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1
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Salles-Silva E, de Castro PL, Ambrozino LC, de Araújo ALE, Lahan-Martins D, Almeida MFA, Lucchesi FR, Pacheco EO, Torres US, D'Ippolito G, Parente DB. Rare Malignant Liver Tumors: Current Insights and Imaging Challenges. Semin Ultrasound CT MR 2025:S0887-2171(25)00015-0. [PMID: 40220972 DOI: 10.1053/j.sult.2025.04.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/14/2025]
Abstract
Rare malignant liver tumors (RMLTs) comprise a diverse group of neoplasms with distinct imaging features and significant diagnostic challenges due to their low prevalence and overlap with more common hepatic lesions. This review highlights the main radiologic characteristics of selected RMLTs-including fibrolamellar hepatocellular carcinoma, hepatic lymphoma, hepatocellular carcinoma in non-cirrhotic liver, mucinous cystic neoplasm, intraductal papillary neoplasm of the bile duct, epithelioid hemangioendothelioma, angiosarcoma, malignant hepatic adenoma, neuroendocrine tumor, hepatocholangiocarcinoma, hepatoblastoma, undifferentiated embryonal sarcoma, and infantile hepatic hemangioendothelioma-focusing on their presentation in CT and MRI. Recognizing specific imaging findings, such as arterial hyperenhancement, biliary communication, target and lollipop signs, and tumor morphology, can help narrow differential diagnoses and guide appropriate clinical management. Despite advancements in imaging, histopathologic confirmation is often required due to nonspecific features. Improved radiologic awareness of these rare entities is essential to facilitate early diagnosis and individualized treatment planning.
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Affiliation(s)
- Eleonora Salles-Silva
- Federal University of Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil; Grupo Fleury, Brazil
| | | | | | - Antonio Luis-Eiras de Araújo
- Federal University of Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil; D'Or Institute for Research and Education, Rio de Janeiro, Brazil
| | | | | | | | | | - Ulysses S Torres
- Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil; Grupo Fleury, Brazil
| | - Giuseppe D'Ippolito
- Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil; Grupo Fleury, Brazil
| | - Daniella Braz Parente
- Federal University of Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil; D'Or Institute for Research and Education, Rio de Janeiro, Brazil; Grupo Fleury, Brazil.
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2
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Nicotra S, Melan L, Busetto A, Bonis A, Lione L, Verzeletti V, Pezzuto F, Dell’Amore A, Calabrese F, Rea F. Aggressive Surgical Management of Bilateral Metachronous Lung Metastases in Fibrolamellar Hepatocellular Carcinoma, a Case Report. LIVERS 2024; 4:398-405. [DOI: 10.3390/livers4030029] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 03/18/2025] Open
Abstract
Fibrolamellar hepatocellular carcinoma (FL-HCC) is a malignant primary hepatic cancer that affects mainly adolescents and young adults without underlying liver disease. Its biology remains unknown, but it is pathologically distinct from traditional HCC. Therapeutic strategies are not well defined and, as chemotherapies seem to have limited efficacy, surgical resection remains the only effective treatment. Here we report on a case of a metastatic FL-HCC in an 18-year-old man successfully treated with aggressive intra-thoracic bilateral lung metastasectomy following primary tumour resection and adjuvant chemotherapy. Survival time after initial hepatectomy is 39 months, with no recurrence of disease to date. Aggressive surgical resection and redo surgery should be considered until more effective multimodality therapies are identified. Multidisciplinary team discussion and involvement of medical and surgical specialties are essential in managing these rare entities.
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Affiliation(s)
- Samuele Nicotra
- Thoracic Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova, 35121 Padova, Italy
| | - Luca Melan
- Thoracic Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova, 35121 Padova, Italy
| | - Alberto Busetto
- Thoracic Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova, 35121 Padova, Italy
| | - Alessandro Bonis
- Thoracic Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova, 35121 Padova, Italy
| | - Luigi Lione
- Thoracic Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova, 35121 Padova, Italy
| | - Vincenzo Verzeletti
- Thoracic Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova, 35121 Padova, Italy
| | - Federica Pezzuto
- Pathology Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, 35121 Padova, Italy
| | - Andrea Dell’Amore
- Thoracic Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova, 35121 Padova, Italy
| | - Fiorella Calabrese
- Pathology Unit, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, 35121 Padova, Italy
| | - Federico Rea
- Thoracic Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences and Public Health, University of Padova, 35121 Padova, Italy
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3
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Matteini F, Cannella R, Garzelli L, Dioguardi Burgio M, Sartoris R, Brancatelli G, Vilgrain V, Ronot M, Vernuccio F. Benign and malignant focal liver lesions displaying rim arterial phase hyperenhancement on CT and MRI. Insights Imaging 2024; 15:178. [PMID: 39020233 PMCID: PMC11254889 DOI: 10.1186/s13244-024-01756-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2024] [Accepted: 06/22/2024] [Indexed: 07/19/2024] Open
Abstract
Rim arterial phase hyperenhancement is an imaging feature commonly encountered on contrast-enhanced CT and MRI in focal liver lesions. Rim arterial phase hyperenhancement is a subtype of arterial phase hyperenhancement mainly present at the periphery of lesions on the arterial phase. It is caused by a relative arterialization of the periphery compared with the center of the lesion and needs to be differentiated from other patterns of peripheral enhancement, including the peripheral discontinuous nodular enhancement and the corona enhancement. Rim arterial phase hyperenhancement may be a typical or an atypical imaging presentation of many benign and malignant focal liver lesions, challenging the radiologists during imaging interpretation. Benign focal liver lesions that may show rim arterial phase hyperenhancement may have a vascular, infectious, or inflammatory origin. Malignant focal liver lesions displaying rim arterial phase hyperenhancement may have a vascular, hepatocellular, biliary, lymphoid, or secondary origin. The differences in imaging characteristics on contrast-enhanced CT may be subtle, and a multiparametric approach on MRI may be helpful to narrow the list of differentials. This article aims to review the broad spectrum of focal liver lesions that may show rim arterial phase hyperenhancement, using an approach based on the benign and malignant nature of lesions and their histologic origin. CRITICAL RELEVANCE STATEMENT: Rim arterial phase hyperenhancement may be an imaging feature encountered in benign and malignant focal liver lesions and the diagnostic algorithm approach provided in this educational review may guide toward the final diagnosis. KEY POINTS: Several focal liver lesions may demonstrate rim arterial phase hyperenhancement. Rim arterial phase hyperenhancement may occur in vascular, inflammatory, and neoplastic lesions. Rim arterial phase hyperenhancement may challenge radiologists during image interpretation.
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Affiliation(s)
- Francesco Matteini
- Department of Biomedicine, Neuroscience and Advanced Diagnostics (Bi.N.D.), University Hospital of Palermo, Palermo, Italy
- Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE), University of Palermo, Palermo, Italy
| | - Roberto Cannella
- Department of Biomedicine, Neuroscience and Advanced Diagnostics (Bi.N.D.), University Hospital of Palermo, Palermo, Italy
| | - Lorenzo Garzelli
- Department of Radiology, Hôpital Beaujon, AP-HP.Nord, Paris, France
- Université Paris Cité, INSERM U1149, "Centre de Recherche sur l'Inflammation"; CRI, Paris, France
| | - Marco Dioguardi Burgio
- Department of Radiology, Hôpital Beaujon, AP-HP.Nord, Paris, France
- Université Paris Cité, INSERM U1149, "Centre de Recherche sur l'Inflammation"; CRI, Paris, France
| | - Riccardo Sartoris
- Department of Radiology, Hôpital Beaujon, AP-HP.Nord, Paris, France
- Université Paris Cité, INSERM U1149, "Centre de Recherche sur l'Inflammation"; CRI, Paris, France
| | - Giuseppe Brancatelli
- Department of Biomedicine, Neuroscience and Advanced Diagnostics (Bi.N.D.), University Hospital of Palermo, Palermo, Italy
| | - Valérie Vilgrain
- Department of Radiology, Hôpital Beaujon, AP-HP.Nord, Paris, France
- Université Paris Cité, INSERM U1149, "Centre de Recherche sur l'Inflammation"; CRI, Paris, France
| | - Maxime Ronot
- Department of Radiology, Hôpital Beaujon, AP-HP.Nord, Paris, France
- Université Paris Cité, INSERM U1149, "Centre de Recherche sur l'Inflammation"; CRI, Paris, France
| | - Federica Vernuccio
- Department of Biomedicine, Neuroscience and Advanced Diagnostics (Bi.N.D.), University Hospital of Palermo, Palermo, Italy.
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4
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Apumayta ED, Kahlam A, Ruiz EF. Fibrolamellar Carcinoma: A Rare Liver Neoplasm. Cureus 2024; 16:e59006. [PMID: 38800256 PMCID: PMC11127726 DOI: 10.7759/cureus.59006] [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: 04/25/2024] [Indexed: 05/29/2024] Open
Abstract
Fibrolamellar carcinoma is a rare liver tumor, with most cases arising in people younger than 40 years of age. We present a case series of five patients with histological confirmation of fibrolamellar carcinoma who had liver resection as the primary treatment. The median age of diagnosis was 24 years with nonspecific clinical manifestations in otherwise healthy patients. Alpha-fetoprotein levels were widely variable. Patients had classical imaging, macroscopic, and microscopic findings. Most of our patients underwent a hemihepatectomy and 60% recurred after the first year.
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Affiliation(s)
- Elily D Apumayta
- Department of Abdominal Surgery, Instituto Nacional de Enfermedades Neoplasicas, Lima, PER
| | - Aaron Kahlam
- Department of Internal Medicine, Rutgers New Jersey Medical School, Newark, NJ, USA
| | - Eloy F Ruiz
- Department of Internal Medicine, Rutgers New Jersey Medical School, Newark, NJ, USA
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Villavicencio Kim J, Wu GY. Focal Nodular Hyperplasia: A Comprehensive Review with a Particular Focus on Pathogenesis and Complications. J Clin Transl Hepatol 2024; 12:182-190. [PMID: 38343607 PMCID: PMC10851072 DOI: 10.14218/jcth.2023.00265] [Citation(s) in RCA: 2] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/09/2023] [Revised: 09/27/2023] [Accepted: 11/09/2023] [Indexed: 01/03/2025] Open
Abstract
Focal nodular hyperplasia is a benign tumor of the liver that is often found incidentally with imaging. The purpose of this review is to discuss the pathophysiology, rare complications that can occur due to these lesions, and management options. A literature review was performed on clinical trials and case reports involving focal nodular hyperplasia complications and management of these, as well as the proposed pathogenesis underlying these tumors. Although exposure to oral contraceptive pills and endogenous hormones have been thought to play a role in the development of these lesions, this has not been proven. Most recently, they are thought to arise as a consequence of a vascular anomaly causing alterations in the expression of angiopoietin genes. Complications are rare, but previous cases have reported associated pain, rupture and compression of nearby structures (hepatic vein, stomach, biliary system). Resection of focal nodular hyperplasia is not usually recommended. However, if there is associated pain with no other identifiable cause or presence of a large or growing lesion with risk of causing a complication, then surgical resection, radiofrequency ablation or arterial embolization should be considered.
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Affiliation(s)
- Jaimy Villavicencio Kim
- Division of Gastroenterology-Hepatology, University of Connecticut Health Center, Farmington, CT, USA
| | - George Y. Wu
- Division of Gastroenterology-Hepatology, University of Connecticut Health Center, Farmington, CT, USA
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6
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Anand AC. Liver. GASTROENTEROLOGY AND HEPATOLOGY 2024:243-414. [DOI: 10.1007/978-981-99-9287-4_5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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7
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Bhagat P, Vij M, Raju LP, Gowrishankar G, Menon J, Shanmugam N, Kaliamoorthy I, Rammohan A, Rela M. Update on the Pathology of Pediatric Liver Tumors: A Pictorial Review. Diagnostics (Basel) 2023; 13:3524. [PMID: 38066766 PMCID: PMC10706829 DOI: 10.3390/diagnostics13233524] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2023] [Revised: 11/18/2023] [Accepted: 11/20/2023] [Indexed: 01/04/2025] Open
Abstract
Liver tumors in children are uncommon and show remarkable morphologic heterogeneity. Pediatric tumors may arise from either the epithelial or mesenchymal component of the liver and rarely may also show both lines of differentiation. Both benign and malignant liver tumors have been reported in children. The most common pediatric liver tumors by age are benign hepatic infantile hemangiomas in neonates and infants, malignant hepatoblastoma in infants and toddlers, and malignant hepatocellular carcinoma in teenagers. Here, we provide an up-to-date review of pediatric liver tumors. We discuss the clinical presentation, imaging findings, pathology, and relevant molecular features that can help in the correct identification of these tumors, which is important in managing these children.
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Affiliation(s)
- Priyanka Bhagat
- Department of Pathology, Choithram Hospital and Research Center, Manik Bagh Road, Indore 452014, Madhya Pradesh, India;
| | - Mukul Vij
- Department of Pathology, Dr. Rela Institute & Medical Centre, No. 7 CLC Works Road Chromepet, Chennai 600044, Tamil Nadu, India; (L.P.R.); (G.G.)
| | - Lexmi Priya Raju
- Department of Pathology, Dr. Rela Institute & Medical Centre, No. 7 CLC Works Road Chromepet, Chennai 600044, Tamil Nadu, India; (L.P.R.); (G.G.)
| | - Gowripriya Gowrishankar
- Department of Pathology, Dr. Rela Institute & Medical Centre, No. 7 CLC Works Road Chromepet, Chennai 600044, Tamil Nadu, India; (L.P.R.); (G.G.)
| | - Jagadeesh Menon
- The Institute of Liver Disease & Transplantation, Dr. Rela Institute & Medical Centre, No. 7 CLC Works Road Chromepet, Chennai 600044, Tamil Nadu, India; (J.M.); (N.S.); (I.K.); (A.R.); (M.R.)
| | - Naresh Shanmugam
- The Institute of Liver Disease & Transplantation, Dr. Rela Institute & Medical Centre, No. 7 CLC Works Road Chromepet, Chennai 600044, Tamil Nadu, India; (J.M.); (N.S.); (I.K.); (A.R.); (M.R.)
| | - Ilankumaran Kaliamoorthy
- The Institute of Liver Disease & Transplantation, Dr. Rela Institute & Medical Centre, No. 7 CLC Works Road Chromepet, Chennai 600044, Tamil Nadu, India; (J.M.); (N.S.); (I.K.); (A.R.); (M.R.)
| | - Ashwin Rammohan
- The Institute of Liver Disease & Transplantation, Dr. Rela Institute & Medical Centre, No. 7 CLC Works Road Chromepet, Chennai 600044, Tamil Nadu, India; (J.M.); (N.S.); (I.K.); (A.R.); (M.R.)
| | - Mohamed Rela
- The Institute of Liver Disease & Transplantation, Dr. Rela Institute & Medical Centre, No. 7 CLC Works Road Chromepet, Chennai 600044, Tamil Nadu, India; (J.M.); (N.S.); (I.K.); (A.R.); (M.R.)
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Watanabe A, Harimoto N, Saito H, Kawabata-Iwakawa R, Seki T, Muranushi R, Hoshino K, Hagiwara K, Ishii N, Tsukagoshi M, Igarashi T, Araki K, Ikota H, Ishige T, Mimori K, Shirabe K. Fibrolamellar hepatocellular carcinoma: a case report and gene analysis. Surg Case Rep 2023; 9:168. [PMID: 37728655 PMCID: PMC10511385 DOI: 10.1186/s40792-023-01751-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/02/2023] [Accepted: 09/07/2023] [Indexed: 09/21/2023] Open
Abstract
BACKGROUND Fibrolamellar hepatocellular carcinoma (HCC) (FL-HCC) is rare in Japan. FL-HCC develops in young patients with no history of cirrhosis and tends to manifest lymphatic metastasis with clinical features similar to those of HCC. We present a case of FL-HCC in a young male patient. CASE PRESENTATION A 14-year-old male patient underwent abdominal computed tomography (CT) to diagnose appendicitis, wherein a hepatic tumor was detected. Dynamic enhanced CT revealed a 35-mm solid tumor, which contrasted at the early phase of dynamic enhanced study of the right hepatic segments, with occlusion of the right portal vein. We performed right hepatectomy for these lesions. The patient experienced a single lymphatic recurrence on the hepatoduodenal ligament 12 months after the initial surgery. We performed lymphadenectomy for the recurrent tumor. We performed RNA sequencing (RNA-seq) and targeted DNA sequencing of the resected specimens (primary tumor, lymphatic metastasis, and normal liver). RNA-seq detected DNAJB1-PRKACA in both primary and metastatic lesions as previously reported. Furthermore, The Cancer Genome Atlas (TCGA) database was used to compare other gene expressions in this case with those of previously reported cases of FL-HCC and HCC in young patients. Principal component analysis of differentially expressed genes in the top 10% revealed that the gene expression in our case was similar to that of previous FL-HCC cases but was a different cluster from that in HCC cases in young patients. Mutational analysis did not detect any somatic mutations associated with carcinogenesis, including previously reported mutations (Kastenhuber et al. in Proc Natl Acad Sci USA 114: 13076-84, 2017). CONCLUSION We encountered a case of FL-HCC, a rare hepatic tumor in an adolescent patient, and evaluated the genetic background. Our findings could contribute to the elucidation of the mechanisms underlying carcinogenesis and progression in patients with FL-HCC and thereby contribute to the development of new therapeutic strategies in the future that may improve patient prognosis.
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Affiliation(s)
- Akira Watanabe
- Division of Hepatobiliary and Pancreatic Surgery, Department of General Surgical Science, Graduate School of Medicine, Gunma University, 3-39-22 Showa-Machi, Maebashi, Gunma, 371-8511, Japan
| | - Norifumi Harimoto
- Division of Hepatobiliary and Pancreatic Surgery, Department of General Surgical Science, Graduate School of Medicine, Gunma University, 3-39-22 Showa-Machi, Maebashi, Gunma, 371-8511, Japan.
| | - Hideyuki Saito
- Department of Surgery, Kyushu University Beppu Hospital, Beppu, Japan
| | - Reika Kawabata-Iwakawa
- Division of Integrated Oncology Research, Gunma University Initiative for Advanced Research, Gunma University, Maebashi, Japan
| | - Takaomi Seki
- Division of Hepatobiliary and Pancreatic Surgery, Department of General Surgical Science, Graduate School of Medicine, Gunma University, 3-39-22 Showa-Machi, Maebashi, Gunma, 371-8511, Japan
| | - Ryo Muranushi
- Division of Hepatobiliary and Pancreatic Surgery, Department of General Surgical Science, Graduate School of Medicine, Gunma University, 3-39-22 Showa-Machi, Maebashi, Gunma, 371-8511, Japan
| | - Kouki Hoshino
- Division of Hepatobiliary and Pancreatic Surgery, Department of General Surgical Science, Graduate School of Medicine, Gunma University, 3-39-22 Showa-Machi, Maebashi, Gunma, 371-8511, Japan
| | - Kei Hagiwara
- Division of Hepatobiliary and Pancreatic Surgery, Department of General Surgical Science, Graduate School of Medicine, Gunma University, 3-39-22 Showa-Machi, Maebashi, Gunma, 371-8511, Japan
| | - Norihiro Ishii
- Division of Hepatobiliary and Pancreatic Surgery, Department of General Surgical Science, Graduate School of Medicine, Gunma University, 3-39-22 Showa-Machi, Maebashi, Gunma, 371-8511, Japan
| | - Mariko Tsukagoshi
- Division of Hepatobiliary and Pancreatic Surgery, Department of General Surgical Science, Graduate School of Medicine, Gunma University, 3-39-22 Showa-Machi, Maebashi, Gunma, 371-8511, Japan
| | - Takamichi Igarashi
- Division of Hepatobiliary and Pancreatic Surgery, Department of General Surgical Science, Graduate School of Medicine, Gunma University, 3-39-22 Showa-Machi, Maebashi, Gunma, 371-8511, Japan
| | - Kenichiro Araki
- Division of Hepatobiliary and Pancreatic Surgery, Department of General Surgical Science, Graduate School of Medicine, Gunma University, 3-39-22 Showa-Machi, Maebashi, Gunma, 371-8511, Japan
| | - Hayato Ikota
- Department of Diagnostic Pathology, Gunma University Hospital, Maebashi, Japan
| | - Takashi Ishige
- Department of Pediatrics, Graduate School of Medicine, Gunma University Maebashi, Maebashi, Japan
| | - Koshi Mimori
- Department of Surgery, Kyushu University Beppu Hospital, Beppu, Japan
| | - Ken Shirabe
- Division of Hepatobiliary and Pancreatic Surgery, Department of General Surgical Science, Graduate School of Medicine, Gunma University, 3-39-22 Showa-Machi, Maebashi, Gunma, 371-8511, Japan
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Alshareefy Y, Shen CY, Prekash RJ. Exploring the molecular pathogenesis, diagnosis and treatment of fibrolamellar hepatocellular carcinoma: A state of art review of the current literature. Pathol Res Pract 2023; 248:154655. [PMID: 37429175 DOI: 10.1016/j.prp.2023.154655] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/13/2023] [Accepted: 06/25/2023] [Indexed: 07/12/2023]
Abstract
This paper aims to present a detailed overview of fibrolamellar carcinoma (FLC), a variant of hepatocellular carcinoma (HCC) that accounts for approximately 1-9% of all cases a. according to the SEER database. Despite ongoing research, the aetiology of FLC tumours remains unclear. Nevertheless, FLC is believed to have a better overall prognosis than other primary liver tumours, such as hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma. This study aims to present a comprehensive overview of fibrolamellar carcinoma (FLC), with a focus on its epidemiology, pathogenesis, diagnosis, treatment, and prognosis. FLC frequently incorporate features of stomach pain, weight loss, and malaise in their clinical signs and symptoms, which are generally nonspecific Ultimately, the most common physical finding is an abdominal mass or hepatomegaly. With this said, several unusual presentations have been documented such as Budd Chiari syndrome, severe anaemia, non-bacterial thrombotic endocarditis and many more. In regards to this tumour's genetic analysis, it is characterised by a 400 kb deletion on chromosome 19 leading to a functional DNAJB1-PRKACA chimeric transcript in addition to tetraploidy in 50% of cases. FLC is chromosomally stable as compared to typical HCC. mTOR pathway activation has also been found to play a critical role in 47% of these tumours and EFGR over-expression is also evident. Fibrolamellar carcinomas (FLCs) exhibit a distinctive gross appearance, characterized by a yellow to pale tan colour, with a consistency that can vary from soft to firm and hard. In addition, a central scar is observed in 60-70% of FLC cases. The central scar is typically white or grey in colour and has a fibrous appearance, which is often surrounded by nodular, tumour-like tissue. Its histologic appearance is characterized by large polygonal cells with abundant eosinophilic cytoplasm, large vesiculated nuclei, large nucleoli, and arranged in lamellar bands of collagen fibres. Lamellar bands of fibrosis, consisting of collagen type I, III and IV, have also been identified as a distinctive histologic feature that is observed under low power magnification. Ultrasound, CT and MRI along with image guided biopsy are the primary modalities in diagnosis. Current management options include systemic therapy which has thus far been unremarkable with platinum-based therapies as well combination therapy with interferon alpha-2b being the most successful options. Surgical resection remains the primary treatment modality and there have been no advances in targeted therapies. Although the prognosis for FLC is favourable as compared to other hepatic cancer subtypes such as intrahepatic cholangiocarcinoma, there is a high rate of recurrence ranging from 33% to 100% with a median recurrence-free survival of 20-48 months. As a result of this there is a low overall cure rate associated with this tumour type and much more research is required to gain an in-depth understanding of the molecular mechanisms occurring in order to provide more adequate treatment to patients who suffer from this condition.
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Affiliation(s)
- Yasir Alshareefy
- School of Medicine, Trinity College Dublin, University of Dublin, Ireland.
| | - Chai Yu Shen
- Department of Medicine, Manipal University College, Malaysia
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Fibrolamellar Hepatocellular Carcinoma: Comprehensive Review of Diagnosis, Imaging, and Management. J Am Coll Surg 2023; 236:399-410. [PMID: 36648268 DOI: 10.1097/xcs.0000000000000476] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/18/2023]
Abstract
BACKGROUND Fibrolamellar hepatocellular carcinoma (FLC) is a rare malignancy that primarily affects patients in late adolescence and young adulthood. FLC tumors are characterized by their unique histologic features and a recently discovered genomic alteration, a chimeric fusion protein found in nearly all tumors. This review article provides the latest advancements in diagnosing, imaging, and managing FLC. STUDY DESIGN A comprehensive systematic review was performed using MEDLINE/PubMed and Web of Science databases, with the end of search date being July 1, 2022, regarding FLC diagnosis, imaging, and management. RESULTS Surgical resection remains the mainstay of therapy offering a chance for cure; however, given the incidence of metastatic disease at diagnosis and high rates of distant relapse, systemic therapies remain a crucial component of disease control. Unfortunately, few systemic therapies have demonstrated proven benefits. Consequently, recent efforts have galvanized around single-institute or small consortia-based studies specifically focused on enrolling patients with FLC or using agents with a biologic rationale. CONCLUSIONS FLC has unique demographic, radiologic, and pathologic features. The rarity of these tumors, coupled with the only recent acknowledgment of the genomic abnormality, has likely led to disease underrecognition and deprioritization of collaborative efforts to establish an evidence-based standard of care. Despite R0 resection, most patients experience recurrence. However, surgical resection is feasible for many recurrences and is associated with good survival. The role of chemotherapy is evolving, and further research is required to define its role in managing this disease.
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11
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Polychronidis G, Murtha-Lemekhova A, Fuchs J, Karathanasi E, Hoffmann K. A Multidisciplinary Approach to the Management of Fibrolamellar Carcinoma: Current Perspectives and Future Prospects. Onco Targets Ther 2022; 15:1095-1103. [PMID: 36212724 PMCID: PMC9541294 DOI: 10.2147/ott.s296127] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2022] [Accepted: 06/08/2022] [Indexed: 11/23/2022] Open
Abstract
Fibrolamellar carcinoma (FLC) is a rare primary liver tumor affecting predominantly younger and otherwise healthy patients. Typically, FLC presents with advanced disease due to the paucity of typical symptoms and no history of underlying liver disease. Depending on tumor characteristics and the patient's general condition, surgical treatment is the most promising treatment modality. Aggressive resection and liver transplantation have been utilized and are presently indispensable curative treatment options. Under certain circumstances surgical resection is also possible for metachronous metastases or local recurrence. Recent tumor biology discoveries have contributed to improved diagnostic specificity and systemic treatment options.
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Affiliation(s)
- Georgios Polychronidis
- Department of General, Visceral and Transplant Surgery, Heidelberg University Hospital, Heidelberg, 69120, Germany
| | - Anastasia Murtha-Lemekhova
- Department of General, Visceral and Transplant Surgery, Heidelberg University Hospital, Heidelberg, 69120, Germany
| | - Juri Fuchs
- Department of General, Visceral and Transplant Surgery, Heidelberg University Hospital, Heidelberg, 69120, Germany
| | - Evdokia Karathanasi
- Post-Graduate Program “Human Genetics- Genetic Counseling”, Faculty of Medicine, University of Thessaly, Larisa, Greece
| | - Katrin Hoffmann
- Department of General, Visceral and Transplant Surgery, Heidelberg University Hospital, Heidelberg, 69120, Germany,Correspondence: Katrin Hoffmann, Department of General, Visceral and Transplant Surgery, Heidelberg University Hospital, Im Neuenheimer Feld 420, Heidelberg, 69120, Germany, Email
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Loy LM, Low HM, Choi JY, Rhee H, Wong CF, Tan CH. Variant Hepatocellular Carcinoma Subtypes According to the 2019 WHO Classification: An Imaging-Focused Review. AJR Am J Roentgenol 2022; 219:212-223. [PMID: 35170359 DOI: 10.2214/ajr.21.26982] [Citation(s) in RCA: 22] [Impact Index Per Article: 7.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022]
Abstract
The 2019 5th edition of the WHO classification of digestive system tumors estimates that up to 35% of hepatocellular carcinomas (HCCs) can be classified as one of eight subtypes defined by molecular characteristics: steatohepatitic, clear cell, macrotrabecular-massive, scirrhous, chromophobe, fibrolamellar, neutrophil-rich, and lymphocyte-rich HCCs. Due to their distinct cellular and architectural characteristics, these subtypes may not display arterial phase hyperenhancement and washout appearance, which are the classic MRI features of HCC, creating challenges in noninvasively diagnosing such lesions as HCC. Moreover, certain subtypes with atypical imaging features have a worse prognosis than other HCCs. A range of distinguishing imaging features may help raise suspicion that a liver lesion represents one of these HCC subtypes. In this review, we describe the MRI features that have been reported in association with various HCC subtypes according to the 2019 WHO classification, with attention given to the current understanding of these subtypes' pathologic and molecular bases and relevance to clinical practice. Imaging findings that differentiate the subtypes from benign liver lesions and non-HCC malignancies are highlighted. Familiarity with these sub-types and their imaging features may allow the radiologist to suggest their presence, though histologic analysis remains needed to establish the diagnosis.
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Affiliation(s)
- Liang Meng Loy
- Department of Diagnostic Radiology, Tan Tock Seng Hospital, 11 Jalan Tan Tock Seng, Singapore 308433, Singapore
| | - Hsien Min Low
- Department of Diagnostic Radiology, Tan Tock Seng Hospital, 11 Jalan Tan Tock Seng, Singapore 308433, Singapore
| | - Jin-Young Choi
- Department of Radiology, Severance Hospital, Research Institute of Radiological Science and Center for Clinical Imaging Data Science, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Hyungjin Rhee
- Department of Radiology, Severance Hospital, Research Institute of Radiological Science and Center for Clinical Imaging Data Science, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Chin Fong Wong
- Department of Pathology, Tan Tock Seng Hospital, Singapore, Singapore
| | - Cher Heng Tan
- Department of Diagnostic Radiology, Tan Tock Seng Hospital, 11 Jalan Tan Tock Seng, Singapore 308433, Singapore
- Lee Kong Chian School of Medicine, Nanyang Technological University, Singapore, Singapore
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13
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MRI features of histologic subtypes of hepatocellular carcinoma: correlation with histologic, genetic, and molecular biologic classification. Eur Radiol 2022; 32:5119-5133. [PMID: 35258675 DOI: 10.1007/s00330-022-08643-4] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/22/2021] [Revised: 01/31/2022] [Accepted: 02/11/2022] [Indexed: 02/07/2023]
Abstract
HCC is a heterogeneous group of tumors in terms of histology, genetic aberration, and protein expression. Advancements in imaging techniques have allowed imaging diagnosis to become a critical part of managing HCC in the clinical setting, even without pathologic diagnosis. With the identification of many HCC subtypes, there is increasing correlative evidence between imaging phenotypes and histologic, molecular, and genetic characteristics of various HCC subtypes. In this review, current knowledge of histologic heterogeneity of HCC correlated to features on gadolinium-enhanced dynamic liver MRI will be discussed. In addition, HCC subtype classification according to transcriptomic profiles will be outlined with descriptions of histologic, genetic, and molecular characteristics of some relatively well-established morphologic subtypes, namely the low proliferation class (steatohepatitic HCC and CTNNB1-mutated HCC) and the high proliferation class (macrotrabecular-massive HCC (MTM-HCC), scirrhous HCC, and CK19-positive HCC). Characteristics of sarcomatoid HCC and fibrolamellar HCC will also be discussed. Further research on radiological characteristics of HCC subtypes may ultimately enable non-invasive diagnosis and serve as a biomarker in predicting prognosis, molecular characteristics, and therapeutic response. In the era of precision medicine, a multidisciplinary effort to develop an integrated radiologic and clinical diagnostic system of various HCC subtypes is necessary. KEY POINTS: • HCC is a heterogeneous group of tumors in terms of histology, genetic aberration, and protein expression, which can be divided into many subtypes according to transcriptome profiles. • There is increasing evidence of a correlation between imaging phenotypes and histologic, genetic, and molecular biologic characteristics of various HCC subtypes. • Imaging characteristics may ultimately enable non-invasive diagnosis and subtype characterization, serving as a biomarker for predicting prognosis, molecular characteristics, and therapeutic response.
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14
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Katabathina VS, Khanna L, Surabhi VR, Minervini M, Shanbhogue K, Dasyam AK, Prasad SR. Morphomolecular Classification Update on Hepatocellular Adenoma, Hepatocellular Carcinoma, and Intrahepatic Cholangiocarcinoma. Radiographics 2022; 42:1338-1357. [PMID: 35776676 DOI: 10.1148/rg.210206] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/11/2022]
Abstract
Hepatocellular adenomas (HCAs), hepatocellular carcinomas (HCCs), and intrahepatic cholangiocarcinomas (iCCAs) are a highly heterogeneous group of liver tumors with diverse pathomolecular features and prognoses. High-throughput gene sequencing techniques have allowed discovery of distinct genetic and molecular underpinnings of these tumors and identified distinct subtypes that demonstrate varied clinicobiologic behaviors, imaging findings, and complications. The combination of histopathologic findings and molecular profiling form the basis for the morphomolecular classification of liver tumors. Distinct HCA subtypes with characteristic imaging findings and complications include HNF1A-inactivated, inflammatory, β-catenin-activated, β-catenin-activated inflammatory, and sonic hedgehog HCAs. HCCs can be grouped into proliferative and nonproliferative subtypes. Proliferative HCCs include macrotrabecular-massive, TP53-mutated, scirrhous, clear cell, fibrolamellar, and sarcomatoid HCCs and combined HCC-cholangiocarcinoma. Steatohepatitic and β-catenin-mutated HCCs constitute the nonproliferative subtypes. iCCAs are classified as small-duct and large-duct types on the basis of the level of bile duct involvement, with significant differences in pathogenesis, molecular signatures, imaging findings, and biologic behaviors. Cross-sectional imaging modalities, including multiphase CT and multiparametric MRI, play an essential role in diagnosis, staging, treatment response assessment, and surveillance. Select imaging phenotypes can be correlated with genetic abnormalities, and identification of surrogate imaging markers may help avoid genetic testing. Improved understanding of morphomolecular features of liver tumors has opened new areas of research in the targeted therapeutics and management guidelines. The purpose of this article is to review imaging findings of select morphomolecular subtypes of HCAs, HCCs, and iCCAs and discuss therapeutic and prognostic implications. Online supplemental material is available for this article. ©RSNA, 2022.
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Affiliation(s)
- Venkata S Katabathina
- From the Department of Radiology, University of Texas Health at San Antonio, 7703 Floyd Curl Dr, San Antonio, TX 78229 (V.S.K., L.K.); Department of Radiology, University of Texas MD Anderson Cancer Center, Houston, Tex (V.R.S., S.R.P.); Departments of Pathology (M.M.) and Radiology (A.K.D.), University of Pittsburgh Medical Center, Pittsburgh, Pa; and Department of Radiology, NYU Medical Center, New York, NY (K.S.)
| | - Lokesh Khanna
- From the Department of Radiology, University of Texas Health at San Antonio, 7703 Floyd Curl Dr, San Antonio, TX 78229 (V.S.K., L.K.); Department of Radiology, University of Texas MD Anderson Cancer Center, Houston, Tex (V.R.S., S.R.P.); Departments of Pathology (M.M.) and Radiology (A.K.D.), University of Pittsburgh Medical Center, Pittsburgh, Pa; and Department of Radiology, NYU Medical Center, New York, NY (K.S.)
| | - Venkateswar R Surabhi
- From the Department of Radiology, University of Texas Health at San Antonio, 7703 Floyd Curl Dr, San Antonio, TX 78229 (V.S.K., L.K.); Department of Radiology, University of Texas MD Anderson Cancer Center, Houston, Tex (V.R.S., S.R.P.); Departments of Pathology (M.M.) and Radiology (A.K.D.), University of Pittsburgh Medical Center, Pittsburgh, Pa; and Department of Radiology, NYU Medical Center, New York, NY (K.S.)
| | - Marta Minervini
- From the Department of Radiology, University of Texas Health at San Antonio, 7703 Floyd Curl Dr, San Antonio, TX 78229 (V.S.K., L.K.); Department of Radiology, University of Texas MD Anderson Cancer Center, Houston, Tex (V.R.S., S.R.P.); Departments of Pathology (M.M.) and Radiology (A.K.D.), University of Pittsburgh Medical Center, Pittsburgh, Pa; and Department of Radiology, NYU Medical Center, New York, NY (K.S.)
| | - Krishna Shanbhogue
- From the Department of Radiology, University of Texas Health at San Antonio, 7703 Floyd Curl Dr, San Antonio, TX 78229 (V.S.K., L.K.); Department of Radiology, University of Texas MD Anderson Cancer Center, Houston, Tex (V.R.S., S.R.P.); Departments of Pathology (M.M.) and Radiology (A.K.D.), University of Pittsburgh Medical Center, Pittsburgh, Pa; and Department of Radiology, NYU Medical Center, New York, NY (K.S.)
| | - Anil K Dasyam
- From the Department of Radiology, University of Texas Health at San Antonio, 7703 Floyd Curl Dr, San Antonio, TX 78229 (V.S.K., L.K.); Department of Radiology, University of Texas MD Anderson Cancer Center, Houston, Tex (V.R.S., S.R.P.); Departments of Pathology (M.M.) and Radiology (A.K.D.), University of Pittsburgh Medical Center, Pittsburgh, Pa; and Department of Radiology, NYU Medical Center, New York, NY (K.S.)
| | - Srinivasa R Prasad
- From the Department of Radiology, University of Texas Health at San Antonio, 7703 Floyd Curl Dr, San Antonio, TX 78229 (V.S.K., L.K.); Department of Radiology, University of Texas MD Anderson Cancer Center, Houston, Tex (V.R.S., S.R.P.); Departments of Pathology (M.M.) and Radiology (A.K.D.), University of Pittsburgh Medical Center, Pittsburgh, Pa; and Department of Radiology, NYU Medical Center, New York, NY (K.S.)
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15
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Cross-Sectional Imaging Findings of Atypical Liver Malignancies and Diagnostic Pitfalls. Radiol Clin North Am 2022; 60:775-794. [DOI: 10.1016/j.rcl.2022.05.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
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Abdelhamed W, El-Kassas M. Fibrolamellar hepatocellular carcinoma: A rare but unpleasant event. World J Gastrointest Oncol 2022; 14:1103-1114. [PMID: 35949219 PMCID: PMC9244987 DOI: 10.4251/wjgo.v14.i6.1103] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/15/2022] [Revised: 03/19/2022] [Accepted: 05/08/2022] [Indexed: 02/06/2023] Open
Abstract
Fibrolamellar carcinoma (FLC) is a rare variant of hepatocellular carcinoma (HCC), comprising 1%-9% of all HCCs. FLC is a poorly understood malignancy, which seems to be more prevalent in young patients with no underlying liver diseases. The term "fibrolamellar" is derived from thick fibrous collagen bands surrounding the tumor cells. Unlike HCC, cirrhosis and viral hepatitis infection are not predisposing to FLC, and it is not associated with elevations in serum alpha-fetoprotein. FLC patients often present with vague abdominal pain, nausea, malaise, and weight loss. Most cases present are at an advanced stage at the time of initial diagnosis. However, curative treatment options can still be offered to up to 70% of patients. Surgery (resection/liver transplantation) is the mainstay of treatment and the only potentially curative option. FLCs have been less chemo-responsive than the conventional HCC, however, in advanced cases, multimodality treatments can be effective. Recent advances in molecular studies of FLC have found a unique DNAJB1-PRKACA fusion transcript in most of the cases studied. The review aims to describe clinical characteristics, diagnostic methods, and therapeutic modalities for this rare tumor to raise awareness among clinicians and surgeons.
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Affiliation(s)
- Walaa Abdelhamed
- Department of Endemic Medicine, Sohag University, Sohag 14322, Egypt
| | - Mohamed El-Kassas
- Department of Endemic Medicine, Faculty of Medicine, Helwan University, Cairo 11795, Egypt
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17
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Onishi S, Tajika M, Tanaka T, Yamada K, Kamiya T, Natsume S, Shimizu Y, Niwa Y. Juvenile Hepatocellular Carcinoma in a Healthy Liver. Intern Med 2022; 61:1531-1536. [PMID: 34670900 PMCID: PMC9177355 DOI: 10.2169/internalmedicine.8305-21] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/11/2021] [Accepted: 09/02/2021] [Indexed: 11/06/2022] Open
Abstract
Primary hepatocellular carcinoma (HCC) in patients <30 years old is extremely rare. In younger patients, HCC develops against a background of persistent hepatitis B virus infection. We herein report a 23-year-old woman with HCC with all-negative hepatitis virus markers developing in an apparently healthy liver. Imaging studies showed a 50-mm hypervascular mass in segment 4 of the left liver lobe, compatible with HCC. The patient underwent surgical resection. A histological examination showed the presence of poorly differentiated HCC. The patient was diagnosed with HCC developing in a healthy liver. This is an extremely rare case of non-B non-C HCC.
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Affiliation(s)
- Sachiyo Onishi
- Department of Endoscopy, Aichi Cancer Center Hospital, Japan
| | - Masahiro Tajika
- Department of Endoscopy, Aichi Cancer Center Hospital, Japan
| | - Tsutomu Tanaka
- Department of Endoscopy, Aichi Cancer Center Hospital, Japan
| | - Keisaku Yamada
- Department of Endoscopy, Aichi Cancer Center Hospital, Japan
| | - Tomoyasu Kamiya
- Department of Endoscopy, Aichi Cancer Center Hospital, Japan
| | - Seiji Natsume
- Department of Gastroenterological Surgery, Aichi Cancer Center Hospital, Japan
| | - Yasuhiro Shimizu
- Department of Gastroenterological Surgery, Aichi Cancer Center Hospital, Japan
| | - Yasumasa Niwa
- Department of Endoscopy, Aichi Cancer Center Hospital, Japan
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18
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Diagnosis and Follow-up of Incidental Liver Lesions in Children. J Pediatr Gastroenterol Nutr 2022; 74:320-327. [PMID: 34984985 DOI: 10.1097/mpg.0000000000003377] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Abstract
Incidental liver lesions are identified in children without underlying liver disease or increased risk of hepatic malignancy in childhood. Clinical and imaging evaluation of incidental liver lesions can be complex and may require a multidisciplinary approach. This review aims to summarize the diagnostic process and follow-up of incidental liver lesions based on review of the literature, use of state-of-the-art imaging, and our institutional experience. Age at presentation, gender, alpha fetoprotein levels, tumor size, and imaging characteristics should all be taken into consideration to optimize diagnosis process. Some lesions, such as simple liver cyst, infantile hemangioma, focal nodular hyperplasia (FNH), and focal fatty lesions, have specific imaging characteristics. Recently, contrast-enhanced ultrasound (CEUS) was Food and Drug Administration (FDA)-approved for the evaluation of pediatric liver lesions. CEUS is most specific in lesions smaller than 3 cm and is most useful in the diagnosis of infantile hemangioma, FNH, and focal fatty lesions. The use of hepatobiliary contrast in MRI increases specificity in the diagnosis of FNH. Recently, lesion characteristics in MRI were found to correlate with subtypes of hepatocellular adenomas and associated risk for hemorrhage and malignant transformation. Biopsy should be considered when there are no specific imaging characteristics of a benign lesion. Surveillance with imaging and alpha fetoprotein (AFP) should be performed to confirm the stability of lesions when the diagnosis cannot be determined, and whenever biopsy is not feasible.
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19
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Differential Diagnosis of Hepatic Mass with Central Scar: Focal Nodular Hyperplasia Mimicking Fibrolamellar Hepatocellular Carcinoma. Diagnostics (Basel) 2021; 12:diagnostics12010044. [PMID: 35054211 PMCID: PMC8775045 DOI: 10.3390/diagnostics12010044] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/23/2021] [Revised: 12/21/2021] [Accepted: 12/22/2021] [Indexed: 01/15/2023] Open
Abstract
Fibrolamellar hepatocellular carcinoma is a primary hepatic tumor that usually appears in young adults. Radical surgery is considered curative for this kind of tumor, so early diagnosis becomes essential for the prognosis of the patients. The main characteristic of this entity is the central scar, which is the center of differential diagnosis. We report the case of a 30-year-old man who was diagnosed with fibrolamellar hepatocellular carcinoma by ultrasonography. Contrast-enhanced CT confirmed this diagnosis, and the patient underwent a [18F] fluorocholine PET/CT. Hypermetabolism and the morphology in the nuclear medicine exploration suggest neoplastic nature of the lesion. Radical surgery was performed, and histopathologic analysis was performed, which resulted in focal nodular hyperplasia. Hepatic masses with central scar could have a difficult differential diagnosis, and focal nodular hyperplasia could mimic fibrolamellar hepatocellular carcinoma imaging patterns. These morphofunctional characteristics have not been described in [18F] Fluorocholine PET/CT, so there is a need to find out the potential role PET/CT in the differential diagnosis of hepatic mass with central scar.
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Takahashi A, Imamura H, Ito R, Kawano F, Gyoda Y, Ichida H, Yoshioka R, Mise Y, Fukumura Y, Sano K, Saiura A. A case report of fibrolamellar hepatocellular carcinoma, with particular reference to preoperative diagnosis, value of molecular genetic diagnosis, and cell origin. Surg Case Rep 2021; 7:208. [PMID: 34533614 PMCID: PMC8448801 DOI: 10.1186/s40792-021-01295-4] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/12/2021] [Accepted: 09/10/2021] [Indexed: 01/12/2023] Open
Abstract
Background Fibrolamellar hepatocellular carcinoma (FL-HCC) is a liver tumor that occurs almost exclusively in young adults without underlying liver disease. In spite of its distinct clinical characteristics and specific imaging findings, preoperative diagnosis is often difficult due to the extremely low incidence of the tumor. Although FL-HCC shows particular morphological features on H&E-stained tissue sections, differential diagnosis from ordinary HCC, especially the scirrhous variant of HCC, and intrahepatic cholangiocarcinoma needs additional immunohistochemical (IHC) analyses and/or molecular genetic testing. Case presentation A 21-year-old male patient was referred to our hospital for further evaluation of a large liver mass. Abdominal ultrasound examination, contrast-enhanced computed tomography, and magnetic resonance imaging revealed a well-defined hypervascular lobulated liver mass, 11 × 11 cm in diameter, with a central scar and calcification, in segments 5/8. Under the diagnosis of FL-HCC, we carried out extended anterior sectorectomy, including a part of segment 4. On microscopic examination, the tumor was composed of proliferating polygonal cells with abundant eosinophilic granular cytoplasm containing nuclei with vesicular chromatin and enlarged nucleoli, in an abundant stroma. Collagen fibers arranged in a parallel lamellar pattern were seen in the tumor stroma. These findings, together with the results of subsequent IHC analyses using HAS, CK7, and CD 67, we made the diagnosis of FL-HCC, which was further confirmed by detection of the DNAJB1-PRKACA fusion gene in the tumor cells by RT-PCR. Conclusion FL-HCC shows distinct imaging appearances. Although it also has characteristic morphological features, combined use of IHC and/or molecular genetic studies are necessary for the final diagnosis.
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Affiliation(s)
- Atsushi Takahashi
- Department of Hepatobiliary-Pancreatic Surgery, Juntendo University School of Medicine, Juntendo University Hospital, 3-1-1 Hongo, Bunkyo-Ku, Tokyo, 113-0033, Japan
| | - Hiroshi Imamura
- Department of Hepatobiliary-Pancreatic Surgery, Juntendo University School of Medicine, Juntendo University Hospital, 3-1-1 Hongo, Bunkyo-Ku, Tokyo, 113-0033, Japan
| | - Ryota Ito
- Department of Hepatobiliary-Pancreatic Surgery, Juntendo University School of Medicine, Juntendo University Hospital, 3-1-1 Hongo, Bunkyo-Ku, Tokyo, 113-0033, Japan
| | - Fumihiro Kawano
- Department of Hepatobiliary-Pancreatic Surgery, Juntendo University School of Medicine, Juntendo University Hospital, 3-1-1 Hongo, Bunkyo-Ku, Tokyo, 113-0033, Japan
| | - Yu Gyoda
- Department of Hepatobiliary-Pancreatic Surgery, Juntendo University School of Medicine, Juntendo University Hospital, 3-1-1 Hongo, Bunkyo-Ku, Tokyo, 113-0033, Japan
| | - Hirofumi Ichida
- Department of Hepatobiliary-Pancreatic Surgery, Juntendo University School of Medicine, Juntendo University Hospital, 3-1-1 Hongo, Bunkyo-Ku, Tokyo, 113-0033, Japan
| | - Ryuji Yoshioka
- Department of Hepatobiliary-Pancreatic Surgery, Juntendo University School of Medicine, Juntendo University Hospital, 3-1-1 Hongo, Bunkyo-Ku, Tokyo, 113-0033, Japan
| | - Yoshihiro Mise
- Department of Hepatobiliary-Pancreatic Surgery, Juntendo University School of Medicine, Juntendo University Hospital, 3-1-1 Hongo, Bunkyo-Ku, Tokyo, 113-0033, Japan
| | - Yuki Fukumura
- Department of Pathology, Juntendo University School of Medicine, Tokyo, Japan
| | - Katsuhiro Sano
- Department of Radiology, Juntendo University School of Medicine, Tokyo, Japan
| | - Akio Saiura
- Department of Hepatobiliary-Pancreatic Surgery, Juntendo University School of Medicine, Juntendo University Hospital, 3-1-1 Hongo, Bunkyo-Ku, Tokyo, 113-0033, Japan.
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Depauw L, De Weerdt G, Gys B, Demeulenaere S, Mebis W, Ysebaert D. Pediatric fibrolamellar hepatocellular carcinoma: case report and review of the literature. Acta Chir Belg 2021; 121:204-210. [PMID: 34082642 DOI: 10.1080/00015458.2019.1660060] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
Abstract
BACKGROUND A 13-year-old boy presented with acute abdominal pain in the right upper quadrant without previous trauma. Abdominal ultrasound (US) revealed a mass in the right liver lobe with free intraperitoneal fluid, suggestive for hemoperitoneum. Magnetic resonance imaging confirmed a subcapsular lesion (5.7 × 4.6 × 4.1 cm), suggestive for fibrolamellar hepatocellular carcinoma (FL-HCC). Positron emission tomography-computed tomography revealed mild to moderate fluorodeoxyglucose (FDG) avidity, with no other FDG avid lesions. Hepatic tumor markers were negative. CASE REPORT An elective right hepatectomy with cholecystectomy and hilar lymph node resection was performed. RESULTS Histology showed a central fibrous scar and confirmed a FL-HCC (pT1bN0M0). The resected lymph nodes were tumor-free. Treatment of FL-HCC should consist of complete tumor resection with concurrent lymph node resection +/- orthotopic liver transplantation. Long-term follow-up is advised. A follow-up interval of 3-4 months in the first 2 years after surgical resection can be justified as FL-HCC have a high recurrence rate of more than 50% within 10-33 months. CONCLUSIONS Malignancy can be a rare cause of abdominal pain in pediatric patients. An abdominal US is essential to prevent misdiagnosis. Treatment of FL-HCC should consist of R0 tumor resection with concurrent lymphadenectomy +/- orthotopic liver transplantation.
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Affiliation(s)
- Laura Depauw
- Department of Hepatobiliairy Surgery, Antwerp University Hospital, Antwerp, Belgium
| | - Glenn De Weerdt
- Department of Hepatobiliairy Surgery, Antwerp University Hospital, Antwerp, Belgium
| | - Ben Gys
- Department of Hepatobiliairy Surgery, Antwerp University Hospital, Antwerp, Belgium
| | - Sofie Demeulenaere
- Department of Hepatobiliairy Surgery, Antwerp University Hospital, Antwerp, Belgium
| | - Wouter Mebis
- Department of Hepatobiliairy Surgery, Antwerp University Hospital, Antwerp, Belgium
| | - Dirk Ysebaert
- Department of Hepatobiliairy Surgery, Antwerp University Hospital, Antwerp, Belgium
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22
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Dong Y, Wang WP, Mao F, Zhang Q, Yang D, Tannapfel A, Meloni MF, Neye H, Clevert DA, Dietrich CF. Imaging Features of Fibrolamellar Hepatocellular Carcinoma with Contrast-Enhanced Ultrasound. ULTRASCHALL IN DER MEDIZIN (STUTTGART, GERMANY : 1980) 2021; 42:306-313. [PMID: 32102105 DOI: 10.1055/a-1110-7124] [Citation(s) in RCA: 23] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 06/10/2023]
Abstract
PURPOSE Fibrolamellar hepatocellular carcinoma (f-HCC) is a rare primary liver tumor. Imaging plays an important role in diagnosis. The aim of this retrospective study was to analyze contrast-enhanced ultrasound (CEUS) features of histologically proven f-HCC in comparison to benign focal nodular hyperplasia (FNH). MATERIALS & METHODS 16 patients with histologically proven f-HCC lesions and 30 patients with FNH lesions were retrospectively reviewed regarding CEUS features to determine the malignant or benign nature of the focal liver lesions (FLL). Five radiologists assessed the CEUS enhancement pattern and came to a consensus using the EFSUMB (European Federation of Societies for Ultrasound in Medicine and Biology) guideline criteria. RESULTS Fibrolamellar hepatocellular carcinoma manifested as a single and huge FLL. On CEUS, f-HCC showed heterogeneous hyperenhancement in the arterial phase and hypoenhancement (16/16, 100 %) in the portal venous and late phases (PVLP) as a sign of malignancy. In contrast to the hypoenhancement of f-HCC in the PVLP, all patients with FNH showed hyperenhancement as the most distinctive feature (P < 0.01). 8 f-HCC lesions showed a central scar as an unenhanced area (8/16, 50.0 %), which could also be detected in 53.3 % (16/30) of FNH lesions (P > 0.05). CONCLUSION By analyzing the hypoenhancement in the PVLP, CEUS imaging reliably diagnosed f-HCC as a malignant FLL. CEUS also showed differentiation between f-HCC and FNH lesions, showing similar non-enhanced central scars, whereas f-HCC lesions showed peripheral hyperenhancement in the arterial phase and early washout in the PVLP.
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Affiliation(s)
- Yi Dong
- Ultrasound, Zhongshan-Hospital Fudan-University, Shanghai, China
| | - Wen-Ping Wang
- Ultrasound, Zhongshan-Hospital Fudan-University, Shanghai, China
| | - Feng Mao
- Ultrasound, Zhongshan-Hospital Fudan-University, Shanghai, China
| | - Qi Zhang
- Ultrasound, Zhongshan-Hospital Fudan-University, Shanghai, China
| | - Daohui Yang
- Ultrasound, Zhongshan-Hospital Fudan-University, Shanghai, China
| | | | | | - Holger Neye
- Department of Internal Medicine II, Helios Hospital Emil von Behring, Berlin, Germany
| | - Dirk-André Clevert
- Interdisciplinary Ultrasound-Center, Department of Radiology, University Hospital Munich, München, Germany
| | - Christoph F Dietrich
- Department Allgemeine Innere Medizin, Kliniken Hirslanden Beau Site, Salem und Permanence, Switzerland
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23
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Tan N, Apostolov R, Au M, Vasudevan A, Nicoll A. Investigating Liver Masses in Young Adults. Am J Med 2021; 134:596-598. [PMID: 33245924 DOI: 10.1016/j.amjmed.2020.10.028] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/03/2020] [Revised: 10/16/2020] [Accepted: 10/21/2020] [Indexed: 11/18/2022]
Affiliation(s)
- Natassia Tan
- Department of Gastroenterology and Hepatology, Eastern Health, Box Hill, Victoria, Australia.
| | - Ross Apostolov
- Department of Gastroenterology and Hepatology, Eastern Health, Box Hill, Victoria, Australia
| | - Minnie Au
- Department of Gastroenterology and Hepatology, Eastern Health, Box Hill, Victoria, Australia
| | - Abhinav Vasudevan
- Department of Gastroenterology and Hepatology, Eastern Health, Box Hill, Victoria, Australia; Eastern Health Clinical School, Monash University, Victoria, Australia
| | - Amanda Nicoll
- Department of Gastroenterology and Hepatology, Eastern Health, Box Hill, Victoria, Australia; Eastern Health Clinical School, Monash University, Victoria, Australia
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Al Zahrani A, Alfakeeh A. Fibrolamellar hepatocellular carcinoma treated with atezolizumab and bevacizumab: two case reports. J Med Case Rep 2021; 15:132. [PMID: 33722275 PMCID: PMC7962306 DOI: 10.1186/s13256-021-02695-8] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/09/2020] [Accepted: 01/26/2021] [Indexed: 02/07/2023] Open
Abstract
Background Fibrolamellar hepatocellular carcinoma is a unique tumor of the liver that differs from the classical hepatocellular carcinoma in diagnosis, behavior, and possibly treatment. There is usually absent underlying liver disease, and it usually occurs in young patients. The survival outcomes in localized fibrolamellar hepatocellular carcinoma are perhaps better than in classical hepatocellular carcinoma if treated early and radically. On the other hand, the prognosis remains poor for locally advanced and metastatic fibrolamellar hepatocellular carcinoma. Many reports suggested a limited benefit from systemic chemotherapy. Sorafenib also did not show major effects on fibrolamellar hepatocellular carcinoma. Given the rarity of fibrolamellar hepatocellular carcinoma, lack of large studies, and absence of standard treatment, the treatment decisions rely on case reports, previously reported cases series, and expert opinions. Recent studies have shown promising effects of immunotherapy with checkpoint inhibitors in the first- and second-line therapy of hepatocellular carcinoma. Atezolizumab with bevacizumab regimen has been approved recently as a first-line treatment for classical hepatocellular carcinoma. Currently, there are no reports yet on the use of atezolizumab with bevacizumab for fibrolamellar hepatocellular carcinoma. Case report In this article, we present two Arabic patients with advanced fibrolamellar hepatocellular carcinoma who received atezolizumab and bevacizumab combinations but did not show any clinical benefits. Conclusion While atezolizumab and bevacizumab combinations had shown benefits in classical hepatocellular carcinoma, the current data showed a lack of benefit and tumor response in fibrolamellar hepatocellular carcinoma.
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Affiliation(s)
- Ali Al Zahrani
- Department of Medical Oncology, Comprehensive Cancer Center, King Fahad Medical City, Makkah Al Mukarramah Branch Road, Riyadh, 12231, Kingdom of Saudi Arabia.
| | - Ali Alfakeeh
- Department of Medical Oncology, Comprehensive Cancer Center, King Fahad Medical City, Makkah Al Mukarramah Branch Road, Riyadh, 12231, Kingdom of Saudi Arabia
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25
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Bello HR, Mahdi ZK, Lui SK, Nandwana SB, Harri PA, Davarpanah AH. Hepatocellular Carcinoma With Atypical Imaging Features: Review of the Morphologic Hepatocellular Carcinoma Subtypes With Radiology-Pathology Correlation. J Magn Reson Imaging 2021; 55:681-697. [PMID: 33682266 DOI: 10.1002/jmri.27553] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/18/2020] [Revised: 01/25/2021] [Accepted: 01/26/2021] [Indexed: 12/17/2022] Open
Abstract
Hepatocellular carcinoma (HCC) is the fastest growing cause of cancer death in the United States with the incidence rate more than doubling in 20 years. HCC is unique since a noninvasive diagnosis can be achieved with imaging alone when specific clinical criteria and imaging characteristics are met, obviating the need for tissue sampling. However, HCC is a highly heterogeneous neoplasm. Atypical HCC subtypes vary significantly in their morphology, which can be attributed to specific histologic and molecular features, and can cause deviations from the classic imaging characteristics. The different morphologic subtypes of HCC frequently present a diagnostic challenge for radiologists and pathologists since their imaging and pathologic features can overlap with those of non-HCC malignancies. Identifying an atypical subtype can have important clinical implications. Liver transplant, albeit a scarce and limited resource, is the optimal treatment for conventional HCC, potentially curing both the tumor and the underlying pre-malignant condition. Some HCC subtypes as well as mimickers are associated with unacceptably high recurrence and poor outcome after transplant, and there remains limited data on the role and prognosis of liver transplantation for treatment of rare HCC subtypes. Other subtypes tend to recur later than classic HCC, potentially requiring a different follow-up scheme. This review will discuss the appearance of different HCC subtypes in relation to their histopathologic features. LEVEL OF EVIDENCE: 5 TECHNICAL EFFICACY: Stage 3.
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Affiliation(s)
- Hernan R Bello
- Department of Radiology and Imaging Sciences, Emory University School of Medicine, Atlanta, Georgia, USA
| | - Zaid K Mahdi
- Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA
| | - Shu K Lui
- Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Atlanta, Georgia, USA
| | - Sadhna B Nandwana
- Department of Radiology and Imaging Sciences, Emory University School of Medicine, Atlanta, Georgia, USA
| | - Peter A Harri
- Department of Radiology and Imaging Sciences, Emory University School of Medicine, Atlanta, Georgia, USA
| | - Amir H Davarpanah
- Department of Radiology and Imaging Sciences, Emory University School of Medicine, Atlanta, Georgia, USA
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Subramanian M, Low HM, Kim MJ, Tan CH. Benign focal liver lesions masquerading as primary liver cancers on MRI. ACTA ACUST UNITED AC 2021; 26:168-175. [PMID: 32229432 DOI: 10.5152/dir.2019.19235] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (ICC) are the most common primary liver malignancies. HCC and ICC have characteristic imaging findings, but a number of benign entities can appear similar and can cause diagnostic dilemma. Ideally, accurate and timely diagnosis of these conditions can help the patient to avoid a needle biopsy or even unnecessary treatment. In this article, we present various benign liver lesions that display imaging characteristics that are similar to HCC and ICC on magnetic resonance imaging (MRI) and discuss salient features that may assist in accurate diagnosis.
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Affiliation(s)
| | - Hsien Min Low
- Department of Diagnostic Radiology, Tan Tock Seng Hospital, Novena, Singapore
| | - Myeong-Jin Kim
- Department of Diagnostic Radiology, Severance Hospital, Yonsei University College of Medicine, Seoul, South Korea
| | - Cher Heng Tan
- Department of Diagnostic Radiology, Tan Tock Seng Hospital, Novena, Singapore
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27
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Lucas B, Ravishankar S, Pateva I. Pediatric Primary Hepatic Tumors: Diagnostic Considerations. Diagnostics (Basel) 2021; 11:333. [PMID: 33670452 PMCID: PMC7922091 DOI: 10.3390/diagnostics11020333] [Citation(s) in RCA: 25] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/29/2020] [Revised: 02/12/2021] [Accepted: 02/15/2021] [Indexed: 02/06/2023] Open
Abstract
The liver is the third most common site of abdominal tumors in children. This review article aims to summarize current evidence surrounding identification and diagnosis of primary hepatic tumors in the pediatric population based upon clinical presentation, epidemiology, and risk factors as well as classical imaging, histopathological, and molecular diagnostic findings. Readers will be able to recognize the features and distinguish between benign and malignant hepatic tumors within different age groups.
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Affiliation(s)
- Bryony Lucas
- Rainbow Babies and Children’s Hospital—Department of Pediatrics, University Hospitals Cleveland Medical Center, Cleveland, OH 44106, USA
| | - Sanjita Ravishankar
- Rainbow Babies and Children’s Hospital—Department of Pathology, University Hospitals Cleveland Medical Center, Cleveland, OH 44106, USA
| | - Irina Pateva
- Rainbow Babies and Children’s Hospital—Department of Pediatric Hematology and Oncology, University Hospitals Cleveland Medical Center, Cleveland, OH 44106, USA
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28
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Gigante E, Paradis V, Ronot M, Cauchy F, Soubrane O, Ganne-Carrié N, Nault JC. New insights into the pathophysiology and clinical care of rare primary liver cancers. JHEP Rep 2021; 3:100174. [PMID: 33205035 PMCID: PMC7653076 DOI: 10.1016/j.jhepr.2020.100174] [Citation(s) in RCA: 25] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/04/2020] [Revised: 08/06/2020] [Accepted: 08/10/2020] [Indexed: 02/07/2023] Open
Abstract
Hepatocholangiocarcinoma, fibrolamellar carcinoma, hepatic haemangioendothelioma and hepatic angiosarcoma represent less than 5% of primary liver cancers. Fibrolamellar carcinoma and hepatic haemangioendothelioma are driven by unique somatic genetic alterations (DNAJB1-PRKCA and CAMTA1-WWTR1 fusions, respectively), while the pathogenesis of hepatocholangiocarcinoma remains more complex, as suggested by its histological diversity. Histology is the gold standard for diagnosis, which remains challenging even in an expert centre because of the low incidences of these liver cancers. Resection, when feasible, is the cornerstone of treatment, together with liver transplantation for hepatic haemangioendothelioma. The role of locoregional therapies and systemic treatments remains poorly studied. In this review, we aim to describe the recent advances in terms of diagnosis and clinical management of these rare primary liver cancers.
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Key Words
- 5-FU, 5-Fluorouracil
- AFP, alpha-fetoprotein
- APHE, arterial phase hyperenhancement
- CA19-9, carbohydrate antigen 19-9
- CCA, cholangiocarcinoma
- CEUS, contrast-enhanced ultrasound
- CK, cytokeratin
- CLC, cholangiolocellular carcinoma
- EpCAM, epithelial cell adhesion molecule
- FISH, fluorescence in situ hybridisation
- FLC, fibrolamellar carcinoma
- Fibrolamellar carcinoma
- HAS, hepatic angiosarcoma
- HCC, hepatocellular carcinoma
- HEH, hepatic epithelioid haemangioendothelioma
- HepPar1, hepatocyte specific antigen antibody
- Hepatic angiosarcoma
- Hepatic hemangioendothelioma
- Hepatocellular carcinoma
- Hepatocholangiocarcinoma
- IHC, immunohistochemistry
- LI-RADS, liver imaging reporting and data system
- LT, liver transplantation
- Mixed tumor
- RT-PCR, reverse transcription PCR
- SIRT, selective internal radiation therapy
- TACE, transarterial chemoembolisation
- WHO, World Health Organization
- cHCC-CCA, combined hepatocholangiocarcinoma
- iCCA, intrahepatic cholangiocarcinoma
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Affiliation(s)
- Elia Gigante
- Service d’hépatologie, Hôpital Avicenne, Hôpitaux Universitaires Paris-Seine-Saint-Denis, Assistance-Publique Hôpitaux de Paris, Bobigny, France
- Centre de recherche sur l’inflammation, Inserm, Université de Paris, INSERM UMR 1149 « De l'inflammation au cancer », Paris, France
- Unité de Formation et de Recherche Santé Médecine et Biologie Humaine, Université Paris 13, Paris, France
| | - Valérie Paradis
- Centre de recherche sur l’inflammation, Inserm, Université de Paris, INSERM UMR 1149 « De l'inflammation au cancer », Paris, France
- Service d'anatomie pathologique, Hôpitaux Universitaires Paris-Nord-Val-de-Seine, Assistance-Publique Hôpitaux de Paris, Clichy, France
- Université de Paris, Paris, France
| | - Maxime Ronot
- Centre de recherche sur l’inflammation, Inserm, Université de Paris, INSERM UMR 1149 « De l'inflammation au cancer », Paris, France
- Service de radiologie, Hôpital Beaujon, Hôpitaux Universitaires Paris-Nord-Val-de-Seine, Assistance-Publique Hôpitaux de Paris, Clichy, France
- Université de Paris, Paris, France
| | - François Cauchy
- Centre de recherche sur l’inflammation, Inserm, Université de Paris, INSERM UMR 1149 « De l'inflammation au cancer », Paris, France
- Service de chirurgie hépato-bilio-pancréatique et transplantation hépatique, Hôpitaux Universitaires Paris-Nord-Val-de-Seine, Assistance-Publique Hôpitaux de Paris, Clichy, France
- Université de Paris, Paris, France
| | - Olivier Soubrane
- Centre de recherche sur l’inflammation, Inserm, Université de Paris, INSERM UMR 1149 « De l'inflammation au cancer », Paris, France
- Service de chirurgie hépato-bilio-pancréatique et transplantation hépatique, Hôpitaux Universitaires Paris-Nord-Val-de-Seine, Assistance-Publique Hôpitaux de Paris, Clichy, France
- Université de Paris, Paris, France
| | - Nathalie Ganne-Carrié
- Service d’hépatologie, Hôpital Avicenne, Hôpitaux Universitaires Paris-Seine-Saint-Denis, Assistance-Publique Hôpitaux de Paris, Bobigny, France
- Unité de Formation et de Recherche Santé Médecine et Biologie Humaine, Université Paris 13, Paris, France
- Centre de Recherche des Cordeliers, Inserm, Sorbonne Université, Université Paris, INSERM UMR 1138, Functional Genomics of Solid Tumors, F-75006, Paris, France
| | - Jean-Charles Nault
- Service d’hépatologie, Hôpital Avicenne, Hôpitaux Universitaires Paris-Seine-Saint-Denis, Assistance-Publique Hôpitaux de Paris, Bobigny, France
- Unité de Formation et de Recherche Santé Médecine et Biologie Humaine, Université Paris 13, Paris, France
- Centre de Recherche des Cordeliers, Inserm, Sorbonne Université, Université Paris, INSERM UMR 1138, Functional Genomics of Solid Tumors, F-75006, Paris, France
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Abstract
With the increasing use of cross-sectional imaging, the detection of incidental liver lesions has become more common. Accurate and reliable characterisation of these is vital for optimal patient care. Owing to the great improvements in medical imaging technology in recent years, particularly magnetic resonance imaging, it is now possible to characterise a significant proportion of these non-invasively. This is of paramount importance in improving patient safety and reducing costs by avoiding unnecessary biopsies. This article gives a synopsis of the different imaging modalities for liver. It depicts the salient imaging features of the common benign and malignant focal liver lesions on different imaging modalities, with emphasis on magnetic resonance imaging. It demonstrates the pseudolesions, variants, mimics and pitfalls that occur in liver imaging. The tailored magnetic resonance imaging protocols including abbreviated ones, the contrast agents and the pathway for managing incidental liver lesions in the author's institution are covered.
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Affiliation(s)
- Anitha James
- Imaging Department, University Hospital of North Midlands, Stoke-on-Trent, UK
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30
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Razik A, Malla S, Goyal A, Gamanagatti S, Kandasamy D, Das CJ, Sharma R, Gupta AK. Unusual Primary Neoplasms of the Adult Liver: Review of Imaging Appearances and Differential Diagnosis. Curr Probl Diagn Radiol 2020; 51:73-85. [PMID: 33199074 DOI: 10.1067/j.cpradiol.2020.10.001] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/16/2020] [Accepted: 10/16/2020] [Indexed: 12/24/2022]
Abstract
The radiological appearance of common primary hepatic tumors such as hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) is widely recognized. Hepatic masses with unusual histology are occasionally encountered, but seldom suspected on imaging. However, many possess characteristic imaging findings, which when assessed along with the clinical and demographic background and serum tumor markers, may enable a prospective diagnosis. This review attempts to familiarize the reader with the clinicopathological characteristics, imaging manifestations, and differential diagnosis of these unusual liver tumors in adults. Biphenotypic primary liver carcinoma is suspected in masses showing distinct areas of HCC and CCA-type enhancement pattern in cirrhotic livers. Fibrolamellar carcinoma occurs in young individuals without underlying chronic liver disease and shows a characteristic T2-hypointense scar frequently showing calcification. Perivascular epithelioid cell tumors are differentials for any arterial hyperenhancing mass in the noncirrhotic liver, particularly in patients with tuberous sclerosis. Multifocal subcapsular tumors showing target-like morphology, capsular retraction and "lollipop" sign are suspicious for epithelioid hemangioendothelioma. On the other hand, multiple hemorrhagic lesions showing patchy areas of bizarre-shaped arterial phase hyperenhancement are suspicious for angiosarcoma. Primary hepatic lymphoma (PHL) is suspected when patients with immunosuppression present with solitary or multifocal masses that insinuate around vessels and bile ducts without causing luminal narrowing. Intense diffusion restriction and low-level homogeneous or target-like enhancement are also ancillary features of PHL. Primary hepatic neuroendocrine tumor shows uptake on Ga-68 DOTANOC PET/CT. Although a straightforward diagnosis may be difficult in these cases, awareness of the characteristic imaging appearances is helpful in suspecting the diagnosis.
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Affiliation(s)
- Abdul Razik
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India
| | - Sundeep Malla
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India
| | - Ankur Goyal
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India
| | - Shivanand Gamanagatti
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India
| | | | - Chandan Jyoti Das
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India
| | - Raju Sharma
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India.
| | - Arun Kumar Gupta
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India
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31
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LeGout JD, Bailey RE, Bolan CW, Bowman AW, Chen F, Cernigliaro JG, Alexander LF. Multimodality Imaging of Abdominopelvic Tumors with Venous Invasion. Radiographics 2020; 40:2098-2116. [PMID: 33064623 DOI: 10.1148/rg.2020200047] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
A broad range of abdominal and pelvic tumors can manifest with or develop intraluminal venous invasion. Imaging features at cross-sectional modalities and contrast-enhanced US that allow differentiation of tumor extension within veins from bland thrombus include the expansile nature of tumor thrombus and attenuation and enhancement similar to those of the primary tumor. Venous invasion is a distinctive feature of hepatocellular carcinoma and renal cell carcinoma with known prognostic and treatment implications; however, this finding remains an underrecognized characteristic of multiple other malignancies-including cholangiocarcinoma, adrenocortical carcinoma, pancreatic neuroendocrine tumor, and primary venous leiomyosarcoma-and can be a feature of benign tumors such as renal angiomyolipoma and uterine leiomyomatosis. Recognition of tumor venous invasion at imaging has clinical significance and management implications for a range of abdominal and pelvic tumors. For example, portal vein invasion is a strong negative prognostic indicator in patients with hepatocellular carcinoma. In patients with rectal cancer, diagnosis of extramural venous invasion helps predict local and distant recurrence and is associated with worse survival. The authors present venous invasion by vascular distribution and organ of primary tumor origin with review of typical imaging features. Common pitfalls and mimics of neoplastic thrombus, including artifacts and anatomic variants, are described to help differentiate these findings from tumor in vein. By accurately diagnosing tumor venous invasion, especially in tumors where its presence may not be a typical feature, radiologists can help referring clinicians develop the best treatment strategies for their patients. ©RSNA, 2020.
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Affiliation(s)
- Jordan D LeGout
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
| | - Ryan E Bailey
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
| | - Candice W Bolan
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
| | - Andrew W Bowman
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
| | - Frank Chen
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
| | - Joseph G Cernigliaro
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
| | - Lauren F Alexander
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
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32
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Mamone G, Di Piazza A, Carollo V, Crinò F, Vella S, Cortis K, Miraglia R. Imaging of primary malignant tumors in non-cirrhotic liver. Diagn Interv Imaging 2020; 101:519-535. [PMID: 32029387 DOI: 10.1016/j.diii.2020.01.010] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/09/2019] [Revised: 01/13/2020] [Accepted: 01/13/2020] [Indexed: 02/07/2023]
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Anysz-Grodzicka A, Podgorska J, Cieszanowski A. State-of-the-art MR Imaging of Uncommon Hepatocellular Tumours: Fibrolamellar Hepatocellular Carcinoma and Combined Hepatocellularcholangiocarcinoma. Curr Med Imaging 2020; 15:269-280. [PMID: 31989878 DOI: 10.2174/1573405614666180927113622] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/25/2017] [Revised: 09/12/2018] [Accepted: 09/14/2018] [Indexed: 12/12/2022]
Abstract
BACKGROUND Fibrolamellar Carcinoma (FLC) and Combined Hepatocellular- Cholangiocarcinoma (CHC) are rare primary liver tumours, which are related to different clinical settings. In both tumours, correlation with clinical data and laboratory tests are extremely important. DISCUSSION Typically, FLC is diagnosed in young patients without any chronic disease and with normal biochemical tests, whereas CHC arises in cirrhotic patients with elevated tumour markers: AFP and/or CA 19-9. The review describes epidemiology, aetiology, pathogenesis, radiological features and treatment of these tumours. Imaging features typical for FLC are: The presence of central scar, calcifications, the large size, heterogeneous and early contrast-enhancement. CONCLUSION The diagnosis of CHC may be suggested in case of elevation of both AFP and CA 19- 9 or inconsistency between elevated tumour markers and imaging findings (i.e., elevated CA 19-9 and radiological features of HCC, or elevated AFP with imaging findings characteristic of ICC).
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Affiliation(s)
- Agnieszka Anysz-Grodzicka
- Department of Radiology, Maria Sklodowska-Curie Memorial Cancer Centre, Institute of Oncology, Warsaw, Poland
| | - Joanna Podgorska
- Department of Clinical Radiology, Medical University of Warsaw, Warsaw, Poland
| | - Andrzej Cieszanowski
- Department of Radiology, Maria Sklodowska-Curie Memorial Cancer Centre, Institute of Oncology, Warsaw, Poland
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Kovac JD, Milovanovic T, Dugalic V, Dumic I. Pearls and pitfalls in magnetic resonance imaging of hepatocellular carcinoma. World J Gastroenterol 2020; 26:2012-2029. [PMID: 32536771 PMCID: PMC7267693 DOI: 10.3748/wjg.v26.i17.2012] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/31/2019] [Revised: 04/20/2020] [Accepted: 04/24/2020] [Indexed: 02/06/2023] Open
Abstract
Hepatocellular carcinoma (HCC) is the most common primary hepatic malignancy, which usually arises in cirrhotic liver. When the typical enhancement pattern, consisting of late arterial hyperenhancement followed by washout, is present in nodules larger than 1 cm, HCC can be confidently diagnosed without the need for tissue biopsy. Nevertheless, HCC can display an atypical enhancement pattern, either as iso or hypovascular lesion, or hypervascular lesion without washout. Not only the enhancement pattern of HCC could be atypical, but also a variety of histological types of HCC, such as steatotic, scirrhous, fibrolamellar, or combined hepatocellular-cholangiocellular carcinoma could raise diagnostic dilemmas. In addition, distinct morphological types of HCC or different growth pattern can occur. Awareness of these atypical and rare HCC presentations on magnetic resonance imaging is important for accurate differentiation from other focal liver lesions and timely diagnosis, which allows optimal treatment of patients.
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Affiliation(s)
- Jelena Djokic Kovac
- Departament of Radiology, Clinical Center Serbia, Belgrade 11000, Serbia
- School of Medicine, Belgrade University, Belgrade 11000, Serbia
| | - Tamara Milovanovic
- Departament of Hepatology, Clinical Center Serbia, Belgrade 11000, Serbia
- School of Medicine, Belgrade University, Belgrade 11000, Serbia
| | - Vladimir Dugalic
- Departament of Surgery, Clinical Center Serbia, Belgrade 11000, Serbia
- School of Medicine, Belgrade University, Belgrade 11000, Serbia
| | - Igor Dumic
- Divison of Hospital Medicine, Mayo Clinic College of Medicine and Sciences, Mayo Clinic Health System, New York, NY 10029, United States
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35
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Cebrián A, Elosua A, González-de la Higuera B, Irisarri R, Ruiz-Clavijo D. Clavicle tumor as an initial manifestation of fibrolamellar hepatocellular carcinoma. REVISTA DE GASTROENTEROLOGÍA DE MÉXICO (ENGLISH EDITION) 2020. [DOI: 10.1016/j.rgmxen.2019.03.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
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36
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Cebrián A, Elosua A, González-de la Higuera B, Irisarri R, Ruiz-Clavijo D. Clavicle tumor as an initial manifestation of fibrolamellar hepatocellular carcinoma. REVISTA DE GASTROENTEROLOGÍA DE MÉXICO 2019; 85:104-106. [PMID: 31677896 DOI: 10.1016/j.rgmx.2019.03.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/10/2018] [Revised: 02/22/2019] [Accepted: 03/07/2019] [Indexed: 11/19/2022]
Affiliation(s)
- A Cebrián
- Aparato Digestivo, Hospital García Orcoyen, Estella, España.
| | - A Elosua
- Aparato Digestivo, Complejo Hospitalario de Navarra, Pamplona, España
| | | | - R Irisarri
- Aparato Digestivo, Hospital García Orcoyen, Estella, España
| | - D Ruiz-Clavijo
- Aparato Digestivo, Hospital García Orcoyen, Estella, España
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37
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Kastenhuber ER, Craig J, Ramsey J, Sullivan KM, Sage J, de Oliveira S, Riehle KJ, Scott JD, Gordan JD, Bardeesy N, Abou-Alfa GK. Road map for fibrolamellar carcinoma: progress and goals of a diversified approach. J Hepatocell Carcinoma 2019; 6:41-48. [PMID: 30951568 PMCID: PMC6362920 DOI: 10.2147/jhc.s194764] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022] Open
Abstract
Fibrolamellar carcinoma is a rare liver cancer, which primarily afflicts adolescents and young adults worldwide and is frequently lethal. Given the rarity of this disease, patient recruitment for clinical trials remains a challenge. In November 2017, the Second Fibrolamellar Cancer Foundation Scientific Summit (Stamford, CT, USA) provided an opportunity for investigators to discuss recent advances in the characterization of the disease and its surrounding liver and immune context. The Fibrolamellar Cancer Foundation has thus set out a road map to identify and test therapeutic targets in the most efficient possible manner.
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Affiliation(s)
- Edward R Kastenhuber
- Cancer Biology and Genetics Program, Memorial Sloan Kettering Cancer Center, New York, NY, USA, .,Louis V. Gerstner Jr. Graduate School of Biomedical Sciences, Memorial Sloan Kettering Cancer Center, New York, NY, USA
| | - John Craig
- Fibrolamellar Cancer Foundation, Greenwich, CT, USA
| | - Jon Ramsey
- Department of Biochemistry, University of Vermont Cancer Center, Burlington, VT, USA
| | - Kevin M Sullivan
- Northwest Liver Research Program, University of Washington, Seattle, WA, USA
| | - Julien Sage
- Department of Pediatrics, Stanford University, Stanford, CA, USA.,Department of Genetics, Stanford University, Stanford, CA, USA
| | - Sofia de Oliveira
- Department of Medical Microbiology and Immunology, University of Wisconsin, Madison, WI, USA
| | - Kimberly J Riehle
- Northwest Liver Research Program, University of Washington, Seattle, WA, USA
| | - John D Scott
- Northwest Liver Research Program, University of Washington, Seattle, WA, USA
| | - John D Gordan
- Department of Medicine, University of California, San Francisco, San Francisco, CA, USA
| | - Nabeel Bardeesy
- Center for Cancer Research, Massachusetts General Hospital, Boston, MA, USA.,Department of Medicine, Harvard Medical School, Boston, MA, USA
| | - Ghassan K Abou-Alfa
- Cancer Biology and Genetics Program, Memorial Sloan Kettering Cancer Center, New York, NY, USA, .,Department of Medicine, Weill Cornell School of Medicine, New York, NY, USA,
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Desai A, Sandhu S, Lai JP, Sandhu DS. Hepatocellular carcinoma in non-cirrhotic liver: A comprehensive review. World J Hepatol 2019; 11:1-18. [PMID: 30705715 PMCID: PMC6354117 DOI: 10.4254/wjh.v11.i1.1] [Citation(s) in RCA: 194] [Impact Index Per Article: 32.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/24/2018] [Revised: 10/04/2018] [Accepted: 12/22/2018] [Indexed: 02/06/2023] Open
Abstract
Hepatocellular carcinoma (HCC) is the most common type of primary liver cancer, which in turns accounts for the sixth most common cancer worldwide. Despite being the 6th most common cancer it is the second leading cause of cancer related deaths. HCC typically arises in the background of cirrhosis, however, about 20% of cases can develop in a non-cirrhotic liver. This particular subgroup of HCC generally presents at an advanced stage as surveillance is not performed in a non-cirrhotic liver. HCC in non-cirrhotic patients is clinically silent in its early stages because of lack of symptoms and surveillance imaging; and higher hepatic reserve in this population. Interestingly, F3 fibrosis in non-alcoholic fatty liver disease, hepatitis B virus and hepatitis C virus infections are associated with high risk of developing HCC. Even though considerable progress has been made in the management of this entity, there is a dire need for implementation of surveillance strategies in the patient population at risk, to decrease the disease burden at presentation and improve the prognosis of these patients. This comprehensive review details the epidemiology, risk factors, clinical features, diagnosis and management of HCC in non-cirrhotic patients and provides future directions for research.
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Affiliation(s)
- Aakash Desai
- Department of Internal Medicine, Case Western Reserve University/MetroHealth Medical Center, Cleveland, OH 44109, United States
| | - Sonia Sandhu
- Department of Hematology and Oncology, Cleveland Clinic/Akron General Medical Center, Akron, OH 44307, United States
| | - Jin-Ping Lai
- Department of Pathology, University of Florida, Gainsville, FL 32611, United States
| | - Dalbir Singh Sandhu
- Division of Gastroenterology and Hepatology, Case Western Reserve University/MetroHealth Medical Center, Cleveland, OH 44109, the United States.
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Tanaka H, Hijioka S, Iwaya H, Mizuno N, Kuwahara T, Okuno N, Ito A, Kuraoka N, Matsumoto S, Obata M, Kurita Y, Yasuda M, Shimizu Y, Kuroda H, Sato Y, Haneda M, Sasaki E, Yatabe Y, Hara K. Fibrolamellar Hepatocellular Carcinoma with Multiple Lung Metastases Treated with Multidisciplinary Therapy. Intern Med 2018; 57:3537-3543. [PMID: 30101933 PMCID: PMC6355421 DOI: 10.2169/internalmedicine.1243-18] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/12/2023] Open
Abstract
A 20-year old man was diagnosed with fibrolamellar hepatocellular carcinoma (FLHCC) with multiple lung metastases, and chemotherapy with FOLFOX was administered. Contrast enhanced CT after 3 cycles of FOLFOX showed no disease progression. We therefore performed surgical resection and radiofrequency ablation of the liver lesions and lung metastases, after obtaining the patient's informed consent. The liver lesions and lung metastases tested positive for DNAJB1-PRKACA. The treatment for FLHCC with extrahepatic metastasis has not been established; however, in a few cases, good long-term prognoses were obtained with multidisciplinary therapy. We herein report a case of FLHCC with multiple lung metastases that was treated with multidisciplinary therapies.
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Affiliation(s)
- Hiroki Tanaka
- Department of Gastroenterology, Aichi Cancer Center Hospital, Japan
- Department of Gastroenterology, Suzuka General Hospital, Japan
| | - Susumu Hijioka
- Department of Gastroenterology, Aichi Cancer Center Hospital, Japan
- Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Japan
| | - Hiromichi Iwaya
- Department of Gastroenterology, Aichi Cancer Center Hospital, Japan
| | - Nobumasa Mizuno
- Department of Gastroenterology, Aichi Cancer Center Hospital, Japan
| | | | - Nozomi Okuno
- Department of Gastroenterology, Aichi Cancer Center Hospital, Japan
| | - Ayako Ito
- Department of Gastroenterology, Aichi Cancer Center Hospital, Japan
| | - Naosuke Kuraoka
- Department of Gastroenterology, Aichi Cancer Center Hospital, Japan
| | | | - Masahiro Obata
- Department of Gastroenterology, Aichi Cancer Center Hospital, Japan
| | - Yusuke Kurita
- Department of Gastroenterology, Aichi Cancer Center Hospital, Japan
| | - Muneji Yasuda
- Department of Gastroenterology, Aichi Cancer Center Hospital, Japan
| | - Yasuhiro Shimizu
- Department of Gastroenterological Surgery, Aichi Cancer Center Hospital, Japan
| | - Hiroaki Kuroda
- Department of Thoracic Surgery, Aichi Cancer Center Hospital, Japan
| | - Yozo Sato
- Department of Diagnostic and Interventional Radiology, Aichi Cancer Center Hospital, Japan
| | - Masataka Haneda
- Department of Pathology and Molecular Diagnostics, Aichi Cancer Center Hospital, Japan
| | - Eiichi Sasaki
- Department of Pathology and Molecular Diagnostics, Aichi Cancer Center Hospital, Japan
| | - Yasushi Yatabe
- Department of Pathology and Molecular Diagnostics, Aichi Cancer Center Hospital, Japan
| | - Kazuo Hara
- Department of Gastroenterology, Aichi Cancer Center Hospital, Japan
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Fibrolamellar Hepatocellular Carcinoma and Noncirrhotic Hyperammonemic Encephalopathy. Case Reports Hepatol 2018; 2018:7521986. [PMID: 30631612 PMCID: PMC6304646 DOI: 10.1155/2018/7521986] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/21/2018] [Accepted: 11/19/2018] [Indexed: 02/06/2023] Open
Abstract
Fibrolamellar hepatocarcinoma is an infrequent liver tumor, currently considered to be a variant different from hepatocarcinoma. The differences lie in genomic alterations, a greater prevalence of fibrolamellar hepatocarcinoma in young patients, and its lack of association with underlying liver disease. The clinical presentation is unspecific, with symptoms ranging from abdominal pain, malaise, and weight loss to atypical manifestation which include hyperammonemic encephalopathy. We present the case of a 33-year-old woman with no prior medical history who presented with a coma and a diagnosis of inoperable fibrolamellar hepatocarcinoma requiring a cadaver donor transplant. While she was on the waiting list, she received hemofiltration and ammonium benzoate treatment, with progressive improvement in her state of consciousness.
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Fibrolamellar hepatocellular carcinoma: multiphasic CT features of the primary tumor on pre-therapy CT and pattern of distant metastases. Abdom Radiol (NY) 2018; 43:3340-3348. [PMID: 29948061 DOI: 10.1007/s00261-018-1657-2] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
OBJECTIVE The aim of our study is to describe the multiphasic CT features of fibrolamellar hepatocellular carcinomas (FLHCCs) and pattern of distant metastases. MATERIALS AND METHODS 33 patients (mean age 28 years: 17M/16F) with pathologically confirmed FLHCC and pre-treatment multiphasic CT were included in the study. Two abdominal radiologists evaluated the multiphasic CT imaging features of these 33 FLHCC patients in consensus. RESULTS Solitary hepatic mass was seen in 67% (22/33). Mean tumor size was 11.3 cm (range 4.6-22 cm). Tumor was well-defined in 64% (21/33). In the pre-contrast CT, 91% (30/33) were hypoattenuating. Central stellate scar was present in 73% (24/33). In FLHCC having central stellate scar, calcification within the central scar was seen in 88% (21/24). In the hepatic arterial phase, 82% (27/33) were hyperattenuating relative to liver. In the portal venous phase, 36% (12/33) were hyperattenuating, 46% (15/33) were isoattenuating, and 18% (6/33) were hypoattenuating. In the delayed phase images, 24% (8/23) were hyperattenuating, 67% (22/33) were isoattenuating, and 9% (3/33) were hypoattenuating. Delayed enhancement of the central stellate scar was seen in 12% (4/33). Distant metastases were seen in 54% (18/33). The most common site of metastases was lungs and was seen in 89% (16/18) of the patients with metastatic disease. CONCLUSION FLHCC typically manifests as a large, solitary mass demonstrating heterogeneous hypervascular enhancement in the arterial phase. Most tend to be isoattenuating in delayed phase. Central stellate scar with internal calcification is a useful imaging feature that can help in the diagnosis of FLHCC. Lungs are the most common site of distant metastases in FLHCC.
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Liver Calcifications and Calcified Liver Masses: Pattern Recognition Approach on CT. AJR Am J Roentgenol 2018; 211:76-86. [DOI: 10.2214/ajr.18.19704] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
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Zarzour JG, Porter KK, Tchelepi H, Robbin ML. Contrast-enhanced ultrasound of benign liver lesions. Abdom Radiol (NY) 2018; 43:848-860. [PMID: 29167944 DOI: 10.1007/s00261-017-1402-2] [Citation(s) in RCA: 28] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Liver lesions are often incidentally detected on ultrasound examination and may be incompletely characterized, requiring further imaging. Contrast-enhanced ultrasound (CEUS) was recently approved by the Food and Drug Administration in the United States for liver lesion characterization. CEUS has the ability to characterize focal liver lesions and has been shown to be superior to color Doppler and power Doppler ultrasound in the detection of tumor vascularity. Differentiating benign from malignant liver lesions is essential to characterizing liver lesions. The CEUS imaging characteristics of benign liver lesions are reviewed, including hepatic cysts, hemangiomas, focal fat, focal nodular hyperplasia, hepatocellular adenomas, abscesses, and traumatic lesions.
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Affiliation(s)
- Jessica G Zarzour
- Department of Radiology, University of Alabama at Birmingham, 619 19th Street South, JTN 338, Birmingham, AL, 35294, USA.
| | - Kristin K Porter
- Department of Radiology, University of Alabama at Birmingham, 619 19th Street South, JTN 338, Birmingham, AL, 35294, USA
| | - Hisham Tchelepi
- Department of Radiology, University of Southern California, Los Angeles, USA
| | - Michelle L Robbin
- Department of Radiology, University of Alabama at Birmingham, 619 19th Street South, JTN 338, Birmingham, AL, 35294, USA
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Durot I, Wilson SR, Willmann JK. Contrast-enhanced ultrasound of malignant liver lesions. Abdom Radiol (NY) 2018; 43:819-847. [PMID: 29094174 DOI: 10.1007/s00261-017-1360-8] [Citation(s) in RCA: 32] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Contrast-enhanced ultrasound (CEUS) is a safe, relatively inexpensive, and widely available imaging technique using dedicated imaging ultrasound sequences and FDA-approved contrast microbubbles that allow detection and characterization of malignant focal liver lesions with high diagnostic accuracy. CEUS provides dynamic real-time imaging with high spatial and temporal capability, allowing for unique contributions to the already established protocols for diagnosing focal liver lesions using CT and MR imaging. In patients with lesions indeterminate on CT and MRI, CEUS is a helpful problem-solving complementary tool that improves patient management. Furthermore, CEUS assists guidance of liver biopsies and local treatment. Variations of CEUS such as DCE-US and ultrasound molecular imaging are emerging for quantitative monitoring of treatment effects and possible earlier detection of cancer. In this review, basic principles of CEUS techniques and ultrasound contrast agents along with a description of the enhancement patterns of malignant liver lesions are summarized. Also, a discussion of the role of CEUS for treatment guidance and monitoring, intraoperative CEUS, and an outlook on emerging applications is provided.
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Palm V, Sheng R, Mayer P, Weiss KH, Springfeld C, Mehrabi A, Longerich T, Berger AK, Kauczor HU, Weber TF. Imaging features of fibrolamellar hepatocellular carcinoma in gadoxetic acid-enhanced MRI. Cancer Imaging 2018; 18:9. [PMID: 29490696 PMCID: PMC5831838 DOI: 10.1186/s40644-018-0143-y] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/19/2017] [Accepted: 02/22/2018] [Indexed: 02/07/2023] Open
Abstract
Background Fibrolamellar hepatocellular carcinoma (FLC) is a rare malignancy occurring in young patients without cirrhosis. Objectives of our study were to analyze contrast material uptake in hepatobiliary phase imaging (HBP) in gadoxetic acid-enhanced liver MRI in patients with FLC and to characterize imaging features in sequence techniques other than HBP. Methods In this retrospective study on histology-proven FLC, contrast material uptake in HBP was quantitatively assessed by calculating the corrected FLC enhancement index (CEI) using mean signal intensities of FLC and lumbar muscle on pre-contrast imaging and HBP, respectively. Moreover, enhancement patterns in dynamic contrast-enhanced MRI and relative signal intensities compared with background liver parenchyma were determined by two radiologists in consensus for HBP, diffusion-weighted imaging using high b-values (DWI), and T2 and T1 weighted pre-contrast imaging. Results In 6 of 13 patients with FLC gadoxetic acid-enhanced liver MRI was available. The CEI suggested presence of HBP contrast material uptake in all FLCs. A mean CEI of 1.35 indicated FLC signal increase of 35% in HBP compared with pre-contrast imaging. All FLCs were hypointense in HBP compared with background liver parenchyma. Three of 6 FLCs had arterial hyperenhancement and venous wash-out. In DWI and T2 weighted imaging, 5 of 6 FLCs were hyperintense. In T1 weighted imaging, 5 of 6 FLCs were hypointense. Conclusion Hepatobiliary uptake of gadoxetic acid was quantitatively measurable in all FLCs investigated in our study. The observation of hypointensity of FLCs in HBP compared with background liver parenchyma emphasizes the role of gadoxetic acid-enhanced liver MRI for non-invasive diagnosis of FLC and its importance in the diagnostic work-up of indeterminate liver lesions.
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Affiliation(s)
- Viktoria Palm
- Department of Diagnostic and Interventional Radiology, Heidelberg University Hospital, INF 110, 69120, Heidelberg, Germany
| | - Ruofan Sheng
- Department of Radiology, Zhongshan Hospital, Fudan University, 180 Fenglin Road, Shanghai, 200032, China
| | - Philipp Mayer
- Department of Diagnostic and Interventional Radiology, Heidelberg University Hospital, INF 110, 69120, Heidelberg, Germany.,Liver Cancer Center Heidelberg, Heidelberg University Hospital, INF 224, 69120, Heidelberg, Germany
| | - Karl-Heinz Weiss
- Liver Cancer Center Heidelberg, Heidelberg University Hospital, INF 224, 69120, Heidelberg, Germany.,Department of Gastroenterology, Infectious Diseases, Intoxication, Heidelberg University Hospital, INF 410, 69120, Heidelberg, Germany
| | - Christoph Springfeld
- Liver Cancer Center Heidelberg, Heidelberg University Hospital, INF 224, 69120, Heidelberg, Germany.,Department of Medical Oncology, National Center for Tumor Diseases (NCT), Heidelberg University Hospital, INF 460, 69120, Heidelberg, Germany
| | - Arianeb Mehrabi
- Liver Cancer Center Heidelberg, Heidelberg University Hospital, INF 224, 69120, Heidelberg, Germany.,Department of General, Visceral and Transplantation Surgery, Heidelberg University Hospital, INF 110, 69120, Heidelberg, Germany
| | - Thomas Longerich
- Liver Cancer Center Heidelberg, Heidelberg University Hospital, INF 224, 69120, Heidelberg, Germany.,Division Translational Gastrointestinal Pathology, Institute of Pathology, Heidelberg University Hospital, INF 224, 69120, Heidelberg, Germany
| | - Anne Katrin Berger
- Department of Medical Oncology, National Center for Tumor Diseases (NCT), Heidelberg University Hospital, INF 460, 69120, Heidelberg, Germany
| | - Hans-Ulrich Kauczor
- Department of Diagnostic and Interventional Radiology, Heidelberg University Hospital, INF 110, 69120, Heidelberg, Germany.,Liver Cancer Center Heidelberg, Heidelberg University Hospital, INF 224, 69120, Heidelberg, Germany
| | - Tim Frederik Weber
- Department of Diagnostic and Interventional Radiology, Heidelberg University Hospital, INF 110, 69120, Heidelberg, Germany. .,Liver Cancer Center Heidelberg, Heidelberg University Hospital, INF 224, 69120, Heidelberg, Germany.
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Semelka RC, Nimojan N, Chandana S, Ramalho M, Palmer SL, DeMulder D, Parada Villavicencio C, Woosley J, Garon BL, Jha RC, Miller FH, Altun E. MRI features of primary rare malignancies of the liver: A report from four university centres. Eur Radiol 2017; 28:1529-1539. [PMID: 29079914 DOI: 10.1007/s00330-017-5102-7] [Citation(s) in RCA: 24] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/22/2017] [Revised: 09/12/2017] [Accepted: 09/27/2017] [Indexed: 12/17/2022]
Abstract
PURPOSE To determine if rare primary malignancies of the liver may have consistent features on magnetic resonance imaging (MRI). MATERIALS AND METHODS This IRB-compliant retrospective study reviewed the records from the pathology departments of four university centres over an 11-year period from 2005-2016 to identify rare primary malignant tumours, which were cross-referenced with MRI records. MRI studies of these patients were reviewed to determine if these tumours exhibited consistent and distinctive features. RESULTS Sixty patients were identified with rare primary liver tumours. The following distinctive features and frequency of occurrence were observed: mixed hepatocellular carcinoma-cholangiocarcinoma showed regions of wash-out in 7/19 of patients; 6/6 of fibrolamellar carcinomas demonstrated large heterogeneous lesions with large heterogeneous central scars; epithelioid haemangioendothelioma larger than 2 cm showed target-like enhancement in late-phase enhancement in 9/13; sarcomas excluding angiosarcoma had central necrosis in 3/9 and haemorrhage in 5/9; angiosarcomas showed centripedal progressive nodular enhancement in 3/6 and showed regions of haemorrhage in 3/6; and 7/7 of primary hepatic lymphomas showed encasement of vessels. CONCLUSION Although helpful features for the differentiation of rare primary malignancies of the liver are identified, no MRI features appear to be specific and therefore histopathological confirmation is usually required for definitive diagnosis. KEY POINTS • No MRI features appear to be specific for rare primary liver malignancies. • Haemorrhage is a helpful sign in diagnosis of primary hepatic sarcomas. • Angiosarcomas may show progressive nodular enhancement towards the centre mimicking haemangioma. • Vessel encasement is a helpful sign in diagnosis of primary hepatic lymphoma.
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Affiliation(s)
- Richard C Semelka
- Department of Radiology, University of North Carolina at Chapel Hill, 101 Manning Drive, Chapel Hill, NC, 27514, USA
| | - Nadesan Nimojan
- Department of Radiology, University of North Carolina at Chapel Hill, 101 Manning Drive, Chapel Hill, NC, 27514, USA
| | - Saman Chandana
- Department of Radiology, University of North Carolina at Chapel Hill, 101 Manning Drive, Chapel Hill, NC, 27514, USA
| | - Miguel Ramalho
- Department of Radiology, Hospital Garcia de Orta, EPE, Almada, Portugal
| | - Suzanne L Palmer
- Department of Radiology, University of South California, Los Angeles, CA, USA
| | | | | | - John Woosley
- Department of Pathology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA
| | - Bonnie L Garon
- Department of Radiology, University of South California, Los Angeles, CA, USA
| | - Reena C Jha
- Department of Radiology, Georgetown University, Washington, DC, USA
| | - Frank H Miller
- Department of Radiology, Northwestern University, Chicago, IL, USA
| | - Ersan Altun
- Department of Radiology, University of North Carolina at Chapel Hill, 101 Manning Drive, Chapel Hill, NC, 27514, USA.
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Sheybani A, Gaba RC, Lokken RP, Berggruen SM, Mar WA. Liver Masses: What Physicians Need to Know About Ordering and Interpreting Liver Imaging. Curr Gastroenterol Rep 2017; 19:58. [PMID: 29044439 DOI: 10.1007/s11894-017-0596-3] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
PURPOSE OF REVIEW This paper reviews diagnostic imaging techniques used to characterize liver masses and the imaging characteristics of the most common liver masses. RECENT FINDINGS The role of recently adopted ultrasound and magnetic resonance imaging contrast agents will be emphasized. Contrast-enhanced ultrasound is an inexpensive exam which can confirm benignity of certain liver masses without ionizing radiation. Magnetic resonance imaging using hepatocyte-specific gadolinium-based contrast agents can help confirm or narrow the differential diagnosis of liver masses.
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Affiliation(s)
- Arman Sheybani
- Department of Radiology, University of Illinois at Chicago, 1740 W Taylor St Rm 2483, MC 931, Chicago, IL, 60612, USA
| | - Ron C Gaba
- Department of Radiology, University of Illinois at Chicago, 1740 W Taylor St Rm 2483, MC 931, Chicago, IL, 60612, USA
| | - R Peter Lokken
- Department of Radiology, University of Illinois at Chicago, 1740 W Taylor St Rm 2483, MC 931, Chicago, IL, 60612, USA
| | - Senta M Berggruen
- Department of Radiology, Northwestern University, NMH/Arkes Family Pavilion Suite 800, 676 N Saint Clair, Chicago, IL, 60611, USA
| | - Winnie A Mar
- Department of Radiology, University of Illinois at Chicago, 1740 W Taylor St Rm 2483, MC 931, Chicago, IL, 60612, USA.
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Alexander LF, Harri P, Little B, Moreno CC, Mittal PK. Magnetic Resonance Imaging of Primary Hepatic Malignancies in Patients With and Without Chronic Liver Disease: A Pictorial Review. Cureus 2017; 9:e1539. [PMID: 28989828 PMCID: PMC5628780 DOI: 10.7759/cureus.1539] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022] Open
Abstract
Primary hepatic malignancies are less common than metastatic diseases, but a recognition of these lesions is important for diagnosis and treatment planning. Magnetic resonance imaging (MRI) provides the most imaging information to diagnose lesions noninvasively and to narrow differential diagnoses. This paper reviews the imaging findings of chronic liver disease and primary hepatic malignancies, including hepatocellular carcinoma (HCC), intrahepatic cholangiocarcinoma (CCA), epithelioid hemangioendothelioma, hepatic angiosarcoma, and primary hepatic lymphoma. Clinical and MRI features are reviewed to improve the readers’ recognition of these tumors, allowing for a narrower differential diagnosis when liver masses are encountered on abdominal imaging.
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Affiliation(s)
- Lauren F Alexander
- Department of Radiology and Imaging Sciences, Emory University School of Medicine
| | - Peter Harri
- Department of Radiology and Imaging Sciences, Emory University School of Medicine
| | - Brent Little
- Department of Radiology and Imaging Sciences, Emory University School of Medicine
| | - Courtney C Moreno
- Department of Radiology and Imaging Sciences, Emory University School of Medicine
| | - Pardeep K Mittal
- Department of Radiology and Imaging Sciences, Emory University School of Medicine
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Espinosa JA, Merlo A, Arafeh MO, Munene G. An unusual case of jaundice: Biliary tumor thrombus in fibrolamellar hepatocellular carcinoma. Int J Surg Case Rep 2017; 36:50-54. [PMID: 28531870 PMCID: PMC5440287 DOI: 10.1016/j.ijscr.2017.03.039] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/13/2017] [Revised: 03/28/2017] [Accepted: 03/29/2017] [Indexed: 11/21/2022] Open
Abstract
Fibrolamellar hepatocellular carcinoma is a rare form of hepatocellullar cancer seen in younger patients without underlying liver disease. Liver resection with lymph node dissection is the mainstay of treatment. Bile duct tumor thrombus that has been reported in hepatocellular cancer has to date never been described in fibrolamellar hepatocellular cancer and must be a consideration in patients who present with obstructive jaundice. Background Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare and unique variant of hepatocellular carcinoma (HCC) whose presentation remains inadequately described. We present a resectable case of FL-HCC which involved tumor thrombus of the common bile duct. Presentation A 27 year-old male presenting with jaundice, abdominal pain, vomiting, hepatic dysfunction and hyperbilirubinemia was found to have a large liver mass and lymphadenopathy on preoperative imaging. A right hepatectomy with perihepatic lymph node dissection and cholecystectomy was performed. Intraoperative cholangiogram demonstrated common bile duct (CBD) obstruction. CBD exploration revealed biliary tumor thrombus relieved with biliary thrombectomy. Discussion FL-HCC can initially present with invading obstructing biliary tumor thrombus of the CBD causing jaundice. Conclusion Preoperative surgical approach should consider CBD exploration on an individual basis for underlying obstructive biliary tumor thrombus.
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Affiliation(s)
- Jairo A Espinosa
- Department of Surgery, Western Michigan University Homer Stryker School of Medicine/West Michigan Cancer Center, Kalamazoo, MI, United States
| | - Alex Merlo
- Department of Surgery, Western Michigan University Homer Stryker School of Medicine/West Michigan Cancer Center, Kalamazoo, MI, United States
| | - Mohamed-Omar Arafeh
- Department of Surgery, Western Michigan University Homer Stryker School of Medicine/West Michigan Cancer Center, Kalamazoo, MI, United States
| | - Gitonga Munene
- Department of Surgery, Western Michigan University Homer Stryker School of Medicine/West Michigan Cancer Center, Kalamazoo, MI, United States.
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