Treatment of Progressive or Recurrent Hepatoblastoma

Site of recurrence.

Previous treatment.

Individual patient considerations.

Surgical resection. In patients with hepatoblastoma that is completely resected at initial diagnosis, aggressive surgical treatment of isolated pulmonary metastases that develop in the course of the disease, in conjunction with an overall strategy that includes chemotherapy, may make extended DFS possible.[117,126,127]If possible, isolated metastases are resected completely in patients whose primary tumor is controlled.[128] A retrospective analysis of patients in the SIOPEL 1, 2, and 3 studies showed a 12% incidence of recurrence after complete remission by imaging and AFP levels. Outcome after recurrence was best if the tumor was amenable to surgery. Of patients who underwent chemotherapy and surgery, the 3-year EFS rate was 34%, and the OS rate was 43%.[126][Level of evidence C1] If all of the recurrent disease cannot be surgically removed, patients should consider enrolling in a clinical trial. Phase I and phase II clinical trials may be appropriate.

Chemotherapy. Analysis of survival after recurrence demonstrated that some patients treated with cisplatin/vincristine/fluorouracil could be salvaged with doxorubicin-containing regimens, but patients treated with doxorubicin/cisplatin could not be salvaged with vincristine/fluorouracil.[129] The addition of doxorubicin to vincristine/fluorouracil/cisplatin was clinically evaluated in the COG study AHEP0731 (NCT00980460). Combined vincristine/irinotecan and single-agent irinotecan have been used with some success.[123]; [122][Level of evidence C1]A review of COG phase I and II studies found no promising agents for relapsed hepatoblastoma.[130]

Liver transplant. Liver transplant should be considered for patients with nonmetastatic disease recurrence in the liver that is not amenable to resection.[71,98,101]

Percutaneous ablation. Percutaneous radiofrequency ablation has been used as an alternative to surgical resection of oligometastatic hepatoblastoma.[131][Level of evidence C1] Percutaneous ablation techniques may also be considered for palliation.[132]