Determining primary or delayed surgery approach in fibrolamellar carcinoma of the liver based on stage.

Abstract

e16269 Background: Fibrolamellar carcinoma (FLC) is a rare, primary liver cancer usually presenting as advanced stage. FLC is often described as a "surgical disease," with primary surgery advocated for new diagnosis and systemic therapy for those deemed unresectable. We reviewed outcomes FLC stratified by stage and systemic therapy. Methods: 216 FLC cases from our database and 24 from the literature included: demographics, surgery, systemic therapy, staging, time to relapse and overall survival. We define “localized” as confined to the liver (AJCC stage 1), “regional” as regional lymph node involvement (AJCC stage 2 and 3), and “metastatic” (AJCC stage 4) as distant metastasis. Results: Of 155 patients with initial surgery, 44 (28%) had adjuvant systemic therapy and 31 of 85 ‘unresectable’ patients converted to resectable(36%). There was100% 10-year survival for localized disease, but frequent relapses nonetheless. Relapses were nearly universal in other stages. Adjuvant therapy gave regional patients a survival advantage and an improvement in RFS. Neo-adjuvant therapy benefitted metastatic patients with a delay in time to relapse. Benefit to 54 metastatic patients who had systemic therapy but no surgery was seen. Conclusions: Primary surgery for localized FLC offers excellent long-term (> 10 year) survival without systemic therapy, despite frequent relapses. Adjuvant therapy appears to benefit all non-localized patients. Regional and metastatic FLC should be considered systemic diseases and neoadjuvant systemic therapy before surgery should always be considered before committing to initial resection. Systemic therapy can covert unresectable patients to resectable. Overall survival (OS) and relapse free survival (RFS). Based on stage and systemic therapy: [No adjuvant versus adjuvant versus neoadjuvant and delayed resection]. Stage n Median follow up (months) Median OS (yrs) 1 yr OS 3 yr OS 5 yr OS 10 yr OS Median RFS (months) 1 yr RFS 3 yr RFS 5 yr RFS 10 yr RFS All Patients with Primary Surgery 155 54 0.98 0.87 71.76 0.46 21 0.61 0.25 0.17 0.03 Localized – Primary Surgery 25 98 >10 1.00 1.00 1.00 1.00 68 0.99 0.84 0.58 0.19 Localized –No Adjuvant 24 >10 1.00 1.00 1.00 1.00 70 0.95 0.87 0.60 0.20 Localized - Adjuvant 1 >10 1.00 1.00 1.00 1.00 18 1.00 0.00 0.00 0.00 Regional-Primary Surgery 97 60 8.5 0.98 0.90 0.69 0.44 14 0.62 0.17 0.13 0.00 Regional- No Adjuvant 71 7.3 0.98 0.89 0.64 0.40 13 0.57 0.13 0.11 0.00 Regional- Adjuvant 26 8.8 1.00 0.91 0.91 0.60 15 0.75 0.27 0.18 0.00 Metastatic-Primary surgery 33 39 3.9 0.97 0.70 0.46 0.14 8 0.32 0.07 0.00 0.00 Metastatic-No Adjuvant 16 4.5 0.94 0.79 0.50 0.33 4 0.13 0.00 0.00 0.00 Metastatic - Adjuvant 17 3.3 1.00 0.56 0.43 0.00 12 0.50 0.13 0.00 0.00 Metastatic- Neoadjuvant 85 35 4.6 0.99 0.72 0.46 0.21 19 0.68 0.06 0.00 0.00 Neoadjuvant andDelayed Resection 31 4.7 1.00 0.75 0.49 0.25 22 0.70 0.17 0.00 0.00 Neoadjuvant -No surgery 54 4.5 0.98 0.70 0.44 0.18 18 0.67 0.00 0.00 0.00