Predictors of Outcome in Patients With Fibrolamellar Carcinoma: Analysis of the National Cancer Database

Abstract

Background: Fibrolamellar carcinoma (FLC) is a very rare liver tumor. We aimed to retrospectively analyze the clinicopathological factors and treatment modalities affecting overall survival (OS) in FLC. The objective of the study was to identify predictors of survival in FLC. Patients and Methods: Using the National Cancer Database, we identified 496 patients diagnosed with FLC between 2004 and 2015. Clinicopathological, treatment, and survival data were collected. Results: Hepatic resection was performed on 254 (51.2%) patients, liver-directed therapy on 13 (2.6%) patients, and liver transplantation on 15 (3.0%) patients.

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

“…46 Curative intent resections achieve R0 resection rates from 81% (reported by Darcy et al in pediatric patients) up to 96% (McDonald et al, National Cancer Database), although there is a notable selection bias and omission of missing data in such estimates. 6,44,47 In very selected cases, palliative surgical tumor reduction can be performed in order to alleviate symptoms such as hyperammonemic encephalopathy but this is absolutely not standard. 48 Resection is a factor greatly influencing the prognosis, with 5-year survival rates reaching up to 86% for stage I patients.…”

“…2,3 In the majority of the literature there is a slightly higher incidence among women and no specific race or geographical region related imbalance in incidence. [4][5][6] No environmental exposures or toxins such as aflatoxin have been directly associated with FLC and neither have alcohol use, iron overload or other factors that contribute to conventional hepatocellular carcinomas. This is substantiated by the fact that the liver tissue surrounding the tumors seldom demonstrates any pathological findings.…”

New insights into the pathophysiology and clinical care of rare primary liver cancers

“…Most FLCs are diagnosed before 40 years, with the median age of diagnosis ranging from 20-29 years. [77][78][79] FLCs are larger (9-13 cm) with a higher rate of lymph node invasion (43-46%) compared to HCC. 71,[75][76][77] The most frequent sites of metastases are the lung (50%), bone (19.2%), and brain (1.9%).…”

“…71,[75][76][77] The most frequent sites of metastases are the lung (50%), bone (19.2%), and brain (1.9%). 78 Pathophysiology At the molecular level, DNAJB1-PRKCA fusiondue to a focal deletion in the chromosome 19is identified in almost all FLCs and is considered highly specific but not pathognomonic (Table 2). [79][80][81] The same fusion was identified in intraductal oncocytic papillary neoplasms of the pancreas and bile duct.…”

“…However, no significant difference in 5-year survival could be observed when focusing on patients without cirrhosis, suggesting that the difference observed in the overall population was likely related to the severity of the underlying liver disease. 70,72,78 Currently, liver resection remains the most effective curativeintent treatment option for FLC; aggressive initial surgical resection along with regional lymphadenectomy is advised. 76,77 In contrast, results of LT are impaired by a high rate of tumour recurrence leading to a 5-year overall survival of 35%.…”