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Jan N.m. Ijzermans - One of the best experts on this subject based on the ideXlab platform.
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New insights in the management of Hepatocellular Adenoma.
Liver international : official journal of the International Association for the Study of the Liver, 2020Co-Authors: Anne J. Klompenhouwer, Rob A. De Man, Jessica Zucman-rossi, Marco Dioguardi Burgio, Valérie Vilgrain, Jan N.m. IjzermansAbstract:Hepatocellular Adenoma (HCA) are benign liver tumours that may be complicated by haemorrhage or malignant transformation to Hepatocellular carcinoma. Epidemiological data are fairly outdated, but it is likely to assume that the incidence has increased over the past decades as HCA are more often incidentally found due to the more widespread use of imaging techniques and the increased incidence of obesity. Various molecular subgroups have been described. Each of these molecular subgroups are defined by specific gene mutations and pathway activations. Additionally, they are all related to specific risk factors and show a various biological behaviour. These molecular subgroups may be identified using immunohistochemistry and molecular characterization. Contrast-enhanced MRI is the recommended imaging modality to analyse patients with suspected Hepatocellular Adenoma allowing to determine the subtype in up to 80%. Surgical resection remains to be the golden standard in treating HCA, although resection is deemed unnecessary in a large number of cases, as studies have shown that the majority of HCA will regress over time without complications such as haemorrhage or malignant transformation occurring. It is preferable to treat patients with suspected HCA in high volume centres with combined expertise of liver surgeons, hepatologists, radiologists and (molecular) pathologists.
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Growth of Hepatocellular Adenoma during pregnancy: A prospective study.
Journal of hepatology, 2019Co-Authors: M. Gaspersz, Susanna M. Van Aalten, Rob A. De Man, Türkan Terkivatan, Anne J. Klompenhouwer, Mirelle Elmira Elizabeth Broker, Maarten Gerardus Josephus Thomeer, Eric A.p. Steegers, Harry J. De Koning, Jan N.m. IjzermansAbstract:Background & Aims The presence of Hepatocellular Adenoma (HCA) in pregnant women requires special consideration, as it has been reported to carry the risk of growth and clinically significant haemorrhage. In this prospective study we assessed aspects of growth of HCA Methods This was a multicentre prospective cohort study in pregnant women with suspected HCA 20%) was closely monitored with ultrasound examinations throughout pregnancy. Results Of the 66 women included, 18 were excluded from analysis because postpartum LCE-MRI did not confirm the diagnosis of HCA and showed the lesion to be focal nodular hyperplasia. The remaining 48 women, with an HCA confirmed by LCE-MRI, were followed during 51 pregnancies. Median age was 30 years (IQR 27–33) and median body mass index 31.9 kg/m2 (IQR 26.3–36.6). Growth of HCA was seen in 13 of the pregnancies (25.5%); the median increase was 14 mm (IQR 8–19). One woman whose HCA grew to >70 mm successfully underwent transarterial embolization at week 26 of pregnancy to prevent further growth. The other 50 pregnancies proceeded without complications. Conclusion This study suggests that an HCA Lay summary The presence of Hepatocellular Adenoma in pregnant women requires special consideration, as it carries the risk of growth and haemorrhage. In this study we followed 48 patients with Hepatocellular Adenoma
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evidence of good prognosis of Hepatocellular Adenoma in post menopausal women
Journal of Hepatology, 2016Co-Authors: Anne J. Klompenhouwer, Jan N.m. Ijzermans, M. Gaspersz, Dave Sprengers, Francois E J A Willemssen, Rob A. De ManAbstract:Background & Aims Hepatocellular Adenoma (HCA) is a rare benign liver tumor, which typically develops in women in their reproductive phase and is associated with the use of oral contraceptives. The aim of this study was to evaluate whether follow-up of HCA can be safely terminated after the occurrence of menopause. Secondary, we studied the impact of the diagnosis HCA on health-related quality of life (HRQoL). Methods This was a cross-sectional cohort study, including 48 post-menopausal women with HCA. Patients underwent ultrasound examination and the size of HCA was compared to size at the last follow-up imaging (CT, MRI or ultrasound). HRQoL was evaluated by the Liver Disease Symptom Index 2.0 and Short Form 12. Results Median time since last follow-up was 60.5months. In 44 patients 43.5% of the lesions were undetectable, 32.6% were stable in size and 19.6% became smaller. Mean diameter of HCA was 17.2mm compared to 35.9mm at last follow-up ( p p Conclusions HCA diameter became significantly smaller after the occurrence of menopause and as time progresses, this regression increased. This suggests that routine follow-up of HCA Lay summary In this study we investigated if Hepatocellular Adenoma, a benign tumor of the liver that is found mostly in women and is associated with female hormones, regresses in size after the occurrence of menopause in female patients over 50years of age. We made an ultrasound of the liver lesion and found that the average size of the Adenomas becomes significantly smaller. This could mean that female patients with a small ( Clinical trial number MEC-2015-385.
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Hepatocellular Adenoma when and how to treat update of current evidence
Therapeutic Advances in Gastroenterology, 2016Co-Authors: Maarten Thomeer, Rob A. De Man, Türkan Terkivatan, Mirelle E E Broker, Joanne Verheij, Michael Doukas, Diederick Bijdevaate, Adriaan Moelker, Jan N.m. IjzermansAbstract:Hepatocellular Adenoma (HCA) is a rare, benign liver tumor. Discovery of this tumor is usually as an incidental finding, correlated with the use of oral contraceptives, or pregnancy. Treatment options have focused on conservative management for the straightforward, smaller lesions ( 5 cm) that pose a greater risk of hemorrhage or malignant progression. In recent years, a new molecular subclassification of HCA has been proposed, associated with characteristic morphological features and loss or increased expression of immunohistochemical markers. This subclassification could possibly provide considerable benefits in terms of patient stratification, and the selection of treatment options. In this review we discuss the decision-making processes and associated risk analyses that should be made based on lesion size, and subtype. The usefulness of this subclassification system in terms of the procedures instigated as part of the diagnostic work-up of a suspected HCA will be outlined, and suitable treatment schemes proposed.
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genotype phenotype correlations in Hepatocellular Adenoma an update of mri findings
Diagnostic and Interventional Radiology, 2014Co-Authors: Maarten Thomeer, Rob A. De Man, Jan N.m. Ijzermans, Roy S. Dwarkasing, Mirelle E E Broker, Quido De Lussanet, Katharina Biermann, Marianne De VriesAbstract:Hepatocellular Adenoma (HCA) is a generally benign liver tumor with the potential for malignancy and bleeding. 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. Magnetic resonance imaging (MRI) plays an important role in the diagnosis, subtype characterization, and detection of HCA complications; it is also used to differentiate HCA from focal nodular hyperplasia. In this review, we present an overview of the genetic abnormalities, oncogenesis, and typical and atypical MRI findings of specific subtypes of HCA using contrast-enhanced MRI with or without hepatobiliary contrast agents (gadobenate dimeglumine and gadoxetate disodium). We also discuss their different management implications after diagnosis.
Sanjay Kakar - One of the best experts on this subject based on the ideXlab platform.
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correlation of exon 3 β catenin mutations with glutamine synthetase staining patterns in Hepatocellular Adenoma and Hepatocellular carcinoma
Modern Pathology, 2016Co-Authors: Gillian Hale, Xinxin Liu, Li Che, David A Solomon, Christos G Tsokos, Nafis Shafizadeh, Xin Chen, Ryan M Gill, Sanjay KakarAbstract:The current clinical practice is based on the assumption of strong correlation between diffuse glutamine synthetase expression and β-catenin activation in Hepatocellular Adenoma and Hepatocellular carcinoma. This high correlation is based on limited data and may represent an oversimplification as glutamine synthetase staining patterns show wide variability in clinical practice. Standardized criteria for interpreting diverse glutamine synthetase patterns, and the association between each pattern and β-catenin mutations is not clearly established. This study examines the correlation between glutamine synthetase staining patterns and β-catenin mutations in 15 typical Hepatocellular Adenomas, 5 atypical Hepatocellular neoplasms and 60 Hepatocellular carcinomas. Glutamine synthetase staining was classified into one of the three patterns: (a) diffuse homogeneous: moderate-to-strong cytoplasmic staining in >90% of lesional cells, without a map-like pattern, (b) diffuse heterogeneous: moderate-to-strong staining in 50-90% of lesional cells, without a map-like pattern, and (c) patchy: moderate-to-strong staining in 50% in both Hepatocellular Adenoma and Hepatocellular carcinoma. The interpretation of β-catenin activation based on glutamine synthetase staining should be performed with caution, and the undetermined significance of various glutamine synthetase patterns should be highlighted in pathology reports.
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diagnostic utility and limitations of glutamine synthetase and serum amyloid associated protein immunohistochemistry in the distinction of focal nodular hyperplasia and inflammatory Hepatocellular Adenoma
Modern Pathology, 2014Co-Authors: Nancy M Joseph, Matthew M. Yeh, Linda D Ferrell, Michael Torbenson, Dhanpat Jain, Sanjay KakarAbstract:Inflammatory Hepatocellular Adenoma can show overlapping histological features with focal nodular hyperplasia, including inflammation, fibrous stroma, and ductular reaction. Expression of serum amyloid-associated protein in inflammatory Hepatocellular Adenoma and map-like pattern of glutamine synthetase in focal nodular hyperplasia can be helpful in this distinction, but the pitfalls and limitations of these markers have not been established. Morphology and immunohistochemistry were analyzed in 54 inflammatory Hepatocellular Adenomas, 40 focal nodular hyperplasia, and 3 indeterminate lesions. Morphological analysis demonstrated that nodularity, fibrous stroma, dystrophic blood vessels, and ductular reaction were more common in focal nodular hyperplasia, while telangiectasia, hemorrhage, and steatosis were more common in inflammatory Hepatocellular Adenoma, but there was frequent overlap of morphological features. The majority of inflammatory Hepatocellular Adenomas demonstrated perivascular and/or patchy glutamine synthetase staining (73.6%), while the remaining cases had diffuse (7.5%), negative (3.8%), or patchy pattern of staining (15%) that showed subtle differences from the classic map-like staining pattern and was designated as pseudo map-like staining. Positive staining for serum amyloid-associated protein was seen in the majority of inflammatory Hepatocellular Adenomas (92.6%) and in the minority of focal nodular hyperplasia (17.5%). The glutamine synthetase staining pattern was map-like in 90% of focal nodular hyperplasia cases, with the remaining 10% of cases showing pseudo map-like staining. Three cases were labeled as indeterminate and showed focal nodular hyperplasia-like morphology but lacked map-like glutamine synthetase staining pattern; these cases demonstrated a patchy pseudo map-like glutamine synthetase pattern along with the expression of serum amyloid-associated protein. Our results highlight the diagnostic errors that can be caused by variant patterns of staining with glutamine synthetase and serum amyloid-associated protein in inflammatory Hepatocellular Adenoma and focal nodular hyperplasia.
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atypical Hepatocellular Adenoma like neoplasms with β catenin activation show cytogenetic alterations similar to well differentiated Hepatocellular carcinomas
Human Pathology, 2013Co-Authors: Kimberley J Evason, James P Grenert, Linda D Ferrell, Sanjay KakarAbstract:The distinction of Hepatocellular Adenoma from well-differentiated Hepatocellular carcinoma (HCC) arising in noncirrhotic liver can be challenging, particularly when tumors histologically resembling Hepatocellular Adenoma occur in unusual clinical settings such as in a man or an older woman or show focal atypical morphologic features. In this study, we examine the morphologic, immunohistochemical, and cytogenetic features of Hepatocellular Adenoma-like neoplasms occurring in men, women 50 years or older or younger than 15 years, and/or those with focal atypia (small cell change, pseudogland formation, and/or nuclear atypia), designated atypical Hepatocellular neoplasms, where the distinction of Hepatocellular Adenoma versus HCC could not be clearly established. Immunohistochemistry was performed for β-catenin, glutamine synthetase, and serum amyloid A in 31 Hepatocellular Adenomas, 20 well-differentiated HCCs, and 40 atypical Hepatocellular neoplasms. Chromosomal gains/losses had previously been determined in 37 cases using comparative genomic hybridization or fluorescence in situ hybridization. β-Catenin activation was observed in 35% of atypical Hepatocellular neoplasms compared with 10% of typical Hepatocellular Adenomas (P < .05) and 55% of well-differentiated HCCs (P = .14). Cytogenetic changes typically observed in HCC were present in all atypical Hepatocellular neoplasms with β-catenin activation. β-Catenin activation in atypical Hepatocellular neoplasms was also associated with atypical morphologic features. Follow-up data were limited, but adverse outcome was observed in 2 atypical Hepatocellular neoplasms with β-catenin activation (1 recurrence, 1 metastasis); transition to areas of HCC was observed in 1 case. The similarity in morphologic and cytogenetic features of β-catenin-activated Hepatocellular Adenoma-like tumors and HCC suggests that the former tumors represent an extremely well-differentiated variant of HCC.
Rob A. De Man - One of the best experts on this subject based on the ideXlab platform.
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New insights in the management of Hepatocellular Adenoma.
Liver international : official journal of the International Association for the Study of the Liver, 2020Co-Authors: Anne J. Klompenhouwer, Rob A. De Man, Jessica Zucman-rossi, Marco Dioguardi Burgio, Valérie Vilgrain, Jan N.m. IjzermansAbstract:Hepatocellular Adenoma (HCA) are benign liver tumours that may be complicated by haemorrhage or malignant transformation to Hepatocellular carcinoma. Epidemiological data are fairly outdated, but it is likely to assume that the incidence has increased over the past decades as HCA are more often incidentally found due to the more widespread use of imaging techniques and the increased incidence of obesity. Various molecular subgroups have been described. Each of these molecular subgroups are defined by specific gene mutations and pathway activations. Additionally, they are all related to specific risk factors and show a various biological behaviour. These molecular subgroups may be identified using immunohistochemistry and molecular characterization. Contrast-enhanced MRI is the recommended imaging modality to analyse patients with suspected Hepatocellular Adenoma allowing to determine the subtype in up to 80%. Surgical resection remains to be the golden standard in treating HCA, although resection is deemed unnecessary in a large number of cases, as studies have shown that the majority of HCA will regress over time without complications such as haemorrhage or malignant transformation occurring. It is preferable to treat patients with suspected HCA in high volume centres with combined expertise of liver surgeons, hepatologists, radiologists and (molecular) pathologists.
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Growth of Hepatocellular Adenoma during pregnancy: A prospective study.
Journal of hepatology, 2019Co-Authors: M. Gaspersz, Susanna M. Van Aalten, Rob A. De Man, Türkan Terkivatan, Anne J. Klompenhouwer, Mirelle Elmira Elizabeth Broker, Maarten Gerardus Josephus Thomeer, Eric A.p. Steegers, Harry J. De Koning, Jan N.m. IjzermansAbstract:Background & Aims The presence of Hepatocellular Adenoma (HCA) in pregnant women requires special consideration, as it has been reported to carry the risk of growth and clinically significant haemorrhage. In this prospective study we assessed aspects of growth of HCA Methods This was a multicentre prospective cohort study in pregnant women with suspected HCA 20%) was closely monitored with ultrasound examinations throughout pregnancy. Results Of the 66 women included, 18 were excluded from analysis because postpartum LCE-MRI did not confirm the diagnosis of HCA and showed the lesion to be focal nodular hyperplasia. The remaining 48 women, with an HCA confirmed by LCE-MRI, were followed during 51 pregnancies. Median age was 30 years (IQR 27–33) and median body mass index 31.9 kg/m2 (IQR 26.3–36.6). Growth of HCA was seen in 13 of the pregnancies (25.5%); the median increase was 14 mm (IQR 8–19). One woman whose HCA grew to >70 mm successfully underwent transarterial embolization at week 26 of pregnancy to prevent further growth. The other 50 pregnancies proceeded without complications. Conclusion This study suggests that an HCA Lay summary The presence of Hepatocellular Adenoma in pregnant women requires special consideration, as it carries the risk of growth and haemorrhage. In this study we followed 48 patients with Hepatocellular Adenoma
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evidence of good prognosis of Hepatocellular Adenoma in post menopausal women
Journal of Hepatology, 2016Co-Authors: Anne J. Klompenhouwer, Jan N.m. Ijzermans, M. Gaspersz, Dave Sprengers, Francois E J A Willemssen, Rob A. De ManAbstract:Background & Aims Hepatocellular Adenoma (HCA) is a rare benign liver tumor, which typically develops in women in their reproductive phase and is associated with the use of oral contraceptives. The aim of this study was to evaluate whether follow-up of HCA can be safely terminated after the occurrence of menopause. Secondary, we studied the impact of the diagnosis HCA on health-related quality of life (HRQoL). Methods This was a cross-sectional cohort study, including 48 post-menopausal women with HCA. Patients underwent ultrasound examination and the size of HCA was compared to size at the last follow-up imaging (CT, MRI or ultrasound). HRQoL was evaluated by the Liver Disease Symptom Index 2.0 and Short Form 12. Results Median time since last follow-up was 60.5months. In 44 patients 43.5% of the lesions were undetectable, 32.6% were stable in size and 19.6% became smaller. Mean diameter of HCA was 17.2mm compared to 35.9mm at last follow-up ( p p Conclusions HCA diameter became significantly smaller after the occurrence of menopause and as time progresses, this regression increased. This suggests that routine follow-up of HCA Lay summary In this study we investigated if Hepatocellular Adenoma, a benign tumor of the liver that is found mostly in women and is associated with female hormones, regresses in size after the occurrence of menopause in female patients over 50years of age. We made an ultrasound of the liver lesion and found that the average size of the Adenomas becomes significantly smaller. This could mean that female patients with a small ( Clinical trial number MEC-2015-385.
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Hepatocellular Adenoma when and how to treat update of current evidence
Therapeutic Advances in Gastroenterology, 2016Co-Authors: Maarten Thomeer, Rob A. De Man, Türkan Terkivatan, Mirelle E E Broker, Joanne Verheij, Michael Doukas, Diederick Bijdevaate, Adriaan Moelker, Jan N.m. IjzermansAbstract:Hepatocellular Adenoma (HCA) is a rare, benign liver tumor. Discovery of this tumor is usually as an incidental finding, correlated with the use of oral contraceptives, or pregnancy. Treatment options have focused on conservative management for the straightforward, smaller lesions ( 5 cm) that pose a greater risk of hemorrhage or malignant progression. In recent years, a new molecular subclassification of HCA has been proposed, associated with characteristic morphological features and loss or increased expression of immunohistochemical markers. This subclassification could possibly provide considerable benefits in terms of patient stratification, and the selection of treatment options. In this review we discuss the decision-making processes and associated risk analyses that should be made based on lesion size, and subtype. The usefulness of this subclassification system in terms of the procedures instigated as part of the diagnostic work-up of a suspected HCA will be outlined, and suitable treatment schemes proposed.
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genotype phenotype correlations in Hepatocellular Adenoma an update of mri findings
Diagnostic and Interventional Radiology, 2014Co-Authors: Maarten Thomeer, Rob A. De Man, Jan N.m. Ijzermans, Roy S. Dwarkasing, Mirelle E E Broker, Quido De Lussanet, Katharina Biermann, Marianne De VriesAbstract:Hepatocellular Adenoma (HCA) is a generally benign liver tumor with the potential for malignancy and bleeding. 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. Magnetic resonance imaging (MRI) plays an important role in the diagnosis, subtype characterization, and detection of HCA complications; it is also used to differentiate HCA from focal nodular hyperplasia. In this review, we present an overview of the genetic abnormalities, oncogenesis, and typical and atypical MRI findings of specific subtypes of HCA using contrast-enhanced MRI with or without hepatobiliary contrast agents (gadobenate dimeglumine and gadoxetate disodium). We also discuss their different management implications after diagnosis.
Türkan Terkivatan - One of the best experts on this subject based on the ideXlab platform.
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Growth of Hepatocellular Adenoma during pregnancy: A prospective study.
Journal of hepatology, 2019Co-Authors: M. Gaspersz, Susanna M. Van Aalten, Rob A. De Man, Türkan Terkivatan, Anne J. Klompenhouwer, Mirelle Elmira Elizabeth Broker, Maarten Gerardus Josephus Thomeer, Eric A.p. Steegers, Harry J. De Koning, Jan N.m. IjzermansAbstract:Background & Aims The presence of Hepatocellular Adenoma (HCA) in pregnant women requires special consideration, as it has been reported to carry the risk of growth and clinically significant haemorrhage. In this prospective study we assessed aspects of growth of HCA Methods This was a multicentre prospective cohort study in pregnant women with suspected HCA 20%) was closely monitored with ultrasound examinations throughout pregnancy. Results Of the 66 women included, 18 were excluded from analysis because postpartum LCE-MRI did not confirm the diagnosis of HCA and showed the lesion to be focal nodular hyperplasia. The remaining 48 women, with an HCA confirmed by LCE-MRI, were followed during 51 pregnancies. Median age was 30 years (IQR 27–33) and median body mass index 31.9 kg/m2 (IQR 26.3–36.6). Growth of HCA was seen in 13 of the pregnancies (25.5%); the median increase was 14 mm (IQR 8–19). One woman whose HCA grew to >70 mm successfully underwent transarterial embolization at week 26 of pregnancy to prevent further growth. The other 50 pregnancies proceeded without complications. Conclusion This study suggests that an HCA Lay summary The presence of Hepatocellular Adenoma in pregnant women requires special consideration, as it carries the risk of growth and haemorrhage. In this study we followed 48 patients with Hepatocellular Adenoma
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Hepatocellular Adenoma when and how to treat update of current evidence
Therapeutic Advances in Gastroenterology, 2016Co-Authors: Maarten Thomeer, Rob A. De Man, Türkan Terkivatan, Mirelle E E Broker, Joanne Verheij, Michael Doukas, Diederick Bijdevaate, Adriaan Moelker, Jan N.m. IjzermansAbstract:Hepatocellular Adenoma (HCA) is a rare, benign liver tumor. Discovery of this tumor is usually as an incidental finding, correlated with the use of oral contraceptives, or pregnancy. Treatment options have focused on conservative management for the straightforward, smaller lesions ( 5 cm) that pose a greater risk of hemorrhage or malignant progression. In recent years, a new molecular subclassification of HCA has been proposed, associated with characteristic morphological features and loss or increased expression of immunohistochemical markers. This subclassification could possibly provide considerable benefits in terms of patient stratification, and the selection of treatment options. In this review we discuss the decision-making processes and associated risk analyses that should be made based on lesion size, and subtype. The usefulness of this subclassification system in terms of the procedures instigated as part of the diagnostic work-up of a suspected HCA will be outlined, and suitable treatment schemes proposed.
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Management of Hepatocellular Adenoma during pregnancy
Journal of hepatology, 2010Co-Authors: Johanna E. Noels, Susanna M. Van Aalten, Dirk J. Van Der Windt, Niels F.m. Kok, Rob A. De Man, Türkan Terkivatan, Jan N.m. IjzermansAbstract:Background & Aims: Hepatocellular Adenoma in pregnant women requires special considerations because of the risk of hormone induced growth and rupture. To prevent these potential lethal complications, pregnancy is either often discouraged or the surgical resection of large Adenomas is recommended. It may be questioned whether it is justified to deny a young woman a pregnancy, as the biological behaviour of Hepatocellular Adenoma may be less threatening than presumed. In this study we establish the management of Hepatocellular Adenoma during pregnancy based on our own experience and literature. Methods: Twelve women with documented Hepatocellular Adenoma were closely monitored during a total of 17 pregnancies between 2000 and 2009. Their files were reviewed. Results: In four cases, Hepatocellular Adenomas grew during pregnancy, requiring a Caesarean section in one patient (two pregnancies) at 36 and 34 weeks because of an assumed high risk of rupture. In one case radiofrequency ablation therapy was applied in the first trimester to treat a hormone sensitive Hepatocellular Adenoma, thereby excluding potential growth later in pregnancy. No intervention was performed in the other 14 cases and all pregnancies had an uneventful course with a successful maternal and fetal outcome. Conclusions: A "wait and see" management may be advocated in pregnant women presenting with a Hepatocellular Adenoma. In women with large tumours or in whom Hepatocellular Adenoma had complicated previous pregnancies, surgical resection may be recommended. In women with smaller Adenomas it may no longer be necessary to discourage pregnancy.
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Diagnosis and Treatment of Hepatocellular Adenoma in the Netherlands: Similarities and Differences
Digestive surgery, 2010Co-Authors: Susanna M. Van Aalten, Dirk J. Van Der Windt, Niels F.m. Kok, Rob A. De Man, Türkan Terkivatan, Roy S. Dwarkasing, Jan N.m. IjzermansAbstract:Background: The diagnosis of Hepatocellular Adenoma (HA) has a great impact on the lives of young women and may pose clinical dilemmas to the clinician since there are no standardiz
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Treatment of ruptured Hepatocellular Adenoma
The British journal of surgery, 2001Co-Authors: Türkan Terkivatan, Rob A. De Man, J.h.w. De Wilt, R. R. Van Rijn, H. W. Tilanus, Jan N.m. IjzermansAbstract:Background: As the morbidity and mortality rates associated with emergency resection in patients with a ruptured Hepatocellular Adenoma are high, the authors have favoured initial non-operative management in haemodynamically stable patients. Methods: A retrospective study was performed to evaluate the treatment of ruptured Hepatocellular Adenoma. Results: Over a 21-year interval, 12 patients presented with a ruptured Hepatocellular Adenoma. Haemodynamic observation and support was the initial management in all 12 patients. Three underwent urgent laparotomy and gauze packing because of haemodynamic instability; no emergency liver resection was necessary. Eight patients had definitive surgery; three developed postoperative complications but none died. Regression of the tumour was observed in three of four patients treated conservatively. Conclusion: The initial management of a ruptured Hepatocellular Adenoma should be haemodynamic stabilization. Definitive resection is required for rebleeding or for tumours exceeding 5 cm in diameter. A conservative approach may well be justified in case of regression of an asymptomatic Adenoma. © 2001 British Journal of Surgery Society Ltd
Jessica Zucman-rossi - One of the best experts on this subject based on the ideXlab platform.
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New insights in the management of Hepatocellular Adenoma.
Liver international : official journal of the International Association for the Study of the Liver, 2020Co-Authors: Anne J. Klompenhouwer, Rob A. De Man, Jessica Zucman-rossi, Marco Dioguardi Burgio, Valérie Vilgrain, Jan N.m. IjzermansAbstract:Hepatocellular Adenoma (HCA) are benign liver tumours that may be complicated by haemorrhage or malignant transformation to Hepatocellular carcinoma. Epidemiological data are fairly outdated, but it is likely to assume that the incidence has increased over the past decades as HCA are more often incidentally found due to the more widespread use of imaging techniques and the increased incidence of obesity. Various molecular subgroups have been described. Each of these molecular subgroups are defined by specific gene mutations and pathway activations. Additionally, they are all related to specific risk factors and show a various biological behaviour. These molecular subgroups may be identified using immunohistochemistry and molecular characterization. Contrast-enhanced MRI is the recommended imaging modality to analyse patients with suspected Hepatocellular Adenoma allowing to determine the subtype in up to 80%. Surgical resection remains to be the golden standard in treating HCA, although resection is deemed unnecessary in a large number of cases, as studies have shown that the majority of HCA will regress over time without complications such as haemorrhage or malignant transformation occurring. It is preferable to treat patients with suspected HCA in high volume centres with combined expertise of liver surgeons, hepatologists, radiologists and (molecular) pathologists.
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Genotype phenotype classification of Hepatocellular Adenoma.
World journal of gastroenterology, 2007Co-Authors: Paulette Bioulac-sage, Jean Frédéric Blanc, Sandra Rebouissou, Charles Balabaud, Jessica Zucman-rossiAbstract:Studies that compare tumor genotype with phenotype have provided the basis of a new histological/molecular classification of Hepatocellular Adenomas. Based on two molecular criteria (presence of a TCF1/HNF1α or β-catenin mutation), and an additional histological criterion (presence or absence of an inflammatory infiltrate), subgroups of Hepatocellular Adenoma can be defined and distinguished from focal nodular hyperplasia. Analysis of 96 Hepatocellular Adenomas performed by a French collaborative network showed that they can be divided into four broad subgroups: the first one is defined by the presence of mutations in TCF1 gene inactivating the hepatocyte nuclear factor 1 (HNF1α); the second by the presence of β-catenin activating mutations; the category without mutations of HNF1α or β-catenin is further divided into 2 subgroups depending on the presence or absence of inflammation. Therefore, the approach to the diagnosis of problematic benign hepatocytic nodules may be entering a new era directed by new molecular information. It is hoped that immunohistological tools will improve significantly diagnosis of liver biopsy in our ability to distinguish Hepatocellular Adenoma from focal nodular hyperplasia (FNH), and to delineate clinically meaningful entities within each group to define the best clinical management. The optimal care of patients with a liver nodule will benefit from the recent knowledge coming from molecular biology and the combined expertise of hepatologists, pathologists, radiologists, and surgeons.
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Hepatocellular Adenoma displaying a HNF1alpha inactivation in a patient with familial Adenomatous polyposis coli.
Journal of Hepatology, 2006Co-Authors: Emmanuelle Jeannot, Dominique Wendum, François Paye, Najat Mourra, Claudia De Toma, Jean-françois Fléjou, Jessica Zucman-rossiAbstract:Patients with familial Adenomatous polyposis coli (FAP) may rarely develop Hepatocellular Adenoma. Here we report the case of a 37-year-old FAP woman presenting a Hepatocellular Adenoma after oestroprogestative oral contraception use. In this steatotic Adenoma, we identified an inactivating biallelic mutation of HNF1alpha. In addition to the known germline APC mutation Q1062fs, we did not find an inactivation of the second APC allele nor an activation of the beta-catenin target genes GLUL and GPR49. Our findings contrast with two Hepatocellular Adenoma cases related to FAP, for which a biallelic inactivation of the APC gene was previously described. Altogether, these results suggest that benign Hepatocellular carcinogenesis may be dependent on or independent of the Wnt/beta-catenin pathway in patients with FAP.
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Hepatocellular Adenoma displaying a HNF1α inactivation in a patient with familial Adenomatous polyposis coli
Journal of hepatology, 2006Co-Authors: Emmanuelle Jeannot, Dominique Wendum, François Paye, Najat Mourra, Claudia De Toma, Jean-françois Fléjou, Jessica Zucman-rossiAbstract:Patients with familial Adenomatous polyposis coli (FAP) may rarely develop Hepatocellular Adenoma. Here we report the case of a 37-year-old FAP woman presenting a Hepatocellular Adenoma after oestroprogestative oral contraception use. In this steatotic Adenoma, we identified an inactivating biallelic mutation of HNF1α. In addition to the known germline APC mutation Q1062fs, we did not find an inactivation of the second APC allele nor an activation of the β-catenin target genes GLUL and GPR49. Our findings contrast with two Hepatocellular Adenoma cases related to FAP, for which a biallelic inactivation of the APC gene was previously described. Altogether, these results suggest that benign Hepatocellular carcinogenesis may be dependent on or independent of the Wnt/β-catenin pathway in patients with FAP.