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Hepatocyte Nuclear Factor 1α-Mutated Hepatocellular Adenomas: An Atypical Presentation

Hepatocellular adenomas (HCAs) are rare benign monoclonal hepatic tumors that commonly occur in females (3–4 per 100,000 women) due to the use of oral contraceptives, its primary risk factor. Recently, HCAs have been classified into 4 distinct subtypes according to genotypic and phenotypic character...

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Autores principales: Sousa, Camila Maria Neves, Gadelha, Patricia Maria Miranda, Cartaxo, Rafaella de Sousa, Pedrosa, George Washington Holanda, Honorio, Rodrigo Schuler, Lima, José Milton de Castro, Coelho, Gustavo Rêgo, Coelho, Clovis Rêgo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5731146/
https://www.ncbi.nlm.nih.gov/pubmed/29282384
http://dx.doi.org/10.1159/000480376
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author Sousa, Camila Maria Neves
Gadelha, Patricia Maria Miranda
Cartaxo, Rafaella de Sousa
Pedrosa, George Washington Holanda
Honorio, Rodrigo Schuler
Lima, José Milton de Castro
Coelho, Gustavo Rêgo
Coelho, Clovis Rêgo
author_facet Sousa, Camila Maria Neves
Gadelha, Patricia Maria Miranda
Cartaxo, Rafaella de Sousa
Pedrosa, George Washington Holanda
Honorio, Rodrigo Schuler
Lima, José Milton de Castro
Coelho, Gustavo Rêgo
Coelho, Clovis Rêgo
author_sort Sousa, Camila Maria Neves
collection PubMed
description Hepatocellular adenomas (HCAs) are rare benign monoclonal hepatic tumors that commonly occur in females (3–4 per 100,000 women) due to the use of oral contraceptives, its primary risk factor. Recently, HCAs have been classified into 4 distinct subtypes according to genotypic and phenotypic characteristics and clinical features: inflammatory HCA (40–50%), which are hypervascular with marked peliosis and a tendency to bleed; hepatocyte nuclear factor 1α (HNF1A)-mutated HCA (H-HCA, 30–40%) that are diffusely steatotic and rarely undergo malignant transformation; β-catenin activated HCA (10–15%), which frequently undergo malignant transformation and may seem hepatocellular carcinoma on imaging; and unclassified HCA (10–25%). In this study, we report the case of a 23-year-old female oral contraceptive user with H-HCA. Usually, H-HCA is considered to be nonsevere in most cases and often requires outpatient follow-up. However, in this case, the injury had substantially increased in volume and evolved with a major bleeding frame, which was an unusual finding for this subtype of adenoma. The therapeutic used for this patient was a laparoscopic left hepatic segmentectomy. Thus, the choice of treatment to be performed in a patient with H-HCA can depend on the tumor size (>5 cm), the outcome of previous bleeding, and the risk of bleeding recurrence.
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spelling pubmed-57311462017-12-27 Hepatocyte Nuclear Factor 1α-Mutated Hepatocellular Adenomas: An Atypical Presentation Sousa, Camila Maria Neves Gadelha, Patricia Maria Miranda Cartaxo, Rafaella de Sousa Pedrosa, George Washington Holanda Honorio, Rodrigo Schuler Lima, José Milton de Castro Coelho, Gustavo Rêgo Coelho, Clovis Rêgo Case Rep Gastroenterol Single Case Hepatocellular adenomas (HCAs) are rare benign monoclonal hepatic tumors that commonly occur in females (3–4 per 100,000 women) due to the use of oral contraceptives, its primary risk factor. Recently, HCAs have been classified into 4 distinct subtypes according to genotypic and phenotypic characteristics and clinical features: inflammatory HCA (40–50%), which are hypervascular with marked peliosis and a tendency to bleed; hepatocyte nuclear factor 1α (HNF1A)-mutated HCA (H-HCA, 30–40%) that are diffusely steatotic and rarely undergo malignant transformation; β-catenin activated HCA (10–15%), which frequently undergo malignant transformation and may seem hepatocellular carcinoma on imaging; and unclassified HCA (10–25%). In this study, we report the case of a 23-year-old female oral contraceptive user with H-HCA. Usually, H-HCA is considered to be nonsevere in most cases and often requires outpatient follow-up. However, in this case, the injury had substantially increased in volume and evolved with a major bleeding frame, which was an unusual finding for this subtype of adenoma. The therapeutic used for this patient was a laparoscopic left hepatic segmentectomy. Thus, the choice of treatment to be performed in a patient with H-HCA can depend on the tumor size (>5 cm), the outcome of previous bleeding, and the risk of bleeding recurrence. S. Karger AG 2017-10-30 /pmc/articles/PMC5731146/ /pubmed/29282384 http://dx.doi.org/10.1159/000480376 Text en Copyright © 2017 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/4.0/ This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.
spellingShingle Single Case
Sousa, Camila Maria Neves
Gadelha, Patricia Maria Miranda
Cartaxo, Rafaella de Sousa
Pedrosa, George Washington Holanda
Honorio, Rodrigo Schuler
Lima, José Milton de Castro
Coelho, Gustavo Rêgo
Coelho, Clovis Rêgo
Hepatocyte Nuclear Factor 1α-Mutated Hepatocellular Adenomas: An Atypical Presentation
title Hepatocyte Nuclear Factor 1α-Mutated Hepatocellular Adenomas: An Atypical Presentation
title_full Hepatocyte Nuclear Factor 1α-Mutated Hepatocellular Adenomas: An Atypical Presentation
title_fullStr Hepatocyte Nuclear Factor 1α-Mutated Hepatocellular Adenomas: An Atypical Presentation
title_full_unstemmed Hepatocyte Nuclear Factor 1α-Mutated Hepatocellular Adenomas: An Atypical Presentation
title_short Hepatocyte Nuclear Factor 1α-Mutated Hepatocellular Adenomas: An Atypical Presentation
title_sort hepatocyte nuclear factor 1α-mutated hepatocellular adenomas: an atypical presentation
topic Single Case
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5731146/
https://www.ncbi.nlm.nih.gov/pubmed/29282384
http://dx.doi.org/10.1159/000480376
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