Fibrolamellar Hepatocellular Carcinoma and Noncirrhotic Hyperammonemic Encephalopathy

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 treated with atezolizumab and bevacizumab: two case reports

Journal of Medical Case Reports ◽

10.1186/s13256-021-02695-8 ◽

2021 ◽

Vol 15 (1) ◽

Author(s):

Ali AL Zahrani ◽

Ali Alfakeeh

Keyword(s):

Hepatocellular Carcinoma ◽

Checkpoint Inhibitors ◽

Case Reports ◽

Locally Advanced ◽

Young Patients ◽

Current Data ◽

Fibrolamellar Hepatocellular Carcinoma ◽

Underlying Liver Disease ◽

Expert Opinions ◽

Clinical Benefits

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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Fibrolamellar Carcinoma: A Multimodal Approach

GE Portuguese Journal of Gastroenterology ◽

10.1159/000507201 ◽

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.

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Patterns and predictive factors of recurrences after complete resection for fibrolamellar hepatocellular carcinoma

Journal of Clinical Oncology ◽

10.1200/jco.2007.25.18_suppl.15071 ◽

2007 ◽

Vol 25 (18_suppl) ◽

pp. 15071-15071

Author(s):

G. El Maalouf ◽

C. Le Tourneau ◽

V. Paradis ◽

F. Degos ◽

O. Farges ◽

...

Keyword(s):

Hepatocellular Carcinoma ◽

Multivariate Analysis ◽

Prognostic Factors ◽

Tumor Invasion ◽

Young Patients ◽

Vascular Tumor ◽

Complete Resection ◽

Fibrolamellar Hepatocellular Carcinoma ◽

Ajcc Staging

15071 Background: Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare subtype of primary HCC which often arises in young patients without underlying hepatic disease or viral infection. Clinical and pathological factors of prognosis are warranted. Methods: Descriptive, univariate and multivariate analysis of survival parameters were analyzed in a monocenter cohort of FL-HCC patients completely resected. Main variables were WHO-PS, age, AJCC stage, AFP level and detailed pathological features including EGFR, p- AKT, and PTEN expression. Results: 23 consecutive patients (median age: 30, M/F:6/17) with pathologically reviewed FL-HCC were analyzed. Median tumor size was 11 cm (range 4–23) and 6 patients had AFP>200ng/ml. two patients had respectable metastasis (lung or peritoneum), 5 patients had vascular invasion and 3 patients had lymph node metastasis achieving the following AJCC staging: I (70%), III (22%) and IV (8%). With median follow-up of 36 months, 3-year survival was 76% . Median time of recurrence was 23 months. Sites of first recurrence were hepatic in 5 patients, extra-hepatic in 4 patients, and both in 3 of 12 patients who recurred (52%). Metastasis occurred in lung, peritoneum, bone, skin and lymph nodes. Expression of EGFR, p-AKT, and PTEN was found in 94%, 69% and 0% of patients, respectively. None of the clinical parameters including AJCC staging predicted survival. In multivariate analysis, bad prognostic factors of recurrence were satellite micro-nodules (p=0.04) and micro-vascular tumor invasion (p=0.03) on pathological specimens. There was a trend toward a better survival in patients with <30% EGFR expression (p=0.06). Conclusions: FL-HCC patients with primary complete resection may develop extra-hepatic metastasis (58%) or isolated intra-hepatic recurrence (42%) within a median follow-up of 2 years. Prognostic factors of survival are pathological satellite nodules and micro-vascular tumor invasion. No significant financial relationships to disclose.

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

Seminars in Liver Disease ◽

10.1055/s-0037-1621710 ◽

2018 ◽

Vol 38 (01) ◽

pp. 051-059 ◽

Cited By ~ 12

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.

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