What is your preferred frontline regimen for metastatic unresectable fibrolamellar carcinoma in an AYA patient?

We have successfully used GEMOX-LEN along with multidisciplinary locoregional tumor debulking to convert more than 50% of unresectable stage IV FLC to definitive R0 resection and surgical remission, nearly 100% achieving 0.00 circulating tumor DNA (we use Signatera):

• Ginters et al., GI Cancer Trials 2024
• Kent et al., ASCO 2024

Our current cohort is at n=108 patients, 85% are stage IV, and 98% are unresectable. We continue to see reliable responses. Important to be patient - best responses come 6-9 months after starting, and in fact, an early increase in size from edema/necrosis/hemorrhage is common and not to be considered progression. About 1/2 of these patients get debulking from radiation (FLC is very radiosensitive, even modest doses ...