Genetic alterations in hepatocellular adenomas: recent findings and new challenges

Zucman–Rossi, Jessica

doi:10.1016/j.jhep.2004.03.012

Cited by 31 publications

(10 citation statements)

References 36 publications

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“…In a few cases, there is a b-catenin activation with or without b-catenin mutation (6,20). No other recurrent genetic alteration has been identified so far (21). Our case corresponds to a nonfamilial LA without HNF-1a mutation or b-catenin activation.…”

Section: Discussionmentioning

confidence: 69%

Regressive liver adenomatosis following androgenic progestin therapy withdrawal: a case report with a 10-year follow-up and a molecular analysis

Svrcek¹,

Jeannot²,

Arrivé³

et al. 2007

eur j endocrinol

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Objective: The relationship between sex hormones and hepatocellular adenoma development is well established. On the contrary, their contribution to liver adenomatosis (LA) development is still a debatable issue. Recently, inactivating mutations of hepatocyte nuclear factor-1a (HNF-1a) transcription factor gene or activating mutations of b-catenin have been demonstrated in some liver adenomas, and a possible link between HNF-1a gene mutations and oral contraceptives has been suggested. Only two cases of regressive LA after hormone withdrawal therapy have been described so far but without any information concerning the molecular characteristics of the tumours. Case: We report the case of a 48-year-old woman with LA, who had been taking an androgenic progestin therapy (lynestrenol) for 10 years. A major regression in the number and size of the lesions was observed 6 months after complete withdrawal of this therapy. Methods: Hepatocellular adenomas were studied by immunohistochemistry for oestrogen, progesterone and androgen receptors (ER, PR and AR respectively), and for b-catenin. Direct sequencing of the HNF-1a gene was also performed. Results: For the first time, we demonstrate significant immunostaining of AR in the hepatocellular adenomas. This staining was negative in the partially regressive adenoma. Immunostainings for ER and PR were negative. HNF-1a and the b-catenin pathways were not involved in tumour pathogenesis. Conclusions: Our case suggests a role of androgenic progestin therapy in some cases of LA. Hormone therapy withdrawal may induce a significant regression in lesions.European Journal of Endocrinology 156 617-621

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Section: Discussionmentioning

confidence: 69%

Regressive liver adenomatosis following androgenic progestin therapy withdrawal: a case report with a 10-year follow-up and a molecular analysis

Svrcek¹,

Jeannot²,

Arrivé³

et al. 2007

eur j endocrinol

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“…Up to 50 % of patients are asymptomatic. HCAs can be complicated by life-threatening bleeding or undergo malignant transformation necessitating surgical management [ 36 ].…”

Section: Hepatocellular Adenoma (Hca)mentioning

confidence: 99%

“…Often these are incidentally discovered on CT performed for other reasons and are asymptomatic. An association with maturity -onset diabetes of the young (MODY), type 3 and familial hepatic adenomatosis has been reported [ 36 , 43 , 44 ].…”

Section: Hnf-1α-mutated Hcasmentioning

confidence: 99%

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Current updates on the molecular genetics and magnetic resonance imaging of focal nodular hyperplasia and hepatocellular adenoma

et al. 2015

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Focal nodular hyperplasia (FNH) and hepatocellular adenomas (HCAs) constitute benign hepatic neoplasms in adults. HCAs are monoclonal neoplasms characterised by an increased predilection to haemorrhage and also malignant transformation. On the other hand, FNH is a polyclonal tumour-like lesion that occurs in response to increased perfusion and has an uneventful clinical course. Recent advances in molecular genetics and genotype-phenotype correlation in these hepatocellular neoplasms have enabled a new classification system. FNHs are classified into the typical and atypical types based on histomorphological and imaging features. HCAs have been categorised into four subtypes: (1) HCAs with HNF-1α mutations are diffusely steatotic, do not undergo malignant transformation, and are associated with familial diabetes or adenomatosis. (2) Inflammatory HCAs are hypervascular with marked peliosis and a tendency to bleed. They are associated with obesity, alcohol and hepatic steatosis. (3) HCAs with β-catenin mutations are associated with male hormone administration and glycogen storage disease, frequently undergo malignant transformation and may simulate hepatocellular carcinoma on imaging. (4) The final type is unclassified HCAs. Each of these except the unclassified subtype has a few distinct imaging features, often enabling reasonably accurate diagnosis. Biopsy with immunohistochemical analysis is helpful in difficult cases and has strong implications for patient management.Teaching points• FNHs are benign polyclonal neoplasms with no risk of haemorrhage or malignancy.• HCAs are benign monoclonal neoplasms classified into four subtypes based on immunohistochemistry.• Inflammatory HCAs show an atoll sign with a risk of bleeding and malignant transformation.• HNF-1α HCAs are steatotic HCAs with minimal complications and the best prognosis.• β-Catenin HCA shows variable MRI features and a high risk of malignancy.

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“…The risk of malignant transformation was a matter of debate. With the new millennium, started a series of in-depth molecular investigations which definitively changed our understanding of this tumour group, with major consequences on their clinical management and their understanding 3. From what was previously seen as a homogeneous entity, rapidly emerged a much more complex picture 4.…”

mentioning

confidence: 99%

Hepatocellular adenomas: one step beyond

Scoazec

2019

Gut

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Genetic alterations in hepatocellular adenomas: recent findings and new challenges

Cited by 31 publications

References 36 publications

Regressive liver adenomatosis following androgenic progestin therapy withdrawal: a case report with a 10-year follow-up and a molecular analysis

Regressive liver adenomatosis following androgenic progestin therapy withdrawal: a case report with a 10-year follow-up and a molecular analysis

Current updates on the molecular genetics and magnetic resonance imaging of focal nodular hyperplasia and hepatocellular adenoma

Hepatocellular adenomas: one step beyond

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