Hepatoblastoma and hepatocellular carcinoma prognosis by PRETEXT stage
The PRETEXT staging system has a moderate degree of interobserver variability, and the preoperative PRETEXT stage agrees with postoperative pathologic findings only 51% of the time, with overstaging in 37% of patients and understaging in 12% of patients.
The 5-year overall survival (OS) in the first international study of hepatoblastoma, in which the study protocol called for treatment of children with preoperative doxorubicin and cisplatin chemotherapy and included children with metastasis, was as follows:
- 100% for PRETEXT stage 1.
- 91% for PRETEXT stage 2.
- 68% for PRETEXT stage 3.
- 57% for PRETEXT stage 4.
- 25% for patients with metastasis.[5,6]
The second international study compared 3-year OS among hepatoblastoma patients by PRETEXT stage absent of extrahepatic disease. The 3-year OS was as follows:
- 100% for PRETEXT stage 1.
- 95% for PRETEXT stage 2.
- 84% for PRETEXT stage 3.
- 61% for PRETEXT stage 4.
The study also prospectively analyzed OS in patients by the presence of intraabdominal extrahepatic disease without distant metastasis (OS, 58%) and distant metastases (OS, 44%). Patients who underwent orthotopic liver transplant are included in all of the international study results. The COG is investigating prospective staging of hepatoblastoma patients using the PRETEXT system to determine the timing of surgery and the timing of early notification of liver transplant centers (COG-AHEP0731).
The 5-year OS for PRETEXT staged hepatocellular carcinoma was as follows:
- 44% for PRETEXT stage 1.
- 44% for PRETEXT stage 2.
- 22% for PRETEXT stage 3.
- 8% for PRETEXT stage 4.
Postsurgical Staging for Childhood Liver Cancer
A staging system based on operative findings and surgical resectability has been used in the United States to group children with liver cancer. This staging system is used to determine treatment.[10,11,12]
Hepatoblastoma prognosis by postsurgical stage
Stages I and II
In stage I hepatoblastoma, the tumor is completely resected.
In stage II hepatoblastoma, microscopic residual tumor remains after resection.
Approximately 20% to 30% of children with hepatoblastoma are stage I or II. Prognosis varies depending on the subtype of hepatoblastoma:
- Pure fetal histology (4% of hepatoblastomas) have a 3- to 5-year OS rate of 100% with minimal or no chemotherapy.[1,12,13]
- Non–pure fetal histology, non–small cell undifferentiated stage I and II hepatoblastomas have a 3- to 4-year OS rate of 90% to 100% with adjuvant chemotherapy.[1,5,7,12,14]
- If any small cell undifferentiated elements are present in stage I or II hepatoblastoma, the 3-year survival rate is 40% to 70%.[1,15]
In stage III hepatoblastoma, there is no distant metastases and one of the following is true:
- The tumor is either unresectable or the tumor is resected with gross residual tumor.
- There are positive lymph nodes.