Journal article
Fibrolamellar Hepatocellular Carcinoma: Comprehensive Review of Diagnosis, Imaging, and Management
Journal of the American College of Surgeons, Vol.236(2), pp.399-410
02/01/2023
DOI: 10.1097/XCS.0000000000000476
PMID: 36648268
Abstract
Fibrolamellar hepatocellular carcinoma (FLC) is a rare malignancy that primarily affects patients in late adolescence and young adulthood. FLC tumors are characterized by their unique histologic features and a recently discovered genomic alteration, a chimeric fusion protein found in nearly all tumors. This review article provides the latest advancements in diagnosing, imaging, and managing FLC.
A comprehensive systematic review was performed using MEDLINE/PubMed and Web of Science databases, with the end of search date being July 1, 2022, regarding FLC diagnosis, imaging, and management.
Surgical resection remains the mainstay of therapy offering a chance for cure; however, given the incidence of metastatic disease at diagnosis and high rates of distant relapse, systemic therapies remain a crucial component of disease control. Unfortunately, few systemic therapies have demonstrated proven benefits. Consequently, recent efforts have galvanized around single-institute or small consortia-based studies specifically focused on enrolling patients with FLC or using agents with a biologic rationale.
FLC has unique demographic, radiologic, and pathologic features. The rarity of these tumors, coupled with the only recent acknowledgment of the genomic abnormality, has likely led to disease underrecognition and deprioritization of collaborative efforts to establish an evidence-based standard of care. Despite R0 resection, most patients experience recurrence. However, surgical resection is feasible for many recurrences and is associated with good survival. The role of chemotherapy is evolving, and further research is required to define its role in managing this disease.
Details
- Title: Subtitle
- Fibrolamellar Hepatocellular Carcinoma: Comprehensive Review of Diagnosis, Imaging, and Management
- Creators
- Hassan Aziz - American College of SurgeonsZachary J Brown - The Ohio State University Wexner Medical CenterSeyedeh Panid Madani - University of BaltimoreIhab R Kamel - University of IowaTimothy M Pawlik - The Ohio State University Wexner Medical Center
- Resource Type
- Journal article
- Publication Details
- Journal of the American College of Surgeons, Vol.236(2), pp.399-410
- DOI
- 10.1097/XCS.0000000000000476
- PMID
- 36648268
- ISSN
- 1879-1190
- eISSN
- 1879-1190
- Language
- English
- Date published
- 02/01/2023
- Academic Unit
- Surgery
- Record Identifier
- 9984701541702771
Metrics
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