Güren Ali Kaan, Polat Hilal, Çoban Eyüp, Çerme Emir, Kahveci Gizem Bakır, Geçgel Aslı, Ezgi Dama Pınar, Can Lamia Şeker, Biter Sedat, Soylu Sıla, Majidova Nargiz, Sever Nadiye, Kocaaslan Erkam, Erel Pınar, Ağyol Yeşim, Çelebi Abdussamet, Arıkan Rukiye, Işık Selver, Ünek İlkay Tuğba, Bayram Ertuğrul, Yazıcı Ozan, Deligönül Adem, Turna Zeynep Hande, Araz Murat, Bayoğlu İbrahim Vedat, Köstek Osman, Sarı Murat
From the Division of Medical Oncology, Department of Internal Medicine, Marmara University School of Medicine, Istanbul, Türkiye.
From the Başakşehir State Hospital, Istanbul, Türkiye.
Ann Saudi Med. 2025 Jul-Aug;45(4):249-255. doi: 10.5144/0256-4947.2025.249. Epub 2025 Aug 7.
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.
Assess the demographics, prognosis, and treatment- particularly systemic therapies-of FLHCC patients across multiple centers in Türkiye.
Retrospective.
Multicenter.
Patients with histopathological diagnosis of pure fibrolamellar hepatocellular carcinoma.
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.
39 patients with FLHCC.
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 (=.031). Median OS was 12.1 months for chemotherapy and 8.8 months for sorafenib (=.853) in the metastatic stage.
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.
Small sample size and the variability of the treatment modalities administered in the patients.
纤维板层型肝细胞癌（FLHCC）是一种罕见的原发性肝脏肿瘤，在组织病理学和临床特征上均与传统肝细胞癌不同。其治疗方法尚无明确共识。
评估土耳其多个中心FLHCC患者的人口统计学、预后及治疗情况，尤其是系统治疗。
回顾性研究。
多中心。
组织病理学诊断为纯纤维板层型肝细胞癌的患者。
评估转移性阶段患者的治疗反应。对转移性阶段一线治疗进行无进展生存期（PFS）和总生存期（OS）分析。
39例FLHCC患者。
所有患者的5年生存率为48%，1期为80%，2期为57%，3期为53%，4期为0%。转移性阶段首个系列中化疗患者（n = 10）和索拉非尼患者（n = 6）的中位PFS分别为5.7个月和2.8个月（P =.031）。转移性阶段化疗的中位OS为12.1个月，索拉非尼为8.8个月（P =.853）。
传统化疗，尤其是吉西他滨和奥沙利铂联合方案可作为系统治疗选择。临床医生可考虑免疫治疗、化疗免疫治疗以及免疫治疗加抗血管内皮生长因子联合方案。
样本量小以及患者所接受治疗方式的变异性。
