|
Hepatocellular Adenoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
There are a total of ten cases of both MODY 3 and HCA phenotypes reported in the literature to date; incomplete penetrance for HCA was observed, and all the patients with HCA developed diabetes.
|
31754975 |
2020 |
|
Hepatocellular Adenoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Classification of HCAs showed 46% of patients with HNF1A-mutated HCA, 31% with inflammatory HCA, 3% with sonic hedgehog HCA, 8% with unclassified HCA.
|
31419515 |
2019 |
|
Hepatocellular Adenoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
Scientific research over the period of three decades has reported it as an important player in various liver malignancies such as hepatocellular cancers (HCCs), hepatocellular adenoma (HA), and a more specific HNF-1α-inactivated human hepatocellular adenoma (H-HCAs).
|
31685031 |
2019 |
|
Hepatocellular Adenoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
HCAs have been divided into four subtypes based on molecular and pathological features: hepatocyte nuclear factor 1α-mutated HCA, inflammatory HCA, β-catenin-mutated HCA, and unclassified HCA.
|
31171888 |
2019 |
|
Hepatocellular Adenoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
Potential predictors included age, body mass index, and HCA diameter at diagnosis (T0), HCA-subtype (hepatocyte nuclear factor 1α inactivated HCA, inflammatory-HCA, unclassified HCA) and "T0-T1 regression-over-time" (percentage of regression between T0 and first follow-up (T1) divided by weeks between T0 and T1).
|
30920416 |
2019 |
|
Hepatocellular Adenoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
After initial diagnostic and aetiologic uncertainty HNF1A germline mutation c.815G>A (p.Arg272His) was confirmed 8 years later.No HCA-related complications occurred.
|
31483937 |
2019 |
|
Hepatocellular Adenoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
Malignant transformation is an exceptionally rare complication of hepatocyte nuclear factor 1α (HNF1A)-inactivated hepatocellular adenomas.
|
31570768 |
2019 |
|
Hepatocellular Adenoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Hepatocellular adenomas with HNF1A mutation or adenomatosis with loss of LFABP warrant thorough sampling and examination.
|
30121369 |
2019 |
|
Hepatocellular Adenoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Mutations in HNF1α gene are frequently associated with maturity-onset diabetes of the young type 3 (MODY3) and hepatocellular adenomas.
|
29101032 |
2018 |
|
Hepatocellular Adenoma
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
Liver fatty acid-binding protein, serum amyloid A, C-reactive protein, prostaglandin D2 synthetase, GS, and β-catenin can be used either on biopsy or surgical specimen to classify hepatocellular adenoma into hepatocyte nuclear factor (HNF-1α) inactivated (steatotic), inflammatory, with dysregulation of sonic hedgehog and prostaglandin pathways, β-catenin mutated, and unclassified.
|
28280721 |
2017 |
|
Hepatocellular Adenoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
A molecular classification has divided HCA in several subgroups linked with risk factors, clinical behaviour, histological features and imaging: HNF1A inactivated HCA, Inflammatory HCA, CTNNB1 mutated HCA in exon 3, CTNNB1 mutated in exon 7 and 8 HCA, sonic hedgehog HCA and unclassified HCA.
|
28733222 |
2017 |
|
Hepatocellular Adenoma
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
HAs presented as a heterogeneous group, with loss of PTEN observed in the inflammatory and HNF1A-mutated subtype and relatively intact PTEN expression in HA with nuclear β-catenin overexpression.
|
29687774 |
2017 |
|
Hepatocellular Adenoma
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
The expression in HA was variable and differed between molecular subtypes of this neoplasm: inflammatory and HNF1A mutation-associated type are characterized by overexpression of c-MET to an extent comparable with poorly-differentiated HCC, whereas Wnt/β-catenin dysfunction-associated type lacks overexpression, and the amount of c-MET protein accumulated in its cells is similar to the levels in non-neoplastic tissue and well- to moderately-differentiated HCC.
|
29256857 |
2017 |
|
Hepatocellular Adenoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
PET-avid hepatocellular adenomas: incidental findings associated with HNF1-α mutated lesions.
|
26776850 |
2016 |
|
Hepatocellular Adenoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
Four subtypes of hepatocellular adenomas (HCA) are recognized: hepatocyte-nuclear-factor-1α mutated (H-HCA), β-catenin-mutated type with upregulation of glutamine synthetase (b-HCA), inflammatory type (IHCA) with serum-amyloid-A overexpression, and unclassified type.
|
26961851 |
2016 |
|
Hepatocellular Adenoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
Hepatocellular adenomas (HCAs) are heterogeneous group of benign tumors; three pathomolecular subtypes have been identified so far: hepatocyte nuclear factor 1 α-inactivated HCA (H-HCA) (35-40%), inflammatory HCA (I-HCA) (>50%), β-catenin activated HCA (10%).
|
25260568 |
2015 |
|
Hepatocellular Adenoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
HNF1α inactivating HCA is associated with bi-allelic mutations in the TCF1 gene and morphologically has marked steatosis.
|
25195525 |
2015 |
|
Hepatocellular Adenoma
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
Our case is the first case of mixed FNH and HNF1A-inactivated HCA documented by immunohistochemistry.
|
26274031 |
2015 |
|
Hepatocellular Adenoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
HCAs are categorized into four subtypes on the basis of genetic and pathological features: hepatocyte nuclear factor 1α-mutated HCA, β-catenin-mutated HCA, inflammatory HCA, and unclassified HCA.
|
24509184 |
2015 |
|
Hepatocellular Adenoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
The HCA cases were further subclassified into hepatocyte nuclear factor 1α inactivated (29%), inflammatory (32%), inflammatory with β-catenin activation (3%), noninflammatory β-catenin activated (0%), and unclassified (36%).
|
24746201 |
2014 |
|
Hepatocellular Adenoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
We described a novel c.618G>A, p.W206X mutation in HNF1α associated with MODY 3 but not with hepatocellular adenoma.
|
24642958 |
2014 |
|
Hepatocellular Adenoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
Key features include metabolic alterations (induced by defects in HNF1A), oncogene-induced inflammation (activation of JAK-STAT signaling in inflammatory adenomas), and an association between activation of Wnt/β-catenin signaling and progression of HCAs in hepatocellular carcinomas.
|
23485860 |
2013 |
|
Hepatocellular Adenoma
|
0.700 |
Biomarker
|
disease |
BEFREE |
The histological features of these tumors were similar to those of focal nodular hyperplasia (FNH) with a central scar and HCA; however, these tumors were diagnosed as HNF-1α-inactivated HCA by immunohistochemistry according to the criteria of the current World Health Organization (WHO) classification.
|
23865574 |
2013 |
|
Hepatocellular Adenoma
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Maturity onset diabetes of the young type 3 (MODY3) and hepatocellular adenomas (HCAs) are associated with mutations in the HNF1A gene.
|
23707370 |
2013 |
|
Hepatocellular Adenoma
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
In contrast, no HNF1A inactivation was observed, showing a different molecular subtype distribution in GSD-associated HCA from that observed in sporadic HCA (p = 0.0008).
|
23046672 |
2013 |