Fibrolamellar carcinoma (FLC) is a rare malignant entity arising from the liver and primarily affecting patients in late adolescence and young adulthood. FLC tumors are characterized by their unique histologic features and an only recently discovered genomic alteration: a chimeric fusion protein found in nearly all tumors. The rarity of these tumors coupled with the only recent acknowledgement of this genomic abnormality has likely led to disease under-recognition and de-prioritization of collaborative efforts aimed at establishing an evidence-guided standard of care. Surgical resection undoubtedly remains a mainstay of therapy and a necessity for cure but given the incidence of metastatic disease at diagnosis and high rates of distant relapse, systemic therapies remain a key component of disease control. There are few systemic therapies that have demonstrated proven benefit. Recent efforts have galvanized around single-institute or small consortia-based studies specifically focused on the enrollment of patients with FLC or use of agents with biologic rationale. This review will outline the current state of FLC epidemiology, histology, biology and trialed therapies derived from available published literature.

纤维板层癌 (FLC) 是一种罕见的恶性肿瘤，起源于肝脏，主要影响青春期晚期和青年期的患者。FLC 肿瘤的特征在于其独特的组织学特征和最近才发现的基因组改变：一种几乎在所有肿瘤中都发现的嵌合融合蛋白。这些肿瘤的罕见性加上最近才承认这种基因组异常可能导致疾病识别不足和旨在建立循证指导护理标准的合作努力的优先级降低。手术切除无疑仍然是治疗的主要手段和治愈的必要条件，但鉴于诊断时转移性疾病的发生率和远处复发率高，全身治疗仍然是疾病控制的关键组成部分。很少有系统疗法已证明有益。最近的努力围绕着单一机构或小型财团的研究，特别关注 FLC 患者的招募或具有生物学原理的药物的使用。这篇综述将概述 FLC 流行病学、组织学、生物学和试验疗法的现状，这些疗法源自已发表的文献。