Chapter 93

### Key Points

1. Liver tumors are rare in children accounting for about 1.5% of all childhood malignancies. Primary pediatric malignant tumors include hepatoblastoma (HB), hepatocellular carcinoma (HCC), rhabdoid tumors, and sarcomas.

2. Age at presentation and level of alpha fetoprotein (AFP) are often the keys to differential diagnosis.

3. HB accounts for about 80% of the malignant tumors in children and the incidence appears to be increasing.

4. Pre-treatment extent of disease (PRETEXT) is based on cross-sectional imaging assessment of the extent of tumor involvement of the 4 main sections of the liver.

5. Surgical resection is recommended (1) by lobectomy or segmentectomy at diagnosis for PRETEXT I and II, (2) by lobectomy or trisegmentectomy after neoadjuvant chemotherapy for PRETEXT III (or POSTTEXT I, II or III and no major venous involvement −V and −P), and (3) by liver transplant or extreme resection for POSTTEXT III+V+P, and for any PRETEXT IV.

6. Only 0.5 to 1% of all Hepatocellular Carcinoma (HCC) manifest before 20 years of age and more than two-thirds of those are older than 10 years of age.

7. Chemotherapy is ineffective for HCC so primary radical tumor resection should be considered and all available techniques should be used to achieve this goal.

### Historical Context

Liver tumors in children encompass a wide spectrum of entities ranging from the congenital and acquired non-neoplastic masses to benign growths to the frankly malignant lesion. Complete surgical excision remains a therapeutic cornerstone in the management of most childhood liver tumors. From a historical standpoint, it is enlightening to realize how far techniques in liver resection have evolved. An early report on liver resection in children by Howat from the 1960s described 14 resections for malignant “hepatoma” of childhood with only 3 survivors and an operative mortality due to hemorrhage of 31%. The main risks of liver surgery, even though segmental vascular and biliary anatomy had been described by Couinaud in 1954 were bleeding and biliary fistula. In 1974, in a landmark survey of the American Academy of Pediatrics (AAP) Surgical Section examining outcomes following liver resection, Exelby reported over a 10% mortality rate following surgical intervention. Two-thirds of the children underwent incomplete tumor excision and none survived. Over the subsequent decade, various technical procedures were established to minimize bleeding including the Pringle maneuver (clamping of the afferent vascular pedicle), total vascular occlusion (clamping of the aorta and balloon occlusion of the inferior vena cava), hypothermia, preresection ligation of the hepatic inflow and outflow vasculature, and hypotensive anesthesia. The result was improved perioperative survival such that in 1982, Price reported a series of 11 pediatric tumor resections with no operative deaths.

With the continued evolution of techniques, development of new technology and a better understanding of the disease process, perioperative mortality following surgical resection of liver tumors have been substantially reduced. Treatment strategies have evolved such that multidisciplinary input should be employed in the management of virtually all children diagnosed with a liver ...

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