*Purpose: Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare tumour accounting for ~1% of all primary liver cancers. Due to its rarity, there is a shortage of data on liver transplantation (LT) for patients with FL-HCC. To gain more insight on the management of these patients, we conducted a comprehensive multicenter analysis of FL-HCC patients looking at both post-listing and post-LT outcomes.

*Methods: Patients diagnosed with FL-HCC and listed for LT or transplanted between 1987-2019 were extracted from the UNOS registry. Re-LT and multi-organ transplants were excluded. Outcomes were waitlist (WL) dropout and ITT overall survival (OS), derived from all patients with a FL-HCC diagnosis at listing, and post-transplant OS, recurrence-free survival (RFS), cumulative incidence of recurrence, and 30-/90-day post-transplant mortality, for all patients with a confirmed FL-HCC diagnosis at explant pathology. WL dropout was defined as dropping out of the WL due to death, clinical deterioration, or medical unsuitability. Median follow-up times were 4.5 years (interquartile range [IQR] 1.2-8.2) from listing and 5.0 years (IQR 2.6-9.8) post-LT.

*Results: A total of 96 unique patients with a diagnosis of FL-HCC at listing or post-LT were extracted. Of these, 53% were female and 76% adult, with a median age of 25.5 years (range 10-66). Cirrhosis was present in 10 patients (10%) and 8 patients had an underlying disease (8%): hepatitis C virus (5), non-alcoholic steatohepatitis (2), glycogen storage disease (1). Of all FL-HCC patients, 72 were diagnosed at listing (75%), 10 post-LT (10.5%), and of 14 the moment of diagnosis was unknown (14.6%). Sixty-nine patients received a LT, of which 55 were post-operatively confirmed of having FL-HCC. Eight patients received a living donor LT. WL dropout at 1- and 2-years post-listing was 15.3% and 19.4%. ITT OS at 1, 3, 5 years was 86.5%, 67.9%, 55.5%, where post-LT OS was 98.2%, 84.4%, 61.9% at 1, 3, 5 years. RFS was 85.3%, 69.9%, and 47.5% at 1, 3, and 5 years. The cumulative incidence of recurrence at 1, 3, 5 years was 12.8%, 24.4%, and 44.7%. There was no statistical difference in all outcomes between adult and pediatric patients.

*Conclusions: LT for FL-HCC patients offers acceptable long-term survival outcomes despite a substantial risk of recurrence. Further research is needed to determine which patients with FL-HCC would benefit most from LT and potential adjuvant therapies to reduce the risk of recurrence.

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