Progression after Immunotherapy for Fibrolamellar Carcinoma

Bauer, Ulrike; Mogler, Carolin; Braren, Rickmer; Algül, Hana; Schmid, Roland M.; Ehmer, Ursula

doi:10.1159/000497464

Cited by 23 publications

(17 citation statements)

References 20 publications

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“…76,78 Casereports on the use of ICIs are limited and response varies from progression to CR. 79 Notably CR was achieved in a patient with PD-L1 expression of 10%. 78 Therefore, further research is required to define the role of ICIs in the treatment of FLHCC (Table 2).…”

Section: Patients With Fibrolamellar and Mixed Hepato-cholangiocellulmentioning

confidence: 97%

Systemic treatment of HCC in special populations

Rimassa

Personeni

Czauderna

et al. 2021

Journal of Hepatology

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Recent years have seen significant progress in the systemic treatment of hepatocellular carcinoma (HCC), including the advent of immunotherapy. While several large phase III trials have provided the evidence for a multi-line treatment paradigm, they have focused on a highly selected group of patients by excluding potentially confounding comorbidities. As a result, high quality evidence for the systemic treatment of HCC in patients with various comorbidities is missing. This review summarises current knowledge on the use of approved medicines in patients with HIV, autoimmune disease, cardiovascular disease, diabetes, fibrolamellar HCC, mixed HCC-cholangiocarcinoma, decompensated cirrhosis (Child-Pugh B and C), a significant bleeding history, vascular invasion or portal vein thrombosis, as well as the elderly, those on haemodialysis, and those after solid organ transplantation. The article highlights relevant knowledge gaps and current clinical challenges. To improve the safety and efficacy of HCC treatment in these subgroups, future trials should be designed to specifically include patients with comorbidities.

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Section: Patients With Fibrolamellar and Mixed Hepato-cholangiocellulmentioning

confidence: 97%

Systemic treatment of HCC in special populations

Rimassa

Personeni

Czauderna

et al. 2021

Journal of Hepatology

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“…However, one might argue that these two patients might be bad candidates or did not have good criteria to receive more courses to show activity. The ineffectiveness and the early progression were also noticed in the study that used immunotherapy in the three cases of metastatic FLC [ 32 ].…”

Section: Discussionmentioning

confidence: 98%

“…One study reported 41% of MSH2 mutations with next-generation sequencing of FLC tumor tissue. In this study, three cases of metastatic FLC progressed after 2–3 months of starting PD-L1 antibodies, one treated with pembrolizumab, and the other two received nivolumab, although the progression was below 20% [ 32 ].…”

Section: Discussionmentioning

confidence: 99%

Fibrolamellar hepatocellular carcinoma treated with atezolizumab and bevacizumab: two case reports

Zahrani

Al-Fakeeh

2021

J Med Case Reports

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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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“…У обоих развилось прогрессирование заболевания через 2 и 3 мес. соответственно [73]. Однако в другом случае отмечен полный ответ после двух месяцев терапии ниволумабом, который продолжался и после 11 мес.…”

Section: лекарственная терапия флкunclassified

Fibrolamellar liver cancer: the modern concept

et al. 2020

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Fibrolamellar hepatocellular carcinoma (FLC) is a relatively rare primary liver tumor of unknown etiology (chronic infections with hepatitis B or C viruses, chronic alcohol poisoning, cirrhosis of the liver), which occurs mainly in the young population. FLK is more often diagnosed already at common stages due to the absence of pronounced clinical manifestations in patients for a long time. In pathomorphological diagnostics, FLC is a cluster of large polygonal eosinophilic cells with a clearly defined vesicular nucleus, surrounded by abundant growths of lamellar fibrosis. In most cases, collagen fibers are arranged in parallel strands; in metastatic tumors, collagen fibers can be arranged haphazardly (the so-called “tangled fibers”).For many years, attempts have been made to identify reliable markers for the detection and differential diagnosis of FLC and to determine the cause of tumors in young patients, and the molecular mechanisms of FLC carcinogenesis have been studied. In 2014 during the full transcriptome analysis of FLC samples, the chimeric transcript DNAJB1PRKACA was discovered, which is formed as a result of deletion of a section of chromosome 19 with a size of 400 thousand nucleotide pairs and leads to the fusion of two genes, which Is found in most FLC samples. Surgery is the only curative treatment of this tumor type and radical method of treating the disease. Given that lymph node involvement is an important prognostic factor, complete periportal lymphadenectomy should be performed as part of radical surgery for patients with FLK. The role and possibilities of locoregional and drug-based treatment methods are not fully clear, and the search for effective treatment regimens and potential targets specific to this form of HCR is urgent. Studies show conflicting results for different chemotherapy regimens and the use of targeted therapy. The literature describes isolated clinical cases of successful use of immunotherapy in patients with PD-L1-expressing tumors. The most important condition for successful treatment is an in-depth study of the molecular mechanisms of FLC carcinogenesis. This review presents current data on epidemiology, classification, clinico-morphological, molecular and genetic aspects, as well as some diagnostic features and FLC treatment modalities.

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Progression after Immunotherapy for Fibrolamellar Carcinoma

Cited by 23 publications

References 20 publications

Systemic treatment of HCC in special populations

Systemic treatment of HCC in special populations

Fibrolamellar hepatocellular carcinoma treated with atezolizumab and bevacizumab: two case reports

Fibrolamellar liver cancer: the modern concept

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