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Kountouri I, Kokkali E, Giotas A, Katsarelas I, Dimasis P. A Rare Case of a Spontaneously Ruptured Hepatocellular Adenoma in the Third Trimester of Pregnancy. Cureus 2024; 16:e72447. [PMID: 39600766 PMCID: PMC11588674 DOI: 10.7759/cureus.72447] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/26/2024] [Indexed: 11/29/2024] Open
Abstract
This case report details the management of a 29-year-old primigravida who presented at 35 weeks of gestation with abdominal pain and vomiting. The patient exhibited tachycardia and fetal bradycardia, with laboratory findings indicating severe anemia and elevated liver enzymes. An emergency cesarean section was performed due to hemodynamic instability, during which a ruptured hepatocellular adenoma (HCA) was discovered, necessitating an atypical left hepatectomy. Despite successful maternal recovery, the neonate succumbed to multiple organ failure. Hepatocellular adenomas (HCA), also known as hepatic adenomas, are rare benign epithelial liver tumors that predominantly occur in women during their reproductive years and have been strongly associated with the intake of oral contraceptives. This case highlights the rare occurrence of HCA rupture during pregnancy, emphasizing the importance of prompt diagnosis and intervention to prevent life-threatening complications. Additionally, it underscores the need for careful monitoring and potential preemptive intervention in pregnant women with large HCAs due to the elevated risk of rupture.
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Affiliation(s)
- Ismini Kountouri
- Department of General Surgery, General Hospital of Katerini, Katerini, GRC
| | - Eftychia Kokkali
- Department of Radiology, General Hospital of Katerini, Katerini, GRC
| | - Amyntas Giotas
- Department of Obstetrics and Gynecology, General Hospital of Katerini, Katerini, GRC
| | | | - Periklis Dimasis
- Department of General Surgery, General Hospital of Katerini, Katerini, GRC
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2
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Liver Disease During Pregnancy. Am J Gastroenterol 2022; 117:44-52. [PMID: 36194033 DOI: 10.14309/ajg.0000000000001960] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/17/2022] [Accepted: 06/23/2022] [Indexed: 11/07/2022]
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3
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Bioulac-Sage P, Gouw ASH, Balabaud C, Sempoux C. Hepatocellular Adenoma: What We Know, What We Do Not Know, and Why It Matters. Histopathology 2021; 80:878-897. [PMID: 34856012 DOI: 10.1111/his.14605] [Citation(s) in RCA: 21] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/05/2021] [Revised: 11/21/2021] [Accepted: 11/25/2021] [Indexed: 11/27/2022]
Abstract
In the last 2 decades there has been significant progress in research 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 will discuss novel methods to identify HCA subtypes, in particular the sonic hedgehog HCAs and the interpretation of glutamine synthetase patterns for the recognition of beta-catenin mutated HCAs. The major complications of HCAs, bleeding and malignant transformation, will be considered, including the dilemmas of atypical and borderline lesions. Paragraphs on HCAs in different clinical and geographical settings, e.g. pregnancy, cirrhosis and non-western countries are included. The natural history of the different HCA subtypes in relation with age, sex and risk factors is a feature 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 tumor, a multicenter and multidisciplinary approach across geographical boundaries will be the appropriate method to establish prospective programs to identify, classify and manage HCAs, focusing on several aspects, e.g. etiology, underlying liver disease, complications, regression and growth. Updating what we know, identifying and addressing features that we do not know matters to warrant optimal patient management.
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Affiliation(s)
| | - Annette S H Gouw
- Departement of Pathology and Medical Biology, University Medical Center Groningen, Groningen, the Netherlands
| | | | - Christine Sempoux
- Service of Clinical Pathology, Institute of Pathology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland
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4
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Gaspar-Figueiredo S, Kefleyesus A, Sempoux C, Uldry E, Halkic N. Focal nodular hyperplasia associated with a giant hepatocellular adenoma: A case report and review of literature. World J Hepatol 2021; 13:1450-1458. [PMID: 34786179 PMCID: PMC8568569 DOI: 10.4254/wjh.v13.i10.1450] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/25/2021] [Revised: 06/15/2021] [Accepted: 08/31/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Focal nodular hyperplasia (FNH) and hepatocellular adenoma (HCA) are well-known benign liver lesions. Surgical treatment is usually chosen for symptomatic patients, lesions more than 5 cm, and uncertainty of diagnosis.
CASE SUMMARY We described the case of a large liver composite tumor in an asymptomatic 34-year-old female under oral contraceptive for 17-years. The imaging work-out described two components in this liver tumor; measuring 6 cm × 6 cm and 14 cm × 12 cm × 6 cm. The multidisciplinary team suggested surgery for this young woman with an unclear HCA diagnosis. She underwent a laparoscopic left liver lobectomy, with an uneventful postoperative course. Final pathological examination confirmed FNH associated with a large HCA. This manuscript aimed to make a literature review of the current management in this particular situation of large simultaneous benign liver tumors.
CONCLUSION The simultaneous presence of benign composite liver tumors is rare. This case highlights the management in a multidisciplinary team setting.
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Affiliation(s)
| | - Amaniel Kefleyesus
- Department of Visceral Surgery, Lausanne University Hospital, Lausanne 1011, Switzerland
| | - Christine Sempoux
- Department of Pathology, Lausanne University Hospital, Lausanne 1011, Switzerland
| | - Emilie Uldry
- Department of Visceral Surgery, Lausanne University Hospital, Lausanne 1011, Switzerland
| | - Nermin Halkic
- Department of Visceral Surgery, Lausanne University Hospital, Lausanne 1011, Switzerland
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5
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Abstract
Chronic liver disease in pregnancy is rare. Historically, many chronic liver diseases were considered contraindications to pregnancy; however, with current monitoring and treatment strategies, pregnancy may be considered in many cases. Preconception and initial antepartum consultation should focus on disease activity, medication safety, risks of pregnancy, as well as the need for additional monitoring during pregnancy. In most cases, a multidisciplinary approach is necessary to ensure optimal maternal and fetal outcomes. Despite improving outcomes, pregnancy in women with the chronic liver disease remains high risk.
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6
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Mittal S, Gopal P, Khatri G, Singal AG. Evaluation and Management of Hepatocellular Adenomas. Clin Liver Dis (Hoboken) 2021; 17:57-60. [PMID: 33680436 PMCID: PMC7916436 DOI: 10.1002/cld.949] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/25/2019] [Revised: 12/29/2019] [Accepted: 02/05/2020] [Indexed: 02/04/2023] Open
Affiliation(s)
- Sukul Mittal
- Division of Digestive and Liver DiseasesUT Southwestern Medical CenterDallasTX
- Department of PathologyUT Southwestern Medical CenterDallasTX
- Department of RadiologyUT Southwestern Medical CenterDallasTX
| | - Purva Gopal
- Division of Digestive and Liver DiseasesUT Southwestern Medical CenterDallasTX
- Department of PathologyUT Southwestern Medical CenterDallasTX
- Department of RadiologyUT Southwestern Medical CenterDallasTX
| | - Gaurav Khatri
- Division of Digestive and Liver DiseasesUT Southwestern Medical CenterDallasTX
- Department of PathologyUT Southwestern Medical CenterDallasTX
- Department of RadiologyUT Southwestern Medical CenterDallasTX
| | - Amit G. Singal
- Division of Digestive and Liver DiseasesUT Southwestern Medical CenterDallasTX
- Department of PathologyUT Southwestern Medical CenterDallasTX
- Department of RadiologyUT Southwestern Medical CenterDallasTX
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7
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Sarkar M, Brady CW, Fleckenstein J, Forde KA, Khungar V, Molleston JP, Afshar Y, Terrault NA. Reproductive Health and Liver Disease: Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology 2021; 73:318-365. [PMID: 32946672 DOI: 10.1002/hep.31559] [Citation(s) in RCA: 67] [Impact Index Per Article: 16.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/08/2020] [Accepted: 09/08/2020] [Indexed: 12/11/2022]
Affiliation(s)
- Monika Sarkar
- University of California, San Francisco, San Francisco, CA
| | | | | | | | | | - Jean P Molleston
- Indiana University and Riley Hospital for Children, Indianapolis, IN
| | - Yalda Afshar
- University of California, Los Angeles, Los Angeles, CA
| | - Norah A Terrault
- Keck School of Medicine, University of Southern California, Los Angeles, CA
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Abstract
Focal nodular hyperplasia and hepatocellular adenoma are benign liver lesions that occur most frequently in women and may be found as incidental findings on imaging. hepatocellular adenomas may be infrequently associated with malignant progression or risk of rupture and as such, require surveillance or definitive treatments based on their size threshold. It is important clinically to differentiate these lesions, and utilizing imaging modalities such as contrast enhanced ultrasound or magnetic resonance imaging can be helpful in diagnosis. Further molecular subtyping of hepatocellular adenoma lesions may be beneficial to describe risk factors and potential future clinical complications.
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Affiliation(s)
- Lauren Myers
- Division of Gastroenterology and Hepatology, Oregon Health and Science University, 3181 Southwest Sam Jackson Park Road, MNP 4112, Portland, OR 97239, USA.
| | - Joseph Ahn
- Division of Gastroenterology and Hepatology, Oregon Health and Science University, 3181 Southwest Sam Jackson Park Road, MNP 4112, Portland, OR 97239, USA
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9
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Sanford B, Hoeppner C, Ju T, Theisen BK, BuAbbud A, Estroff JM. Multidisciplinary management of the pregnant patient in haemorrhagic shock secondary to an undiagnosed ruptured liver adenoma. BMJ Case Rep 2020; 13:13/2/e231995. [PMID: 32060109 DOI: 10.1136/bcr-2019-231995] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/22/2022] Open
Abstract
Management of a ruptured hepatocellular adenoma during pregnancy is a rare and potentially life-threatening entity. Few case reports have described management of the pregnant patient who presents in haemorrhagic shock secondary to a ruptured liver adenoma. A 30-year-old primigravid woman at 31 weeks pregnant presented with abdominal pain and fetal bradycardia. After stat caesarean delivery of the infant, she had continued hemoperitoneum and was in shock secondary to an undiagnosed ruptured liver mass. General surgery was consulted intraoperatively and performed an exploratory laparotomy, packing and temporary closure. She was subsequently taken to interventional radiology (IR) for angioembolisation of the left hepatic artery. After stabilisation, she underwent formal abdominal closure. Management of a ruptured hepatocellular adenoma in pregnancy requires urgent multidisciplinary care including obstetrics gynaecology, general surgery and IR.
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Affiliation(s)
- Brittany Sanford
- Department of Obstetrics & Gynecology and Women's Health, Albert Einstein College of Medicine/Montefiore Medical Center, Bronx, NY, USA
| | - Catherine Hoeppner
- Department of Obstetrics and Gynecology, George Washington University, Washington, DC, USA
| | - Tammy Ju
- Department of Surgery, George Washington University, Washington, DC, USA
| | - Brian K Theisen
- Department of Pathology, George Washington University, Washington, DC, USA
| | - Anna BuAbbud
- Department of Obstetrics and Gynecology, George Washington University, Washington, DC, USA
| | - Jordan M Estroff
- Department of Surgery, George Washington University, Washington, DC, USA
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10
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Growth of hepatocellular adenoma during pregnancy: A prospective study. J Hepatol 2020; 72:119-124. [PMID: 31550458 DOI: 10.1016/j.jhep.2019.09.011] [Citation(s) in RCA: 22] [Impact Index Per Article: 4.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/05/2019] [Revised: 08/28/2019] [Accepted: 09/10/2019] [Indexed: 12/29/2022]
Abstract
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 <5 cm during pregnancy. METHODS This was a multicentre prospective cohort study in pregnant women with suspected HCA <5 cm on imaging. Definitive HCA diagnosis was established by MRI with hepatobiliary contrast agents (LCE-MRI), preferably before pregnancy. If at study inclusion a definitive diagnosis was lacking, LCE-MRI was performed after giving birth. Growth of the adenoma (defined as an increase of >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 <5 cm confers minimal risk to a pregnant woman and none to her child. HCA increased in size during a quarter of pregnancies, so we recommend close monitoring with ultrasound examinations, enabling intervention if needed. In light of the large proportion of misdiagnosed HCA, LCE-MRI should be performed to prevent unnecessary anxiety in women with a benign liver lesion. 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 <5 cm during 51 pregnancies and found that a hepatocellular adenoma during pregnancy confers minimal risk to the pregnant woman and none to her child.
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Bernstein J, Spitzer Y, Reddy S, Mazur A. Hepatic adenoma during pregnancy and anesthetic management. Int J Obstet Anesth 2019; 39:137-140. [DOI: 10.1016/j.ijoa.2019.01.007] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/24/2018] [Revised: 12/19/2018] [Accepted: 01/07/2019] [Indexed: 12/14/2022]
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Glinka J, Clariá RS, Fratanoni E, Spina J, Mullen E, Ardiles V, Mazza O, Pekolj J, de Santibañes M, de Santibañes E. Malignant transformation of hepatocellular adenoma in a young female patient after ovulation induction fertility treatment: A case report. World J Gastrointest Surg 2019; 11:229-236. [PMID: 31123560 PMCID: PMC6513787 DOI: 10.4240/wjgs.v11.i4.229] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/24/2019] [Revised: 04/17/2019] [Accepted: 04/23/2019] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Hepatocellular adenoma (HCA) is a rare benign liver tumor usually affecting young women with a history of prolonged use of hormonal contraception. Although the majority is asymptomatic, a low proportion may have significant complications such as bleeding or malignancy. Despite responding to the hormonal stimulus, the desire for pregnancy in patients with small HCA is not contraindicated. However, through this work we demonstrate that intensive hormonal therapies such as those used in the treatment of infertility can trigger serious complications
CASE SUMMARY A 33-year-old female with a 10-year history of oral contraceptive use was diagnosed with a hepatic tumor as an incidental finding in an abdominal ultrasound. The patient showed no symptoms and physical examination was unremarkable. Laboratory functional tests were within normal limits and tests for serum tumor markers were negative. An abdominal magnetic resonance imaging (MRI) was performed, showing a 30 mm × 29 mm focal lesion in segment VI of the liver compatible with HCA or Focal Nodular Hyperplasia with atypical behavior. After a total of six years of follow-up, the patient underwent ovulation induction treatment for infertility. On a following MRI, a suspected malignancy was warned and hence, surgery was decided. The surgical specimen revealed malignant transformation of HCA towards trabecular hepatocarcinoma with dedifferentiated areas. There was non-evidence of tumor recurrence after three years of clinical and imaging follow-up.
CONCLUSION HCAs can be malignant regardless its size and low-risk appearance on MRI when an ovultation induction therapy is indicated.
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Affiliation(s)
- Juan Glinka
- Department of General Surgery, Hepato-bilio-pancreatic Unit, Hospital Italiano de Buenos Aires, Buenos Aires C1181ACH, Argentina
| | - Rodrigo Sanchez Clariá
- Department of General Surgery, Hepato-bilio-pancreatic Unit, Hospital Italiano de Buenos Aires, Buenos Aires C1181ACH, Argentina
| | - Eugenia Fratanoni
- Department of General Surgery, Hepato-bilio-pancreatic Unit, Hospital Italiano de Buenos Aires, Buenos Aires C1181ACH, Argentina
| | - Juan Spina
- Department of Radiology, Hospital Italiano de Buenos Aires, Buenos Aires C1181ACH, Argentina
| | - Eduardo Mullen
- Department of Surgical Pathology, Hospital Italiano de Buenos Aires, Buenos Aires C1181ACH, Argentina
| | - Victoria Ardiles
- Department of General Surgery, Hepato-bilio-pancreatic Unit, Hospital Italiano de Buenos Aires, Buenos Aires C1181ACH, Argentina
| | - Oscar Mazza
- Department of General Surgery, Hepato-bilio-pancreatic Unit, Hospital Italiano de Buenos Aires, Buenos Aires C1181ACH, Argentina
| | - Juan Pekolj
- Department of General Surgery, Hepato-bilio-pancreatic Unit, Hospital Italiano de Buenos Aires, Buenos Aires C1181ACH, Argentina
| | - Martín de Santibañes
- Department of General Surgery, Hepato-bilio-pancreatic Unit, Hospital Italiano de Buenos Aires, Buenos Aires C1181ACH, Argentina
| | - Eduardo de Santibañes
- Department of General Surgery, Hepato-bilio-pancreatic Unit, Hospital Italiano de Buenos Aires, Buenos Aires C1181ACH, Argentina
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13
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Kevat D, Chen M, Wyld D, Fagermo N, Lust K. A case of pulmonary carcinoid in pregnancy and review of carcinoid tumours in pregnancy. Obstet Med 2017; 10:142-149. [PMID: 29051783 DOI: 10.1177/1753495x16687700] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/06/2016] [Accepted: 11/28/2016] [Indexed: 12/24/2022] Open
Abstract
Carcinoid tumours are rare slow growing tumours which arise from primitive neuroendocrine cells. The effect of the pregnant state on carcinoid tumours and vice versa remains unclear, as does the optimal management of carcinoid tumours during the pregnancy including labour. We report the rare case of a 36 year old primigravida woman with large bilateral pulmonary carcinoid tumours. The patient's disease was minimally symptomatic with no clinical suspicion of carcinoid syndrome. Under close observation, the pregnancy progressed well and the patient proceeded to a spontaneous vaginal delivery of a healthy child. We conduct the the first literature review in 30 years of all reported cases in this area and make suggestions as to assessment and monitoring of cases of carcinoid during pregnancy.
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Affiliation(s)
- D Kevat
- Division of Medicine, Royal Brisbane and Women's Hospital, Brisbane, Australia.,School of Public Health, Monash University, Melbourne, Australia
| | - M Chen
- Division of Medicine, Royal Brisbane and Women's Hospital, Brisbane, Australia
| | - D Wyld
- Division of Medicine, Royal Brisbane and Women's Hospital, Brisbane, Australia.,Faculty of Medicine, University of Queensland, Brisbane, Australia
| | - N Fagermo
- Division of Medicine, Royal Brisbane and Women's Hospital, Brisbane, Australia
| | - K Lust
- Division of Medicine, Royal Brisbane and Women's Hospital, Brisbane, Australia
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14
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Thomeer MG, Broker M, Verheij J, Doukas M, Terkivatan T, Bijdevaate D, De Man RA, Moelker A, IJzermans JN. Hepatocellular adenoma: when and how to treat? Update of current evidence. Therap Adv Gastroenterol 2016; 9:898-912. [PMID: 27803743 PMCID: PMC5076773 DOI: 10.1177/1756283x16663882] [Citation(s) in RCA: 33] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023] Open
Abstract
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), with resection preferred for larger 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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Affiliation(s)
| | - Mirelle Broker
- Department of Surgery, Erasmus MC University Medical Center, Rotterdam, The Netherlands
| | - Joanne Verheij
- Department of Pathology, Amsterdam University Medical Center, Amsterdam, The Netherlands
| | - Michael Doukas
- Department of pathology, Erasmus MC University Medical Center, Rotterdam, The Netherlands
| | - Turkan Terkivatan
- Department of Surgery, Erasmus MC University Medical Center, Rotterdam, The Netherlands
| | - Diederick Bijdevaate
- Department of Radiology, Erasmus MC University Medical Center, Rotterdam, The Netherlands
| | - Robert A. De Man
- Department of Gastroenterology and Hepatology, Erasmus MC University Medical Center, Rotterdam, The Netherlands
| | - Adriaan Moelker
- Department of Radiology, Erasmus MC University Medical Center, Rotterdam, The Netherlands
| | - Jan N. IJzermans
- Department of Surgery, Erasmus MC University Medical Center, Rotterdam, The Netherlands
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15
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Strauss E, Ferreira ADSP, França AVC, Lyra AC, Barros FMDR, Silva I, Garcia JHP, Parise ER. Diagnosis and treatment of benign liver nodules: Brazilian Society of Hepatology (SBH) recommendations. ARQUIVOS DE GASTROENTEROLOGIA 2015; 52 Suppl 1:47-54. [PMID: 26959805 DOI: 10.1590/s0004-28032015000500003] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/08/2023]
Abstract
Space-occupying lesions of the liver may be cystic or solid. Ultrasonography is an extremely useful method for initial screening, and suffices for diagnosis of simple hepatic cysts. Complex cysts and solid masses require computed tomography or magnetic resonance imaging for confirmation. Wide surgical excision is indicated in cystadenoma or cystadenocarcinoma. Clinical and epidemiological data are important, as nodules in noncirrhotic livers are more likely to be benign. Hemangiomas, the most common benign tumors, require no follow-up after diagnostic confirmation if they are small and asymptomatic. Patients with giant, symptomatic hemangiomas or compression of adjacent structures should be referred to hepatobiliary centers for potential surgery. The genetic heterogeneity of hepatocellular adenomas and their epidemiology and prognosis prompted classification of these tumors into four subtypes based on histology and immunohistochemistry. The major complications of hepatocellular adenomas are rupture with bleeding and malignant transformation. Rupture occurs in approximately 30% of cases. The main risk factors are tumors size >5 cm and inflammatory subtype. Hepatocellular adenomas may enlarge during pregnancy due to marked hormonal stimulation. As oral contraceptive pills and anabolic steroids have associated with hepatocellular adenomas growth, particularly of the hepatocyte nuclear factor-1alfa subtype, these drugs should be discontinued. Focal nodular hyperplasia is the second most common benign tumor of the liver. It is most frequent in women aged 20 to 60, and 70% to 90% of cases are asymptomatic. In the absence of a central scar and/or other hallmarks of Focal nodular hyperplasia, with uncertainty between this diagnosis and hepatocellular adenoma, liver-specific contrast agents are indicated.
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Affiliation(s)
- Edna Strauss
- Faculdade de Medicina, Universidade de São Paulo, SP, Brazil
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16
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Vijay A, Elaffandi A, Khalaf H. Hepatocellular adenoma: An update. World J Hepatol 2015; 7:2603-2609. [PMID: 26557953 PMCID: PMC4635146 DOI: 10.4254/wjh.v7.i25.2603] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/11/2015] [Revised: 08/30/2015] [Accepted: 10/27/2015] [Indexed: 02/06/2023] Open
Abstract
Hepatocellular adenomas (HCA) are rare benign liver tumors. Recent technological advancements have helped in the early identification of such lesions. However, precise diagnosis of hepatocellular incidentalomas remains challenging. Studies at the molecular level have provided new insights into the genetics and pathophysiology of these lesions. These in turn have raised questions over their existing management modalities. However, the rarity of the tumor still restricts the quality of evidence available for current recommendations and guidelines. This article provides a comprehensive review on the etiology, molecular biology, patho-physiology, clinical manifestations, and complications associated with HCA. It also elaborates on the genetic advancements, existing diagnostic tools and current guidelines for management for such lesions.
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17
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Abstract
Hepatic adenomatosis (HeAs) is a rare clinical entity defined by the presence of 10 or more hepatic adenomas (HA) within the background of an otherwise normal liver parenchyma, in the absence of glycogen storage disease or anabolic steroid use. HA is a benign tumor associated with oral contraceptive use. Recent advances in pathogenesis and classification of HA have questioned the distinction between these two diseases. HA are currently classified into four different subtypes with genotypic and phenotypic correlation: HNF-1a inactivated HA, B-catenin activated HA, inflammatory HA, and undetermined subtype. The clinical presentation of HA depends on the lesion size and the subtype. MRI using hepatospecific contrast agents is helpful in diagnosing the most common subtypes. When diagnosis is uncertain, biopsy with immunohistochemistry is used to diagnose and classify the lesions. Management is governed by the molecular subtype and tumor size. Pregnancy is not routinely discouraged but management is individualized.
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Affiliation(s)
- Manish Thapar
- Department of Medicine, Drexel University College of Medicine, Philadelphia, PA, 19142, USA,
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18
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Thomeer MG, E Bröker ME, de Lussanet Q, Biermann K, Dwarkasing RS, de Man R, Ijzermans JN, de Vries M. Genotype-phenotype correlations in hepatocellular adenoma: an update of MRI findings. Diagn Interv Radiol 2015; 20:193-9. [PMID: 24509184 DOI: 10.5152/dir.2013.13315] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]
Abstract
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.
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Affiliation(s)
- Maarten G Thomeer
- From the Departments of Radiology Medical Center Zuiderzee, Lelystad, the Netherlands.
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Blanc JF, Frulio N, Chiche L, Sempoux C, Annet L, Hubert C, Gouw ASH, de Jong KP, Bioulac-Sage P, Balabaud C. Hepatocellular adenoma management: call for shared guidelines and multidisciplinary approach. Clin Res Hepatol Gastroenterol 2015; 39:180-7. [PMID: 25434466 DOI: 10.1016/j.clinre.2014.10.003] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/13/2014] [Revised: 09/17/2014] [Accepted: 10/06/2014] [Indexed: 02/07/2023]
Abstract
Hepatocellular adenomas are rare benign nodules developed mainly in women taking oral contraceptives. They are solitary or multiple. Their size is highly variable. There is no consensus in the literature for their management except that once their size exceeds 5 cm nodules are taken out to prevent 2 major complications: bleeding and malignant transformation. There are exceptions particularly in men where it is recommended to remove smaller nodules. Since the beginning of this century, major scientific contributions have unveiled the heterogeneity of the disease. HCA are composed of four major subtypes. HNF1A (coding for hepatocyte nuclear factor 1a) inactivating mutations (H-HCA); inflammatory adenomas (IHCA); the β-catenin-mutated HCAs (β-HCA) and unclassified HCA (UHCA) occurring in 30-40%, 40-50%, 10-15% and 10% of all HCA, respectively. Half of β-HCAs are also inflammatory (β-IHCA). Importantly, β-catenin mutations are associated with a high risk of malignant transformation. HCA subtypes can be identified on liver tissue, including biopsies using specific immunomarkers with a good correspondence with molecular data. Recent data has shown that TERT promoter mutation was a late event in the malignant transformation of β-HCA, β-IHCA. Furthermore, in addition to β-catenin exon 3 mutations, other mutations do exist (exon 7 and 8) with a lower risk of malignant transformation. With these new scientific informations, we have the tools to better know the natural history of the different subtypes, in terms of growth, disappearance, bleeding, malignant transformation and to investigate HCA in diseased livers (vascular diseases, alcoholic cirrhosis). A better knowledge of HCA should lead to a more rational management of HCA. This can be done only if the different subspecialties, including hepatologists, liver pathologists, radiologists and surgeons work altogether in close relationship with molecular biologists. It is a long way to go.
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Affiliation(s)
- Jean Frédéric Blanc
- Service d'hépato-gastroentérologie, hôpital Saint-André, CHU de Bordeaux, Inserm UMR 1053, université de Bordeaux, Bordeaux, France.
| | - Nora Frulio
- Service de radiologie, hôpital Saint-André, CHU de Bordeaux, Bordeaux, France.
| | - Laurence Chiche
- Service hépatobiliaire et pancréatique, maison du Haut-Lévèque, CHU de Bordeaux, 33604 Pessac cedex, France.
| | - Christine Sempoux
- Service d'anatomie pathologique, cliniques universitaires Saint-Luc, université catholique de Louvain, 1200 Brussels, Belgium.
| | - Laurence Annet
- Service de radiologie, cliniques universitaires Saint-Luc, université catholique de Louvain, 1200 Brussels, Belgium.
| | - Catherine Hubert
- Service de chirurgie digestive, cliniques universitaires Saint-Luc, université catholique de Louvain, 1200 Brussels, Belgium.
| | - Annette S H Gouw
- Department of Pathology and Medical Biology, University Medical Center Groningen, 9700 Groningen, The Netherlands.
| | - Koert P de Jong
- Department of Hepato-Pancreato-Biliary Surgery & Liver Transplantation, University Medical Center Groningen, 9700 Groningen, The Netherlands.
| | - Paulette Bioulac-Sage
- Inserm UMR 1053, université de Bordeaux, service de pathologie, hôpital Pellegrin, CHU de Bordeaux, 33075 Bordeaux cedex, France.
| | - Charles Balabaud
- Inserm UMR 1053, université de Bordeaux, 146, rue Léo-Saignat, 33076 Bordeaux cedex, France.
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Beegle RD, Brown LM, Weinstein DA. Regression of hepatocellular adenomas with strict dietary therapy in patients with glycogen storage disease type I. JIMD Rep 2014; 18:23-32. [PMID: 25308557 DOI: 10.1007/8904_2014_344] [Citation(s) in RCA: 31] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/24/2014] [Revised: 07/15/2014] [Accepted: 07/23/2014] [Indexed: 12/21/2022] Open
Abstract
Hepatocellular adenomas (HCAs) are a common complication in patients with glycogen storage disease type I (GSD I). In this series, we report regression of HCAs in a cohort of patients who achieved metabolic control with strict dietary therapy. A retrospective review of the clinical records for all patients with GSD I was performed at our institution. All available imaging studies were reviewed in patients with reported regression of HCAs in the medical record. The charts of 163 patients with GSD Ia and 42 patients with GSD Ib were reviewed, and HCAs were documented in 47 subjects (43 Ia/4 Ib). After review of all available imaging studies, eight patients met criteria of being followed with both magnetic resonance imaging and ultrasound and were found to show evidence of regression of HCAs. In these individuals, regression of the HCAs occurred once metabolic control was obtained, as determined by decreasing levels of serum triglyceride levels. The average triglyceride level in all patients prior to regression of HCAs was 753 mg/dL (SD ± 293). The average serum triglyceride level in all patients at the time of regression of HCAs was 340 mg/dL (SD ± 164). These findings suggest that strict dietary therapy may cause regression of HCAs. If HCAs are documented in a patient with suboptimal metabolic control, intensive medical therapy may be an alternative to surgical intervention in some individuals.
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Affiliation(s)
- Richard D Beegle
- Department of Radiology, University of Florida College of Medicine, Gainesville, FL, USA
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ACG clinical guideline: the diagnosis and management of focal liver lesions. Am J Gastroenterol 2014; 109:1328-47; quiz 1348. [PMID: 25135008 DOI: 10.1038/ajg.2014.213] [Citation(s) in RCA: 278] [Impact Index Per Article: 25.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2013] [Accepted: 05/22/2014] [Indexed: 12/11/2022]
Abstract
Focal liver lesions (FLL) have been a common reason for consultation faced by gastroenterologists and hepatologists. The increasing and widespread use of imaging studies has led to an increase in detection of incidental FLL. It is important to consider not only malignant liver lesions, but also benign solid and cystic liver lesions such as hemangioma, focal nodular hyperplasia, hepatocellular adenoma, and hepatic cysts, in the differential diagnosis. In this ACG practice guideline, the authors provide an evidence-based approach to the diagnosis and management of FLL.
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Sempoux C, Chang C, Gouw A, Chiche L, Zucman-Rossi J, Balabaud C, Bioulac-Sage P. Benign hepatocellular nodules: what have we learned using the patho-molecular classification. Clin Res Hepatol Gastroenterol 2013; 37:322-7. [PMID: 23876350 DOI: 10.1016/j.clinre.2013.04.008] [Citation(s) in RCA: 25] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/19/2013] [Revised: 04/04/2013] [Accepted: 04/05/2013] [Indexed: 02/04/2023]
Abstract
Focal nodular hyperplasia (FNH) and hepatocellular adenoma (HCA) are benign hepatocellular tumors that develop most frequently in females and in non-cirrhotic livers. HCA are prone to bleed and to transform into hepatocellular carcinoma (HCC). Four major subgroups of HCA have been thus far identified: HNF1α mutated HCA, inflammatory HCA (IHCA), β-catenin mutated HCA (b-HCA and b-IHCA), based on mutations in specific oncogenes and tumor suppressors. B-HCA and b-IHCA are strongly associated with HCC transformation. Benign hepatocellular tumors can be classified using immunohistochemistry (LFABP, CRP, GS, b-catenin). Analysis of HCA phenotypes has led to the identification of patients at risk of HCC transformation and therefore improved the indications provided by invasive and non-invasive diagnostic techniques, such as biopsies and MRI. These recent advances have broadened the clinical scope of HCA in various conditions, such as their presence in males, in obese patients, in patients suffering from liver vascular disorders, genetic diseases. However, specific immunohistochemistry has shown limitations particularly for the identification of b-HCA, thereby, outlining the importance of molecular studies to improve the diagnosis/prognosis of HCA. If evaluation of prognosis and treatment has benefited from these advances, much more needs to be done to obtain guidelines for good clinical practice.
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Affiliation(s)
- Christine Sempoux
- Service d'Anatomie Pathologique, Cliniques universitaires Saint-Luc, Université catholique de Louvain, 1200 Brussels, Belgium.
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