[size=20px]A two-year-old boy presents with a large right upper quadrant mass. Imaging reveals a central tumor adjacent to the bifurcation of the portal vein with four lung lesions on the right side. Biopsy of the mass is consistent with hepatoblastoma. Chemotherapy is instituted. After three courses of chemotherapy, the hepatic tumor is smaller but remains intimately involved with the portal vein bifurcation and the lung lesions are diminished in size but not cleared. The oncologist would like to refer this patient to a pediatric transplant center but would like your advice regarding the lung lesions.

 

What is the best next course of action regarding his lung lesions?

 

a Radiofrequency ablation (RFA) of primary tumor

 

b Continue with additional chemotherapy

 

c Radiofrequency ablation (RFA) of metastasis

 

d Perform metastasectomy prior to liver transplant

 

e Perform liver transplant followed by metastasectomy[/size]

D

D

d

D

D, Residual lung lesions must be resected to ensure control of metastatic disease before proceeding with liver transplantation.

D

B

B

Correct answer

d Perform metastasectomy prior to liver transplant

 

Complete surgical resection remains the mainstay of treatment for hepatoblastoma (HB). Many tumors initially deemed unresectable or those with pulmonary metastasis may demonstrate a favorable response to neoadjuvant chemotherapy. Tumors adjacent to the hepatic vein confluence, portal vein bifurcation or retrohepatic inferior vena cava often do not demonstrate significant regression from these vascular structures with chemotherapy despite getting smaller in size. The current recommendation is for children with HB tumors that remain near the hepatic vein confluence, portal vein bifurcation or retrohepatic inferior vena cava, or tumors that are multicentric and have not responded after three cycles of chemotherapy to be referred to a pediatric liver transplant center for evaluation.

 

The recommended approach to pulmonary metastasis that do not resolve with chemotherapy depends upon the resectability of the primary tumor. Tumors that are amenable to complete resection following neoadjuvant chemotherapy should be removed with planned pulmonary metastasectomy following the procedure. For tumors that are not resectable, it is recommended to remove all extrahepatic disease prior to transplantation.

 

Continued chemotherapy is not recommended as there is not likely to be a significant response. Radiofrequency ablation (RFA) of the pulmonary metastasis or primary liver tumor would not be appropriate for this scenario. RFA is a secondary approach to local control for unresectable hepatocellular carcinoma, but is not currently recommended for treatment of primary HB. Investigators have reported treating pulmonary metastases in the face of recurrent or refractory disease with RFA, but it is not currently a first-line therapy for the scenario presented.