Factors Influencing Overall Survival for Patients with Fibrolamellar Hepatocellular Carcinoma: Analysis of the Surveillance, Epidemiology, and End Results Database

Abstract

Background The study aimed to develop a nomogram to predict overall survival (OS) for patients with FLC using a national database. Methods The Surveillance, Epidemiology, and End Results database of the National Cancer Institute was reviewed to identify FLC cases with histological confirmation between 2004 and 2014. Cox proportional hazards models were used to identify factors associated with OS. The validation of the nomogram was performed using concordance index (C-index) and calibration curves. Results Out of 170 cases with complete follow-up, 87 received surgery/ablation and 12 received transplantation with significantly higher OS than chemotherapy alone while transplantation combined with chemotherapy showed better survival than solely transplantation. The combination of surgery and chemotherapy showed worse OS than surgery alone. Survival was negatively influenced by T4 stadium (HR = 5.91), while young age and surgery were positive predictive factors. There was no influence of gender, ethnicity or nodal status on survival. The rate of AFP positivity was comparable with and without the presence of distal metastases. Conclusion FLC survival is greatly dependent upon appropriate surgical management irrespective of tumor stadium.

A Multidisciplinary Approach to the Management of Fibrolamellar Carcinoma: Current Perspectives and Future Prospects

“…33 This misclassification could account for the discrepancies in patient ages reported in original studies and the SEER database analyses where a higher proportion of patients older than 50 years of age are commonly described. 1,4,[34][35][36][37][38] After the discovery of the DNAJB1-PRKACA chimera, FISH DNAJB1-PRKACA has been introduced as a diagnostic technique increasing specificity to almost 100% but has not yet been introduced to routine practice. 31,39 https://doi.org/10.2147/OTT.S296127…”

“…40 In the available reports the tumor stage was most often T1, comprising 42%, but vascular invasion is frequent in the tumor, thus T2 (13%) and T3 (28%) are also prevalent at diagnosis. 34 T4 has been reported in approximately 8% of cases. Unknown stage at presentation was reported in 9% of cases, as reported by a recent SEER database analysis.…”

“…Unknown stage at presentation was reported in 9% of cases, as reported by a recent SEER database analysis. 34 Most FLC are diagnosed at an advanced stage, with AJCC stage III and IV amounting to 70-80% due to frequent lymph node and major blood vessels' involvements. 41…”

“…Unfavorable factors include vascular and lymph-node invasion, tumor multiplicity and metastases at baseline. 34,35,49,52 Furthermore, young age has been shown to positively influence survival, especially in the SEER database analyses, while risk estimates on sex are either insignificant or in favor of advantageous prognosis for female patients. Liver transplantation is not a common therapeutic strategy for FLC on organizational grounds such as the need for specialized centers and lack of grafts in some regions but also due to the existence of nodal infiltration.…”

Fibrolamellar Carcinoma with DNAJB1-PRKACA Fusion in a 16-Year-Old: Case Report and Review of Literature

“…Even though surgery is the primary mode of treatment, only 30% of newly diagnosed patients undergo surgery, and the 5-year survival rate is less than 80% (23). Regardless of combination with neoadjuvant chemotherapy, surgery offers better survival than systemic therapy alone (24). Most inoperable HCC cases remain unresponsive to chemotherapy, with a poor prognosis (25).…”