scholarly journals Fibrolamellar Carcinoma: A Multimodal Approach

2020 ◽  
Vol 27 (6) ◽  
pp. 429-433
Author(s):  
João Vasco Barreira ◽  
Nádia Silva ◽  
Anuraj Parmanande ◽  
Manuel Rocha ◽  
João S. Coelho ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Viral Hepatitis ◽  
Cancer Patients ◽  
Clinical Presentation ◽  
Multidisciplinary Teams ◽  
Multimodal Approach ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Serum Alpha Fetoprotein ◽  
Fibrolamellar Carcinoma ◽  
Well Differentiated

Fibrolamellar carcinoma is a rare variant of hepatocellular carcinoma not associated with cirrhosis or viral hepatitis. Serum alpha-fetoprotein levels are usually normal; the histology is of a well-differentiated tumor, and the staging is the same as for hepatocellular carcinoma. We describe the case of a female patient in her 4th decade of life with a diagnosis of fibrolamellar hepatocellular carcinoma with a multimodal approach. The rare incidence of this cancer and its unusual clinical presentation justifies reporting this case and highlights the importance of multidisciplinary teams in the treatment of cancer patients.

scholarly journals Fibrolamellar Hepatocellular Carcinoma and Noncirrhotic Hyperammonemic Encephalopathy

2018 ◽  
Vol 2018 ◽  
pp. 1-6 ◽  
Author(s):  
Oscar Suarez ◽  
María Perez ◽  
Martin Garzon ◽  
Rodrigo Daza ◽  
Geovanny Hernandez ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Weight Loss ◽  
Clinical Presentation ◽  
Young Patients ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Underlying Liver Disease ◽  
Hyperammonemic Encephalopathy ◽  
Cadaver Donor ◽  
Atypical Manifestation ◽  
Donor Transplant

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.

scholarly journals A Misdiagnosis of a Breast Tumor: Histology Solves It

2020 ◽  
Author(s):  
Nádia Rodrigues Silva ◽  
João Vasco Barreira ◽  
Susana Nunes ◽  
Inês Morujão ◽  
Ana Correia
Keyword(s):  
Gastric Cancer ◽  
Cancer Patients ◽  
Breast Tumor ◽  
Clinical Presentation ◽  
Stage Iv ◽  
Multidisciplinary Teams ◽  
Breast Lump ◽  
Stage Iv Gastric Cancer ◽  
Tumor Histology ◽  
Primary Neoplasm

A breast lump can be benign or malignant, the late one can be primary or metastatic. When the primary neoplasm is unknown the diagnosis, management and treatment maybe quite difficult. We present a case of stage IV gastric cancer, where the first complaint was a breast lump. The rare incidence of such clinical presentation justifies reporting this case and highlights the importance of multidisciplinary teams in the management of cancer patients.

scholarly journals Fibrolamellar Carcinoma: Recent Advances and Unresolved Questions on the Molecular Mechanisms

2018 ◽  
Vol 38 (01) ◽  
pp. 051-059 ◽  
Author(s):  
Gadi Lalazar ◽  
Sanford Simon
Keyword(s):  
Hepatocellular Carcinoma ◽  
Overall Survival ◽  
Molecular Mechanisms ◽  
Current Knowledge ◽  
Primary Liver Cancer ◽  
Rare Form ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Underlying Liver Disease ◽  
Recent Advances ◽  
Fibrolamellar Carcinoma

AbstractFibrolamellar hepatocellular carcinoma (FLC) is a rare form of primary liver cancer that affects adolescents and young adults without underlying liver disease. Surgery remains the mainstay of therapy; however, most patients are either not surgical candidates or suffer from recurrence. There is no approved systemic therapy and the overall survival remains poor. Historically classified as a subtype of hepatocellular carcinoma (HCC), FLC has a unique clinical, histological, and molecular presentation. At the genomic level, FLC contains a single 400kB deletion in chromosome 19, leading to a functional DNAJB1-PRKACA fusion protein. In this review, we detail the recent advances in our understanding of the molecular underpinnings of FLC and outline the current knowledge gaps.

Increased levels of typically fetal bile acid species in patients with hepatocellular carcinoma

2001 ◽  
Vol 100 (5) ◽  
pp. 499-508 ◽  
Author(s):  
Mohamad Y. EL-MIR ◽  
Maria D. BADIA ◽  
Nazaret LUENGO ◽  
Maria J. MONTE ◽  
Jose J. G. MARIN
Keyword(s):  
Hepatocellular Carcinoma ◽  
Liver Cirrhosis ◽  
Liver Metastasis ◽  
Viral Hepatitis ◽  
Chronic Viral Hepatitis ◽  
Control Group ◽  
Serum Alpha Fetoprotein ◽  
Liver Neoplasia ◽  
Urine And Serum ◽  
Serum And Urine

The aim of this work was to investigate the reappearance during liver neoplasia of bile acids (BAs) species, which are unusual in healthy adults, but common in fetuses. Serum and urine samples were collected from patients with hepatocellular carcinoma (HCC; n = 27), and for comparative purposes, with liver cirrhosis (n = 49), liver metastasis (n = 19), chronic viral hepatitis (n = 11) and healthy volunteer (control group; n = 26) groups. BAs were identified and measured by GC–MS. Hypercholanaemia was found in all groups of patients. In HCC, this was characterized by a marked increase in the chenodeoxycholate/cholate ratio in both serum and urine. Although increased levels of BAs, with hydroxylations at unusual positions, and oxo-BAs were found in HCC, these were not significantly different from those observed in other groups. However, BAs with a flat structure, i.e. Δ4-unsaturated- and 5α- or allo-BAs, which were almost absent in healthy subjects, were markedly increased in the serum and urine of HCC patients. They were also detected, although in much lower amounts, in liver metastasis and liver cirrhosis, but not in viral hepatitis. Flat-BAs were better detected in urine than in serum. Urinary Δ4-unsaturated-BA output was significantly lower in patients with small tumours (< 3 cm) compared with those with higher size tumours. No correlation between flat-BA output into urine and serum alpha-fetoprotein or total BAs was found. These results suggest that Δ4- and/or allo-BAs are particularly elevated in patients with HCC, which may be a potentially useful complementary, rather than alternative, marker for early detection of liver neoplasia.

scholarly journals Molecular Basis of Hyperammonemic Encephalopathy in Fibrolamellar Hepatocellular Carcinoma

2021 ◽  
Author(s):  
Rodrigo Cañada Trofo Surjan ◽  
Thais Martins de Lima ◽  
Elizabeth Santana dos Santos ◽  
Sergio do Prado Silveira ◽  
Marcel Cerqueira Cesar Machado ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Ornithine Decarboxylase ◽  
Aurora Kinase ◽  
Chromosome 19 ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Adequate Treatment ◽  
Aurora Kinase A ◽  
Hyperammonemic Encephalopathy ◽  
Fibrolamellar Carcinoma ◽  
Kinase A

Abstract Purpose hyperammonemic encephalopathy is a potentially fatal condition associated with fibrolamellar hepatocellular carcinoma. The mechanism involved in hyperammonemia in patients with fibrolamellar carcinoma was unclear until a possible physiopathological pathway was recently proposed. An ornithine transcarboxylase dysfunction was suggested as a result of increased ornithine decarboxylase activity induced by c-Myc overexpression. This c-Myc overexpression resulted from Aurora Kinase A overexpression derived from the activity of a chimeric kinase that is the final transcript of a deletion in chromosome 19, common to all fibrolamellar carcinomas. Methods we performed the analysis of the expression of all enzymes involved and tested for the mutation in chromosome 19 in fresh frozen samples of fibrolamellar hepatocellular carcinoma, non-tumor liver and hepatic adenomatosis. Results specific DNAJB-PRKACA fusion protein that results from the recurrent mutation on chromosome 19 common to all fibrolamellar carcinoma was detected only in the fibrolamellar carcinoma sample. Fibrolamellar carcinoma and adenomiomatosis samples presented increased expression of Aurora Kinase A, c-MYC and ornithine decarboxylase when compared to normal liver, while ornithine transcarbamylase was decreased. Conclusion The proposed physiopathological pathway is correct and that overexpression of c-Myc may also be responsible of hyperammonemia in patients with other types of rapidly growing hepatomas. This gives further evidence to apply new and adequate treatment to this severe complication.

scholarly journals Fibrolamellar hepatocellular carcinoma: Case report

2020 ◽  
Author(s):  
Ivan I. Shtefanov ◽  
Darkhan N. Akhmedin ◽  
Assiya M. Kukanova ◽  
Ainagul A. Zhakipova ◽  
Abai K. Makishev
Keyword(s):  
Hepatocellular Carcinoma ◽  
Case Report ◽  
Surgical Treatment ◽  
Surgical Intervention ◽  
Tumour Size ◽  
Staging System ◽  
Malignant Tumour ◽  
Cancer Staging ◽  
Fibrolamellar Hepatocellular Carcinoma ◽  
Fibrolamellar Carcinoma

Introduction: Fibrolamellar carcinoma is a rare primary hepatic malignant tumour, which was first described as a pathological variant of hepatocellular carcinoma. Aim: The aim of the paper is to discuss the case report of surgical treatment of a multicentric form of presumably fibrolamellar carcinoma significantly exceeding the Barcelona Clinic Liver Cancer staging system criteria, although tumour size and multi-organ lesions are not a contraindication to resection. Case study: This case report is an original one because the surgical intervention was performed on a patient with a multicentric fibrolamellar hepatocellular carcinoma with the initial foci of 16.0 × 12.0 × 9.0 cm and 10.5 × 8.7 × 7.5 cm. Results and discussion: The surgical intervention (right hemihepatectomy, lymphatic dissection D2) was performed as an independent treatment without prior chemotherapy. Conclusions: The surgical treatment occurred 5 years ago, and at the time of writing there has been no relapse and no sign of progression.

MicroRNAs as Potential Diagnostic and Prognostic Biomarkers in Hepatocellular Carcinoma

2019 ◽  
Vol 20 (11) ◽  
pp. 1129-1140 ◽  
Author(s):  
Seyed Mostafa Parizadeh ◽  
Reza Jafarzadeh-Esfehani ◽  
Maryam Ghandehari ◽  
Fatemeh Goldani ◽  
Seyed Mohammad Reza Parizadeh ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Early Diagnosis ◽  
Biological Activities ◽  
Radical Resection ◽  
High Rate ◽  
Average Survival ◽  
Specific Tumor ◽  
Serum Alpha Fetoprotein ◽  
Novel Biomarkers ◽  
Patients Experience

Hepatocellular carcinoma (HCC) is a common cancer, and the second most common cause of cancer-associated death globally. One of the major reasons for this high rate of mortality is a failure to make an early diagnosis. The average survival in untreated HCC patients is estimated to be approximately three months. The 5-year overall survival rate after radical resection is about 15-40% and within two years, more than two third of patients experience a relapse. To date, the most common biomarker which has been used for the diagnosis of HCC is serum alpha-fetoprotein (AFP). However, there is a lack of sensitive and specific tumor biomarkers for the early diagnosis of HCC. MicroRNAs are a class of short endogenous RNA with crucial role in many biological activities and cellular pathways and can be found in various tissues and body fluids. The aim of this review was to summarize the results of recent studies investigating miRNAs as novel biomarkers for the early diagnosis and prognostic risk stratification of patients with this type of liver cancer.

scholarly journals PO-56 Relationship between clinical presentation of SARS-CoV-2 infection and risk of thromboembolic events and prognosis in cancer patients

2021 ◽  
Vol 200 ◽  
pp. S45-S47
Author(s):  
J.L. Catoya Villa ◽  
A. Gutiérrez Ortiz de la Tabla ◽  
D.S. Juliao Caamaño ◽  
C. López Jiménez ◽  
C. Blanco Abad ◽  
...  
Keyword(s):  
Cancer Patients ◽  
Clinical Presentation ◽  
Thromboembolic Events
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