A new study, recently published in Nature Precision Oncology, indicates that the incidence rate of fibrolamellar carcinoma (FLC) may be significantly higher than commonly cited statistics for the disease. The study, led by Dr. Travis Zack, Dr. John Gordan and Kurt Losert, combined a detailed analysis of electronic medical records (EMR) from a healthcare system with nationwide billing data.

Rare diseases like fibrolamellar are often poorly understood because of the difficulty in assembling accurate patient cohorts and the lack of ICD billing codes specific to the disease. This study used a unique approach that combined “narrow but detailed” EMR data with “broad but less specific” national payer billing information to define FLC incidence. The analysis suggests that FLC’s incidence rate is likely 5-8 times higher than the 0.02 per 100,000 rate reported in the Surveillance, Epidemiology, and End Results (SEER) Program statistics. While this approach might be artificially inflated by long-distance referrals of FLC patients to major medical centers, even the lowest estimate made was still five times higher than current SEER data after adjusting the analysis for referral distances.

The study also included an analysis of clinical trial data which identified a higher level of hyperammonemia in FLC patients than is currently recognized in clinical practice, suggesting hyperammonemia is an underappreciated source of comorbidity in FLC patients.

Click here to read or download the complete study.