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    Childhood Liver Cancer Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Stage Information

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    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.[11,12,13]

    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.[1,13,14]
    • 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.[1,6,8,13,15]
    • If any small cell undifferentiated elements are present in stage I or II hepatoblastoma, the 3-year survival rate is 40% to 70%.[1,16]

    Stage III

    In stage III hepatoblastoma, there are 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.

    Approximately 50% to 70% of children with hepatoblastoma are stage III. The 3- to 5-year OS rate for children with stage III hepatoblastoma is less than 70%.[1,6,8,13,17]

    Stage IV (distant metastases)

    In stage IV hepatoblastoma, there is distant metastasis regardless of the extent of liver involvement.

    Approximately 10% to 20% of children with hepatoblastoma are stage IV. The 3- to 5-year OS rate for children with stage IV hepatoblastoma vary widely based on published reports, from 20% to approximately 60%.[1,6,7,8,13,17]

    Hepatocellular carcinoma prognosis by postsurgical stage of disease at diagnosis

    • Children with stage I hepatocellular carcinoma have a good outcome.[18]
    • Stage II is too rarely seen to predict outcome.
    • Stages III and IV are usually fatal.[10,19]

    Treatment Options Under Clinical Evaluation: COG Hepatoblastoma Risk Groups

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