Abstract

Primary tumors of the liver can be of epithelial, mesenchymal, or mixed origin. Primary malignant epithelial tumors are the most common hepatic tumors. They include hepatocellular carcinoma (HCC), cholangiocarcinoma (CCA), hepatoblastoma (HB), and a variety of other more rare tumors. HCC has a relentless clinical course. It is characterized by a fulminant evolution, a low rate of resectability, and poor response to nonsurgical therapies. Reports on surgical resection of HCC point toward a complete resectability rate of 3% to 30%. Reported survival ranges from 55% to 90% at 1 year to 10 years and 50% at 5 years. Operative mortality for cirrhotics is between 7% and 25%, although it decreases to less than 3% for noncirrhotics. Multifocal tumors are associated with higher recurrence rates and lower survival times. Tumor size (with 5 cm the usual cutoff point) affects not only survival, but also resectability. Tumor location, well-differentiated histologic grade, presence of a tumor capsule, absence of vascular invasion, and fibrolamellar variety seem to be associated with better outcomes. Tumor size, spread and location, and hepatic functional reserve capacity should be considered before surgery. Ascites and jaundice are unfavorable factors that are associated with adverse outcomes. Favorable prognostic factors include the following: (1) encapsulation of the tumor, (2) negative resection margins, (3) lack of capsular invasion, (4) lack of venous invasion, and. (5) absence of microsatellite lesions. Fibrolamellar carcinoma behaves in a more benevolent fashion in that it has a 50% to 75% resectability rate, with an average survival of 32 to 68 months. HCC treated with liver transplantation may have a recurrence rate sometimes as high as 65%. Actuarial survival rates at 1 and 5 years range from 42% to 71% and 20% to 45%, respectively. Based on results reported by the Klintmalm registry, patient survival is significantly influenced by tumor size greater than 5 cm, vascular invasion, node involvement, and histologic grade. No difference in survival was observed when incidental tumors, fibrolamellar variety, multifocality, and bilobar involvement were considered by univariate analysis. Only histologic grade had a negative impact on overall survival in patients with known tumors. When tumors found incidentally were considered, patient survival was affected by multifocality and age older than 60 years. Postoperative recurrence-free survival was significantly diminished by tumor histologic grade and size greater than 5 cm. Others have found that favorable prognostic factors include incidental finding of tumor during hepatectomy or pathologic analysis, fibrolamellar variety, and small (<5 cm) tumor size. Unfavorable factors include poor differentiation, vascular invasion, extrahepatic metastases, and lymph node involvement (closely linked with pTNM staging). Although reports are somewhat conflicting, cirrhosis seems to affect survival. CCA is classified by its location. We have chosen to subdivide CCA into 2 main groups: central (intrahepatic and hilar) and peripheral. Although there have been numerous reports of surgical treatment of hilar CCA long-term survival has been poor, with few exceptions. One- and 5-year survival rates after resection range from 58% to 79% and 5% to 40%. High perioperative mortality, due to hepatic failure and sepsis, is common. The role of liver transplantation has been debatable in the face of organ shortage. However, categorical denial of liver transplantation for CCA is unjustifiable. An evaluation of multiple studies points toward a surgical resectability rate of 20% to 50% and an operative mortality of 0% to 27%. One-, 3-, and 5-year survival rates were 70% to 100%, 20% to 50%, and 8% to 35%, respectively. Recurrence rates were found to be as high as 75%. HB is the most common malignant liver tumor in children under the age of 5 years. During the last decade, developments in imaging technology, chemotherapy, and surgical techniques have improved the treatment of pediatric liver tumors. Surgery still remains the cornerstone of therapy within a multimodality treatment protocol. Liver transplantation allowed for improved survival in patients with otherwise unresectable disease and should be used, in such instances, as part of a well outlined protocol. Hepatic epithelioid hemangioendothelioma is a low- to intermediate-grade vascular endothelial malignant tumor of unpredictable natural history. No clear-cut association between pretransplantation liver spread and posttransplantation recurrence and survival has been found.

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