Plain English summary of "Hepatocellular carcinoma in the pediatric population: a population based clinical outcomes study involving 257 patients from the surveillance, epidemiology, and end result (SEER) database (1973-2011)"
Published November 2015 in Hepatobiliary Surgery
Liver cancers that originate in the liver (primary liver cancers) in childhood are rare and comprise 1-2% of all pediatric tumors. Hepatocellular carcinoma (HCC) is the second most common primary liver cancer in children after hepatoblastoma and accounts for 1/3 of all primary liver cancers in children. Incidence of HCC is greater in areas of the world where hepatitis B infection is more frequent. Surgery remains the mainstream treatment for patients with HCC; however, survival rates are poor.
The SEER database is provided by the National Cancer Institute (NCI) from the National Institutes of Health (NIH). This study examined a large group of adult and pediatric HCC patients from the SEER database. Patients were grouped as pediatric (age ≤ 19) or adult (age ≥ 20). This study examined the 1-, 2- and 5- year overall survival, mortality and cancer-specific mortality.
63,771 cases of HCC were reported to the SEER database between 1973 and 2011. Average age of diagnosis was ~64. The overwhelming majority of HCC occurred in adults, however, 0.4% of the total cases observed (257 patients) occurred in pediatric patients with an average age of ~13 years. HCC was found to be more common among Caucasians in both the pediatric (50.4%) and adult population groups (50.5%).
Of the pediatric patients, 24.1% had fibrolamellar hepatocellular carcinoma (FLHCC), and was far more common among pediatric patients compared to adult patients (24.1% pediatric vs. 0.3% adult). 37% of FLHCC cases were diagnosed while the tumor was “localized disease” (only in the liver), 33.3% had regional disease (tumor found in the lymph nodes nearby) and 29.5% had distant disease (metastasis). FLHCC had a higher rate of regional disease and distant disease and a lower rate of localized disease compared to non-FLHCC. Patients with FLHCC had longer 1-, 2- and 5- year survival rates (67, 50, and 31%) compared to non-FLHCC (34, 23, and 12%). Despite presenting with larger tumors and more advanced disease, FLHCC was associated with prolonged survival compared to non-FLHCC in both the pediatric and adult populations.
Despite being the second most common primary hepatic malignancy in children, HCC remains a rare disease among pediatric patients accounting for approximately 0.5% of all new pediatric malignancies. Diagnosing pediatric HCC is often delayed, resulting in more advanced disease and larger tumors.
The takeaway: FLHCC is associated with prolonged survival compared to non-FLHCC in both the pediatric and adult populations.
--Dr. Benjamin Farber
Fibrolamellar Registry Scientific Advisory Board