Clinicopathological features and treatment outcomes of patients with fibrolamellar hepatocellular carcinoma: a retrospective multicenter study
BACKGROUND: Fibrolamellar Hepatocellular Carcinoma (FLHCC) is a rare primary liver tumour that is distinct from conventional HCC in both histopathological and clinical features. There is no clear consensus on its treatment. OBJECTIVE: Assess the demographics, prognosis, and treatment— particularly s...
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King Faisal Specialist Hospital and Research Centre
2025-07-01
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| Series: | Annals of Saudi Medicine |
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| Online Access: | http://www.annsaudimed.net/doi/10.5144/0256-4947.2025.249 |
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| author | Ali Kaan Güren Hilal Polat Eyüp Çoban Emir Çerme Gizem Bakır Kahveci Aslı Geçgel Pınar Ezgi Dama Lamia Şeker Can Sedat Biter Sıla Soylu Nargiz Majidova Nadiye Sever Erkam Kocaaslan Pınar Erel Yeşim Ağyol Abdussamet Çelebi Rukiye Arıkan Selver Işık İlkay Tuğba Ünek Ertuğrul Bayram Ozan Yazıcı Adem Deligönül Zeynep Hande Turna Murat Araz İbrahim Vedat Bayoğlu Osman Köstek Murat Sarı |
| author_facet | Ali Kaan Güren Hilal Polat Eyüp Çoban Emir Çerme Gizem Bakır Kahveci Aslı Geçgel Pınar Ezgi Dama Lamia Şeker Can Sedat Biter Sıla Soylu Nargiz Majidova Nadiye Sever Erkam Kocaaslan Pınar Erel Yeşim Ağyol Abdussamet Çelebi Rukiye Arıkan Selver Işık İlkay Tuğba Ünek Ertuğrul Bayram Ozan Yazıcı Adem Deligönül Zeynep Hande Turna Murat Araz İbrahim Vedat Bayoğlu Osman Köstek Murat Sarı |
| author_sort | Ali Kaan Güren |
| collection | DOAJ |
| description | BACKGROUND: Fibrolamellar Hepatocellular Carcinoma (FLHCC) is a rare primary liver tumour that is distinct from conventional HCC in both histopathological and clinical features. There is no clear consensus on its treatment. OBJECTIVE: Assess the demographics, prognosis, and treatment— particularly systemic therapies—of FLHCC patients across multiple centers in Türkiye. DESIGN: Retrospective SETTINGS: Multicenter PATIENTS AND METHODS: Patients with histopathological diagnosis of pure fibrolamellar hepatocellular carcinoma. MAIN OUTCOME MEASURES: Treatment responses of metastatic stage patients were evaluated. Progression-free survival (PFS) and overall survival (OS) analyses of metastatic stage first-line treatments were performed. SAMPLE SIZE: 39 patients with FLHCC RESULTS: The 5-year survival for all patients was 48%, 80% in stage 1, 57% in stage 2, 53% in stage 3 and 0% in stage 4. The median PFS for chemotherapy (n=10) and sorafenib (n=6) patients in the metastatic stage first series was 5.7 months and 2.8 months, respectively (P=.031). Median OS was 12.1 months for chemotherapy and 8.8 months for sorafenib (P=.853) in the metastatic stage. CONCLUSION: Conventional chemotherapies, especially gemcitabine and oxaliplatin combination can be used as systemic treatment options. Immunotherapies, chemoimmunotherapy and immunotherapy plus anti vascular endothelial growth factor combinations may be considered by clinicians. LIMITATIONS: Small sample size and the variability of the treatment modalities administered in the patients. |
| format | Article |
| id | doaj-art-dc971181e2914c4ea69a2daed95b3dfa |
| institution | Kabale University |
| issn | 0256-4947 0975-4466 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | King Faisal Specialist Hospital and Research Centre |
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| series | Annals of Saudi Medicine |
| spelling | doaj-art-dc971181e2914c4ea69a2daed95b3dfa2025-08-20T04:00:43ZengKing Faisal Specialist Hospital and Research CentreAnnals of Saudi Medicine0256-49470975-44662025-07-0145424925510.5144/0256-4947.2025.249Clinicopathological features and treatment outcomes of patients with fibrolamellar hepatocellular carcinoma: a retrospective multicenter studyAli Kaan Güren0Hilal Polat1Eyüp Çoban2Emir Çerme3Gizem Bakır Kahveci4Aslı Geçgel5Pınar Ezgi Dama6Lamia Şeker Can7Sedat Biter8Sıla Soylu9Nargiz Majidova10Nadiye Sever11Erkam Kocaaslan12Pınar Erel13Yeşim Ağyol14Abdussamet Çelebi15Rukiye Arıkan16Selver Işık17İlkay Tuğba Ünek18Ertuğrul Bayram19Ozan Yazıcı20Adem Deligönül21Zeynep Hande Turna22Murat Araz23İbrahim Vedat Bayoğlu24Osman Köstek25Murat Sarı26From the Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, TürkiyeFrom the Başakşehir State Hospital, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Uludag University School of Medicine, Bursa, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University-Cerrahpasa, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Trakya University School of Medicine, Edirne, TurkeFrom the Division of Medical Oncology, Department of Internal Medicine, Ege University School of Medicine, Izmır, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Ege University School of Medicine, Izmır, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Dokuz Eylul University School of Medicine, Izmir, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Bezmiâlem Foundation University, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Cukurova University School of Medicine, Adana, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Dokuz Eylul University School of Medicine, Izmir, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Cukurova University School of Medicine, Adana, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Gazi University School of Medicine, Ankara, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Uludag University School of Medicine, Bursa, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Cerrahpasa Medical Faculty, Istanbul University-Cerrahpasa, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Necmettin Erbakan University School of Medicine, Konya, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Medipol University School of Medicine, Istanbul, TürkiyeFrom the Division of Medical Oncology, Department of Internal Medicine, Medipol University School of Medicine, Istanbul, TürkiyeBACKGROUND: Fibrolamellar Hepatocellular Carcinoma (FLHCC) is a rare primary liver tumour that is distinct from conventional HCC in both histopathological and clinical features. There is no clear consensus on its treatment. OBJECTIVE: Assess the demographics, prognosis, and treatment— particularly systemic therapies—of FLHCC patients across multiple centers in Türkiye. DESIGN: Retrospective SETTINGS: Multicenter PATIENTS AND METHODS: Patients with histopathological diagnosis of pure fibrolamellar hepatocellular carcinoma. MAIN OUTCOME MEASURES: Treatment responses of metastatic stage patients were evaluated. Progression-free survival (PFS) and overall survival (OS) analyses of metastatic stage first-line treatments were performed. SAMPLE SIZE: 39 patients with FLHCC RESULTS: The 5-year survival for all patients was 48%, 80% in stage 1, 57% in stage 2, 53% in stage 3 and 0% in stage 4. The median PFS for chemotherapy (n=10) and sorafenib (n=6) patients in the metastatic stage first series was 5.7 months and 2.8 months, respectively (P=.031). Median OS was 12.1 months for chemotherapy and 8.8 months for sorafenib (P=.853) in the metastatic stage. CONCLUSION: Conventional chemotherapies, especially gemcitabine and oxaliplatin combination can be used as systemic treatment options. Immunotherapies, chemoimmunotherapy and immunotherapy plus anti vascular endothelial growth factor combinations may be considered by clinicians. LIMITATIONS: Small sample size and the variability of the treatment modalities administered in the patients.http://www.annsaudimed.net/doi/10.5144/0256-4947.2025.249Fibrolamellar HCCGemcitabine-oxaliplatinSorafenibImmunotherapyDNAJB1-PRKACA |
| spellingShingle | Ali Kaan Güren Hilal Polat Eyüp Çoban Emir Çerme Gizem Bakır Kahveci Aslı Geçgel Pınar Ezgi Dama Lamia Şeker Can Sedat Biter Sıla Soylu Nargiz Majidova Nadiye Sever Erkam Kocaaslan Pınar Erel Yeşim Ağyol Abdussamet Çelebi Rukiye Arıkan Selver Işık İlkay Tuğba Ünek Ertuğrul Bayram Ozan Yazıcı Adem Deligönül Zeynep Hande Turna Murat Araz İbrahim Vedat Bayoğlu Osman Köstek Murat Sarı Clinicopathological features and treatment outcomes of patients with fibrolamellar hepatocellular carcinoma: a retrospective multicenter study Annals of Saudi Medicine Fibrolamellar HCC Gemcitabine-oxaliplatin Sorafenib Immunotherapy DNAJB1-PRKACA |
| title | Clinicopathological features and treatment outcomes of patients with fibrolamellar hepatocellular carcinoma: a retrospective multicenter study |
| title_full | Clinicopathological features and treatment outcomes of patients with fibrolamellar hepatocellular carcinoma: a retrospective multicenter study |
| title_fullStr | Clinicopathological features and treatment outcomes of patients with fibrolamellar hepatocellular carcinoma: a retrospective multicenter study |
| title_full_unstemmed | Clinicopathological features and treatment outcomes of patients with fibrolamellar hepatocellular carcinoma: a retrospective multicenter study |
| title_short | Clinicopathological features and treatment outcomes of patients with fibrolamellar hepatocellular carcinoma: a retrospective multicenter study |
| title_sort | clinicopathological features and treatment outcomes of patients with fibrolamellar hepatocellular carcinoma a retrospective multicenter study |
| topic | Fibrolamellar HCC Gemcitabine-oxaliplatin Sorafenib Immunotherapy DNAJB1-PRKACA |
| url | http://www.annsaudimed.net/doi/10.5144/0256-4947.2025.249 |
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