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 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 (=.031). Median OS was 12.1 months for chemotherapy and 8.8 months for sorafenib (=.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.