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HNF1alpha-Inactivated Hepatocellular Adenoma

MedGen UID:
474641
•Concept ID:
C3273008
•
Neoplastic Process

Definition

A hepatocellular adenoma caused by inactivating mutations of the HNF1A gene. It is characterized by the presence of lobulated contours, steatosis, and absence of inflammation or nuclear atypia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHNF1alpha-Inactivated Hepatocellular Adenoma

Professional guidelines

PubMed

Fat in mass in primary liver lesions: are usual MRI diagnostic criteria of both malignant and benign liver lesions still applicable?
Reizine E, Meurgey A, Amaddeo G, Laurent A, Calderaro J, Mule S, Luciani A
Abdom Radiol (NY) 2022 Jul;47(7):2347-2355. Epub 2022 Jun 7 doi: 10.1007/s00261-022-03561-x. PMID: 35672475
Hepatocellular adenomas in the Turkish population: reclassification according to updated World Health Organization criteria.
Deniz K, Umetsu SE, Ferrell L, Yılmaz F, Güllüoğlu M, Sağol Ö, Doğusoy GB, Kırımlıoğlu H, Turhan N, Doran F, Kepil N, Çelikel ÇA, Nart D, Özgüven BY, Ceyran B, Karadağ N, Kır G, Erden E, Yılmaz G, Akyol G
Histopathology 2021 Jul;79(1):23-33. Epub 2021 Apr 12 doi: 10.1111/his.14330. PMID: 33406290
Subclassification of hepatocellular adenomas: practical considerations in the implementation of the Bordeaux criteria.
Miller GC, Campbell CM, Manoharan B, Bryant R, Cavallucci D, O'Rourke N, Clouston AD
Pathology 2018 Oct;50(6):593-599. Epub 2018 Aug 24 doi: 10.1016/j.pathol.2018.05.003. PMID: 30149989

Recent clinical studies

Diagnosis

Repeat surgery in HNF1alpha-inactivated adenomatosis.
Balabaud C, Laurent C, Frulio N, Marie Christine SP, Le Bail B, Possenti L, Blanc JF, Chiche L, Bioulac-Sage P
Clin Res Hepatol Gastroenterol 2019 Aug;43(4):460-467. Epub 2019 Mar 20 doi: 10.1016/j.clinre.2018.11.001. PMID: 30902584
Unexpected discovery of small HNF1α-inactivated hepatocellular adenoma in pathological specimens from patients resected for liver tumours.
Balabaud C, Laurent C, Le Bail B, Castain C, Possenti L, Frulio N, Chiche L, Blanc JF, Bioulac-Sage P
Liver Int 2018 Jul;38(7):1273-1279. Epub 2018 Jan 17 doi: 10.1111/liv.13667. PMID: 29265678

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