Myxoid hepatocellular adenoma, a rare variant of hepatocellular adenoma with distinct imaging features: A case report with immunohistochemical and molecular analysis and literature review

Vos, Nicolas De; Meulen, Joni Van der; Linden, Malaïka Van Der; Claes, Kathleen; Candaele, Ann-Sophie; Vanlander, Aude; Troisi, Roberto; Vlierberghe, Hans Van; Smeets, Peter; Dorpe, Jo Van; Hoorens, Anne

doi:10.1016/j.clinre.2020.06.004

Cited by 5 publications

(9 citation statements)

References 14 publications

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“…In addition, 3 of 5 cases showed features of malignant transformation. Previous studies have also shown that myxoid hepatic adenomas consistently have a loss of LFABP expression 13–15. Our data, in conjunction with recent molecular findings,16 suggests that the myxoid hepatic adenoma is a unique morphologic variant that develops in the setting of HNF1A inactivation.…”

Section: Discussionsupporting

confidence: 84%

“…Previous studies have also shown that myxoid hepatic adenomas consistently have a loss of LFABP expression. [13][14][15] Our data, in conjunction with recent molecular findings, 16 suggests that the myxoid hepatic adenoma is a unique morphologic variant that develops in the setting of HNF1A inactivation. It remains unclear, however, if the myxoid hepatic adenomas should be classified with typical HNF1A-inactivated adenomas.…”

Section: Discussionsupporting

confidence: 67%

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Hepatic Adenomas in Patients 60 and Older Are Enriched forHNF1AInactivation and Malignant Transformation

Yasir

Chen

Jain

et al. 2022

American Journal of Surgical Pathology

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Hepatic adenomas occur most commonly in women between the ages of 20 and 40 years, but rarely they occur in older aged persons, including those 60 years of age or older. This group of adenomas, however, has not been systemically examined. Twenty-six hepatic adenomas in persons 60 years of age or older were studied, along with a control group of 50 hepatic adenomas in persons aged 30 to 39. Hepatic adenomas in persons 60 or more years of age were found in 21 women and 5 men, while the control group had 44 women and 6 men. Subtyping the adenomas in persons 60 years or older showed the following results: 18 HNF1A-inactivated adenomas (69%), 4 inflammatory adenomas (15%), and 4 unclassified adenomas (15%). In contrast, the control group showed a significantly different pattern (P=0.003), with a greater percentage of inflammatory adenomas (28, 56%), fewer HNF1A-inactivated adenomas (8, 16%), and more unclassified adenomas (14, 28%). Atypia and malignant transformation within the hepatic adenomas was studied next. Of the hepatic adenomas in persons age 60 or greater, 3 (12%) showed atypical histologic features, and 6 (23%) had a malignant transformation. In contrast, for hepatic adenomas in the control group, only 4 (8%) adenomas showed atypical histologic features, and 3 (6%) had undergone malignant transformation. In addition, the hepatic adenomas that were atypical or showed early malignant transformation were less likely to have beta-catenin activation in patients over 60 (2/9 cases) compared with those between 30 and 39 years (5/7 cases). Myxoid change and heavy lipofuscin deposition were also more common in adenomas in older aged persons. In conclusion, hepatic adenomas in persons 60 years of age or older are enriched for HNF1A-inactivated adenomas and have a higher frequency of malignant transformation. Malignant transformation, however, is less likely to develop through activation of the beta-catenin pathway.

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Section: Discussionsupporting

confidence: 84%

Section: Discussionsupporting

confidence: 67%

Hepatic Adenomas in Patients 60 and Older Are Enriched forHNF1AInactivation and Malignant Transformation

Yasir

Chen

Jain

et al. 2022

American Journal of Surgical Pathology

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“…Myxoid changes can be present to a variable extent, and many of these cases show HNF1A abnormalities (e.g. heterozygous and monoallelic) and concurrent variable abnormalities of other genes 61,62 …”

Section: Hca Classificationmentioning

confidence: 99%

“…heterozygous and monoallelic) and concurrent variable abnormalities of other genes. 61,62 Loss of liver fatty acid-binding protein (LFABP) is the hallmark of H-HCA, but lack of LFABP in a hepatic focal lesion does not automatically lead to the diagnosis of H-HCA. Loss or diminished expression of LFABP can be seen in HCC without any association with HCA.…”

Section: H -H C Amentioning

confidence: 99%

Hepatocellular adenoma: what we know, what we do not know, and why it matters

et al. 2022

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Hepatocellular adenoma: what we know, what we do not know, and why it mattersIn the last two decades there has been significant progress in research on and diagnosis of hepatocellular adenoma (HCA), resulting in the establishment of a molecular and immunohistological HCA classification. This review aims to fine-tune the current expertise in order to enhance the histopathological diagnostic possibilities, by refining issues that are already known, addressing diagnostic difficulties, and identifying still unknown aspects of HCA. We discuss novel methods to identify HCA subtypes, in particular the sonic hedgehog HCAs and the interpretation of glutamine synthetase patterns for the recognition of b-cateninmutated HCAs. The major complications of HCAs, i.e. bleeding and malignant transformation, are considered, including the dilemmas of atypical and borderline lesions. HCAs in different clinical and geographical settings, e.g. pregnancy, cirrhosis and non-western countries, are also discussed. The natural history of the different HCA subtypes in relation to age, sex and risk factors is a feature that is still insufficiently investigated. This is also true for the risks of clinical bleeding and malignant transformation in association with HCA subtypes. As HCA is a relatively rare tumour, a multicentre and multidisciplinary approach across geographical boundaries will be the appropriate method to establish prospective programmes with which to identify, classify and manage HCAs, focusing on several aspects, e.g. aetiology, underlying liver disease, complications, regression, and growth. Updating what we know and identifying and addressing what we do not know matters for optimal patient management.

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“…1 The imaging findings are also unique, reflecting the distinctive deposition of myxoid material. 2,3 Myxoid hepatic adenomas have an increased risk of malignant transformation, 1,4 so their recognition is important for patient management. 5 Myxoid hepatic adenomas characteristically show loss of liver fatty acid-binding protein (LFABP) by immunohistochemistry, 1 presumably through inactivation of hepatocyte nuclear factor 1 alpha (HNF1A), although the precise mechanism is not known.…”

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confidence: 99%

Morphologic and Molecular Findings in Myxoid Hepatic Adenomas

Rowan

Yasir

Chen

et al. 2021

American Journal of Surgical Pathology

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Myxoid hepatic adenomas are a rare subtype of hepatic adenomas with distinctive deposition of extracellular myxoid material between the hepatic plates. A total of 9 cases were identified in 6 women and 3 men with an average of 59±12 years. The myxoid adenomas were single tumors in 5 cases and multiple in 4 cases. In 1 case with multiple adenomas, the myxoid adenoma arose in the background of GNAS-mutated hepatic adenomatosis. Myxoid hepatic adenomas had a high frequency of malignant transformation (N=5 cases). They were characterized at the molecular level by HNF1A inactivating mutations, leading to loss of LFABP protein expression. In addition, myxoid adenomas had recurrent mutations in genes within the protein kinase A (PKA) pathway or in genes that regulate the PKA pathway: GNAS, CDKN1B (encodes p27), and RNF123. In sum, myxoid adenomas are rare, occur in older-aged persons, have a high risk of malignant transformation, and are characterized by the combined inactivation of HNF1A and additional mutations that appear to cluster in the PKA pathway.

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Myxoid hepatocellular adenoma, a rare variant of hepatocellular adenoma with distinct imaging features: A case report with immunohistochemical and molecular analysis and literature review

Cited by 5 publications

References 14 publications

Hepatic Adenomas in Patients 60 and Older Are Enriched forHNF1AInactivation and Malignant Transformation

Hepatic Adenomas in Patients 60 and Older Are Enriched forHNF1AInactivation and Malignant Transformation

Hepatocellular adenoma: what we know, what we do not know, and why it matters

Morphologic and Molecular Findings in Myxoid Hepatic Adenomas

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