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Cancer 2022 May 13. Epub 2022 May 13.

Division of Pediatric Surgery, Children's Hospital, London Health Sciences Center, London, Ontario, Canada.

Background: Hepatocellular carcinoma (HCC) is a rare cancer in children, with various histologic subtypes and a paucity of data to guide clinical management and predict prognosis.

Methods: A multi-institutional review of children with hepatocellular neoplasms was performed, including demographic, staging, treatment, and outcomes data. Patients were categorized as having conventional HCC (cHCC) with or without underlying liver disease, fibrolamellar carcinoma (FLC), and hepatoblastoma with HCC features (HB-HCC). Read More

AJR Am J Roentgenol 2022 May 11. Epub 2022 May 11.

Department of Radiology, Emory University and Children's Healthcare of Atlanta, 1405 Clifton Rd., Atlanta, GA 30322.

Pediatric hepatocellular carcinoma (HCC) is an aggressive malignancy for which imaging findings remain poorly described. In comparison with adult HCC, pediatric HCC more commonly occurs without underlying risk factors, and standardized surveillance guidelines for those with predispositions are lacking. To evaluate imaging findings of non-fibrolamellar pediatric HCC and to identify associations between these imaging findings and presence of predisposing factors. Read More

Oncol Res Treat 2022 May 10. Epub 2022 May 10.

Introduction: Fibrolamellar carcinoma (FLC) is a rare liver cancer that predominantly affects younger patients without a history of liver disease. Surgical resection is the cornerstone of therapy and represents the best potentially curative treatment option. Modest objective responses with cytotoxic chemotherapy alone or combined with immune checkpoint inhibitors (ICIs) have been reported, however there are no established systemic therapy regimens for unresectable or metastatic FLC. Read More

JCI Insight 2022 Apr 28. Epub 2022 Apr 28.

Department of Biomedical Sciences, Cornell University, Ithaca, United States of America.

Background: Fibrolamellar carcinoma (FLC) is an aggressive liver cancer primarily afflicting adolescents and young adults. Patients with FLC harbor a heterozygous deletion on chromosome 19 that leads to the oncogenic gene fusion, DNAJB1-PRKACA. There are currently no effective therapeutics for FLC. Read More

Front Oncol 2022 8;12:864106. Epub 2022 Apr 8.

Hepatobiliary Center, The First Affiliated Hospital of Nanjing Medical University, Nanjing, China.

Background: Hepatocellular carcinoma (HCC) is a highly heterogeneous tumor with several rare pathological subtypes and which is still poorly understood. This study aimed to describe the epidemiological and clinical spectrum of five rare HCC subtypes and develop a competing risk nomogram for cancer-specific survival prediction.

Methods: The study cohort was recruited from the Surveillance, Epidemiology, and End Results database. Read More

Int J Surg Case Rep 2022 Mar 29;94:106980. Epub 2022 Mar 29.

Surgical Gastroenterology Unit, Division of General Surgery, University of Cape Town Health Sciences Faculty and Groote Schuur Hospital, Cape Town, South Africa.

Introduction: Fibrolamellar carcinoma (FLC) is a rare pathologically distinct primary liver cancer. Surgical resection is the only treatment associated with prolonged survival. Trans-arterial embolization (TAE), which is a recognised treatment for hepatocellular carcinoma has been used to treat FLC. Read More

Sci Rep 2022 Apr 12;12(1):6116. Epub 2022 Apr 12.

Department of Energy Engineering and Physics, Amirkabir University of Technology, 15875-4413, Tehran, Iran.

Laser-induced fluorescence (LIF) spectroscopy has recently gained regards for diagnosis of the cancer in various tissues of the human body. This method in its conventional form, when used for assay of highly scattering media, encounters a lot of noise due to multiple scattering and inner filter effects which overshadows the sensitivity and specificity of the method. Here, angular dependence of the LIF spectral shift due to the reabsorption events have been investigated for characterization of the bio-tissues. Read More

Cancer Diagn Progn 2021 Mar-Apr;1(1):23-28. Epub 2021 Mar 3.

Department of Internal Medicine II, Fundeni Clinical Institute,Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.

Background/aim: Fibrolamellar carcinoma is a rare primary hepatic malignancy that has recently been recognized as a distinct clinical entity, highly different from the well-known hepatocellular carcinoma. This report describes the clinical and paraclinical aspects of the fibrolamellar carcinoma, emphasizing its particularities.

Case Report: A 30-year-old patient presented to the hospital with nonspecific symptoms and weight loss, with imaging findings showing abdominal and mediastinal masses. Read More

J Surg Res 2022 Apr 7;276:261-271. Epub 2022 Apr 7.

William Beaumont Army Medical Center, Department of Surgery, Fort Bliss, Texas. Electronic address:

Introduction: Hepatocellular carcinoma (HCC) is rare among adolescent and young adult (AYA) patients, and resection or transplant remains the only curative therapy. The role of lymph node (LN) sampling is not well-defined. The aim of this study was to describe practice patterns, as well as investigate the impact of LN sampling on survival outcomes in this population. Read More

Curr Probl Cancer 2022 Mar 2;46(3):100847. Epub 2022 Mar 2.

Department of Medical Intensive Care, Hamad Medical corporation, Doha, Qatar.

Fibrolamellar hepatocellular carcinoma is a rare type of hepatocellular carcinoma with unclear etiology. Its prevalence ranges from 0.6%-5%. Read More

Pediatrics 2022 04;149(4)

Divisions of Pediatric Endocrinology.

Immune checkpoint inhibitor (ICI) therapies are now first-line therapy for many advanced malignancies in adults, with emerging use in children. With increasing ICI use, prompt recognition and optimal management of ICI-associated immune-related adverse events (IRAEs) are critical. Nearly 60% of ICI-treated adults develop IRAEs, which commonly manifest as autoimmune skin, gastrointestinal, and endocrine disease and can be life-threatening. Read More

Cancers (Basel) 2022 Mar 2;14(5). Epub 2022 Mar 2.

Department of Surgery, Division of Hepatobiliary Surgery and Liver Transplantation, Vanderbilt University Medical Center, Nashville, TN 37232, USA.

Liver transplantation (LT) is the only potentially curative option for children with unresectable hepatocellular carcinoma (HCC). We performed a systematic review of the MEDLINE, Scopus, Cochrane Library, and Web of Science databases (end-of-search date: 31 July 2020). Our outcomes were overall survival (OS) and disease-free survival (DFS). Read More

Eur Radiol 2022 Mar 8. Epub 2022 Mar 8.

Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.

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. Read More

JHEP Rep 2022 Apr 13;4(4):100416. Epub 2021 Dec 13.

Liver Tumor Program, Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.

Hepatocellular carcinoma (HCC) is the predominant primary cancer arising from the liver and is one of the major causes of cancer-related mortality worldwide. The cellular origin of HCC has been a topic of great interest due to conflicting findings regarding whether it originates in hepatocytes, biliary cells, or facultative stem cells. These cell types all undergo changes during liver injury, and there is controversy about their contribution to regenerative responses in the liver. Read More

Genes Chromosomes Cancer 2022 05;61(5):285-297

Division of Laboratory of Genetics and Genomics, Mayo Clinic, Rochester, Minnesota, USA.

Fusion genes have been identified in a wide array of human neoplasms including hematologic and solid tumors, including gastrointestinal tract neoplasia. A fusion gene is the product of parts of two genes that are joined together following a deletion, translocation, or chromosomal inversion. Together with single nucleotide variants, insertions, deletions, and amplification, fusion genes represent one of the key genomic mechanisms for tumor development. Read More

Nat Rev Gastroenterol Hepatol 2022 May 21;19(5):328-342. Epub 2022 Feb 21.

Department of Biomedical Sciences, Cornell University, Ithaca, NY, USA.

Fibrolamellar carcinoma (FLC), a rare, lethal hepatic cancer, occurs primarily in adolescents and young adults. Unlike hepatocellular carcinoma, FLC has no known association with viral, metabolic or chemical agents that cause cirrhosis. Currently, surgical resection is the only treatment demonstrated to achieve cure, and no standard of care exists for systemic therapy. Read More

AJR Am J Roentgenol 2022 Feb 16:1-12. Epub 2022 Feb 16.

Department of Diagnostic Radiology, Tan Tock Seng Hospital, 11 Jalan Tan Tock Seng, Singapore 308433, Singapore.

Please see the Editorial Comment by Antonio Luna discussing this article. 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. Read More

PLoS One 2022 15;17(2):e0263829. Epub 2022 Feb 15.

Department of Surgery, Boston Children's Hospital, Boston, MA, United States of America.

Fibrolamellar carcinoma (FLC) is a primary liver cancer that most commonly arises in adolescents and young adults in a background of normal liver tissue and has a poor prognosis due to lack of effective chemotherapeutic agents. The DNAJB1-PRKACA gene fusion (DP) has been reported in the majority of FLC tumors; however, its oncogenic mechanisms remain unclear. Given the paucity of cellular models, in particular FLC tumor cell lines, we hypothesized that engineering the DP fusion gene in HEK293T cells would provide insight into the cellular effects of the fusion gene. Read More

Front Pharmacol 2021 20;12:747895. Epub 2022 Jan 20.

Laboratory of Translational Oncology and Experimental Cancer Therapeutics, The Warren Alpert Medical School, Brown University, Providence, RI, United States.

Chromosomal fusions encoding novel molecular drivers have been identified in several solid tumors, and in recent years the identification of such pathogenetic events in tumor specimens has become clinically actionable. Pediatric sarcomas and other rare tumors that occur in children as well as adults are a group of heterogeneous tumors often with driver gene fusions for which some therapeutics have already been developed and approved, and others where there is opportunity for progress and innovation to impact on patient outcomes. We review the chromosomal rearrangements that represent oncogenic events in pediatric solid tumors outside of the central nervous system (CNS), such as Ewing Sarcoma, Rhabdomyosarcoma, Fibrolamellar Hepatocellular Carcinoma, and Renal Cell Carcinoma, among others. Read More

Diagnostics (Basel) 2021 Dec 27;12(1). Epub 2021 Dec 27.

Nuclear Medicine Department, Virgen de las Nieves Hospital, 18014 Granada, Spain.

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. Read More

Int J Gen Med 2022 8;15:393-406. Epub 2022 Jan 8.

Department of General, Visceral and Transplant Surgery, Heidelberg University Clinic, Heidelberg, Germany.

Background: The study aimed to develop a nomogram to predict overall survival (OS) for patients with FLC using a national database.

Methods: The Surveillance, Epidemiology, and End Results database of the National Cancer Institute was reviewed to identify FLC cases with histological confirmation between 2004 and 2014. Cox proportional hazards models were used to identify factors associated with OS. Read More

Pediatr Transplant 2022 05 15;26(3):e14209. Epub 2021 Dec 15.

Department of Pediatrics, Columbia University Irving Medical Center, New York, New York, USA.

Background: Immunotherapy, specifically immune checkpoint inhibitors (ICIs), including anti-programmed cell death 1 (anti-PD1), has recently received clinical approval for the treatment of adult hepatocellular carcinoma (HCC). However, the safety and efficacy of ICIs prior to solid organ transplant are unknown, especially in pediatrics. Safety reports are variable in adults, with some series describing subsequent allograft rejection and loss while others report successful transplants without allograft rejection. Read More

J Gastrointest Cancer 2021 Nov 5. Epub 2021 Nov 5.

Department of Surgery and Liver Transplant Institute, Inonu University Faculty of Medicine, Elazig Yolu 10. Km, 44280, Malatya, Turkey.

Course of blood procalcitonin levels by days. Read More

J Hepatocell Carcinoma 2021 22;8:1269-1279. Epub 2021 Oct 22.

Department of General, Visceral, and Transplantation Surgery, Heidelberg University Hospital, Heidelberg, Germany.

Background: Aberrant subtypes of hepatocellular carcinoma (HCC) account for 20-30% of all HCCs and habitually present a challenge in diagnosis and treatment. Scirrhous hepatocellular carcinoma (s-HCC) is often misdiagnosed as cholangiocarcinoma, fibrolamellar hepatocellular carcinoma, or metastasis.

Methods: Electronic databases (PubMed, Web of Knowledge, Google Scholar, Cochrane Library, and WHO International Clinical Trials Registry Platform) were searched for publications on scirrhous hepatocellular carcinoma without date or language restrictions. Read More

Am J Clin Pathol 2022 Feb;157(2):305-313

Department of Pathology, New Haven, CT, USA.

Objectives: The pathologic differences between hepatocellular carcinoma (HCC) arising in noncirrhotic and cirrhotic livers have not been well studied.

Methods: We performed a retrospective analysis of 378 HCC cases (95 in noncirrhotic, 283 in cirrhotic livers) from pathology archives (2010-2017).

Results: Patients without cirrhosis were more likely to have hepatitis B (13. Read More

Surg Case Rep 2021 Sep 17;7(1):208. Epub 2021 Sep 17.

Department of Hepatobiliary-Pancreatic Surgery, Juntendo University School of Medicine, Juntendo University Hospital, 3-1-1 Hongo, Bunkyo-Ku, Tokyo, 113-0033, Japan.

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. Read More

World J Clin Cases 2021 Aug;9(23):6734-6746

Department of Surgery, John A Burns School of Medicine, University of Hawaii at Manoa, Honolulu, HI 96817, United States.

Background: The incidence and mortality rates of hepatocellular carcinoma (HCC) are increasing in the United States. However, the increases in different racial and socioeconomic groups have not been homogeneous. Access to healthcare based on socioeconomic status and cost of living index (COLI), especially in HCC management, is under characterized. Read More

J Proteome Res 2021 10 26;20(10):4815-4830. Epub 2021 Aug 26.

Department of Biochemistry, Molecular Biology, and Biophysics, University of Minnesota, Minneapolis, Minnesota 55455, United States.

The fusion is the signature genetic event of fibrolamellar hepatocellular carcinoma (FL-HCC), a rare but lethal liver cancer that primarily affects adolescents and young adults. A deletion fuses the first exon of the HSP40 gene (), with exons 2-10 of protein kinase A (), producing the chimeric kinase DNAJB1-PKA (J-PKA). The HSP40 portion's scaffolding/chaperone function has been implicated in redirecting substrate recognition to upregulate oncogenic pathways, but the direct substrates of this fusion are not fully known. Read More

Cureus 2021 Jul 19;13(7):e16486. Epub 2021 Jul 19.

Hematology and Oncology, State University of New York Upstate Medical University, Syracuse, USA.

Fibrolamellar variant of hepatocellular carcinoma (FLHCC) is a rare malignancy. Our patient presented to the hospital with increasing weight loss, decreased appetite, and night sweats. Imaging revealed a large liver mass and multiple pulmonary nodules concerning for metastasis. Read More

Eur J Med Genet 2021 Nov 18;64(11):104313. Epub 2021 Aug 18.

BCLC group, Liver Unit, Hospital Clinic Barcelona, IDIBAPS, CIBERehd, University of Barcelona, Barcelona, Spain.

Hepatocellular carcinoma (HCC) constitutes 80% of all primary liver cancers. Based on key developments in the understanding of its carcinogenesis and the advancement of treatment options, detailed algorithms and practice guidelines have been published to guide the clinical management of HCC. Furthermore, several subclasses of HCC have been described based on molecular profiles and linked to pathological characteristics, clinical features, and disease aggressiveness. Read More