Recent improvement in survival outcomes and reappraisal of prognostic factors in hepatoblastoma

Abstract

Background Prognostic factors in hepatoblastoma need to be reevaluated considering the advances in treatment modalities. The study aimed to evaluate current outcomes of hepatoblastoma and reappraise the association of prognostic factors, including pre‐treatment extent of tumor (PRETEXT) stage with annotation factors and Children's Hepatic tumors International Collaboration‐Hepatoblastoma Stratification (CHIC‐HS) system, with survival outcomes. Methods We evaluated 103 consecutive patients with hepatoblastoma retrospectively according to the treatment period based on the introduction of a liver transplantation program. Results The 5‐year overall survival (OS), event‐free survival (EFS), and transplant‐free survival rates were 80.2%, 74.2%, and 61.8%, respectively. EFS and OS were improved significantly from 58.6% to 81.6% (P = 0.024) and from 58.6% to 90.8% (P < 0.001), respectively, in the late period (N = 74) compared with the early period (N = 29). The PRETEXT stage was significant or marginally significant for EFS and OS in the early period but not in the late period. The P, F, R, and C factors were significant for OS and EFS in the early period. However, in the late period, only the P factor was significant for OS, and the F and M factors were significant for EFS. The CHIC‐HS system was significant or marginally significant for EFS in both the early and late periods; however, it was significant for OS only in the early period. Conclusion Survival rates were significantly improved in children with hepatoblastoma, especially in those with advanced PRETEXT stages with positive annotation factors and in a high‐risk CHIC‐HS group. Prognostic factors had different clinical implications with evolved treatment modalities.

A practical approach to pediatric liver transplantation in hepatoblastoma and hepatocellular carcinoma

“…-Radical resection and efficient chemotherapy are both essential for the cure of HBL, which supports the role of LT for complete disease removal in the case of PRETEXT IV at diagnosis, in order to avoid local recurrence [17,[25][26][27] .…”

A quantitative universal NGS-based ctDNA assay for hepatoblastoma

“…Survival rates have significantly improved in children with hepatoblastoma, especially in the high risk group. Contributors to improved survival include the use of risk-adapted chemotherapy and advanced surgical approaches such as indocyanine green (ICG) fluorescence-guided surgery and liver transplantation 4 . However, about 12% of hepatoblastoma patients who have achieved a complete remission are likely to relapse in the liver and/or lungs 1 , and could benefit from better measurable residual disease (MRD) detection and modification of treatment options.…”

SNHG9 promotes Hepatoblastoma Tumorigenesis via miR-23a-5p/Wnt3a Axis

“…To date, treatment of HB patients mainly relies on surgical resection, neoadjuvant chemotherapy, and hepatic transplantation 19 - 23 . Over the last few decades, a revolutionary change has occurred in diagnostic tools, surgical techniques, and chemotherapeutic treatment regimes, resulting in a substantial improvement in survival rates 24 . However, there remains a small group (20-25%) of the HB patients with non-resectable HB tumors, chemoresistance and metastasis with a poor survival outcome.…”

“…Similarly, in papillary thyroid carcinoma, LncRNA Gas5 controls the PTEN expression by sponging miR-222-3p 29 , 30 . Small nucleolar RNA host gene 9 (SNHG9) 551 nucleotide base pair intergenic lncRNA located on chr16p13.3 have all been linked to the onset and progression of distinct cancers including pancreatic cancer 21 , glioblastoma 22 , lung cancer 23 , ovarian cancer 24 . However, the exact function and underlying mechanism of SNHG9 in hepatoblastoma tumorigenesis is unknown and needs to be explored.…”