good prognostic effect. Following principal component analyses (PCA), functional enrichment analyses, immune function analyses, and TMB analyses, these eight DEGs were identified as possibly affecting HCC-induced VCI and the immune microenvironment. As well as gene expression and gene set enrichment analyses (GSEA), a potential drug screen was conducted to investigate the possible mechanisms involved in HCC-induced VCI. The drug screening revealed the potential clinical efficacy of A-443654, A-770041, AP-24534, BI-2536, BMS- 509744, CGP-60474, and CGP-082996.ConclusionHCC-associated metabolic DEGs may influence the development of VCI in HCC patients.

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

Cited by 3 publications

References 12 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

Current Advances in the Treatment of Fibrolamellar Carcinoma of Liver

Metabolomic analysis of vascular cognitive impairment due to hepatocellular carcinoma

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