The liver is both a site for primary malignancies as well as for secondary metastasis. While hcc in cirrhosis represents more than 75% of malignancies and is associated with a long-term survival of 70% if specific selection criteria are applied, in selected cases of non-hcc malignancies or even liver metastases, ltx may be indicated.

Hilar Cholangiocarcinoma
Surgical resection and ltx are the only potentially curative treatment options for patients with hilar cholangiocarcinoma. After surgical resection, 5-year survival rates of 20% to 60% have been reported. If surgical resection is limited by proximal tumor extension, ltx has been proposed. Initial results of ltx for hilar cholangiocarcinoma have been disappointing and 5-year survival rates were rarely above 30%. Ltx using neoadjuvant radiochemotherapy, in combination with staging laparotomy to definitely exclude extrahepatic tumor involvement. The long-term patient survival of this cohort was excellent (5-year survival, 76%) and thus may be considered for selected patients. Nevertheless, the role of earlier diagnostic detection, biochemical and genetic markers, and neoadjuvant and/or adjuvant treatment approaches need to be further defined.

Intrahepatic Cholangiocarcinoma
Intrahepatic cholangiocarcinomas might be considered for ltx provided the tumor is either unresectable due to bilobar growth or the recipient has concomitant liver cirrhosis, which occurs in about 5% of patients. Overall, the 5-year survival in a spain cohort and in the unos database is around 40%, whereas it is slightly lower in the european liver transplant registry data for intrahepatic cholangiocarcinoma after ltx.

Hepatoblastoma
Unresectable hepatoblastoma, being the most common hepatic malignancy in early childhood, can be an indication for ltx. In cases of unresectable disease, a total experience of more than 300 cases (mainly us and european patient) demonstrate excellent 5-year survival rates of 70% to 80% after ltx. The results of “rescue transplantation” after previous incomplete tumor resection or after tumor recurrence are less encouraging, leading to 5-year survival rates of only 30% in one series.

Epitheloid Hemangioendothelioma
Epithelioid hemangioendothelioma is a rare malignancy and predominantly observed in young female patients. A major problem is the precise diagnosis and the prediction of its future course. The main differential diagnosis is hepatic angiosarcoma, which has a very poor prognosis (median survival of 6 months). Whereas ltx is contraindicated in angiosarcoma, epithelioid hemangioendothelioma has a good prognosis after transplantation. Ltx is often the only option, since resection is rarely possible due to the bilobar, multifocal nature of this tumor. A number of transplant centers and registries have analyzed their experience with epithelioid hemangioendothelioma and reported 5-year survival rates of 55% to 75%. Surprisingly, even the presence of extrahepatic metastases did not correlate with long-term survival, and therefore this specific tumor is not necessarily a contraindication for transplantation.

Liver Metastases of Neuroendocrine Tumors
Neuroendocrine tumors often present with unresectable liver metastases. Numerous treatment options have been applied including palliative surgery, chemotherapy, and interventional techniques, but all of these are associated with poor survival rates. Therefore, ltx has been evaluated, resulting in an overall ~50% 5-year survival. The reported results vary between 33% and 80% in various series, presumably due to different patient selection. Several factors associated with a more favorable survival have been defined. These include primary tumor located in the small bowel/lung, no previous extensive operations, no lymph node metastases and no extrahepatic disease, age below 50, normal e-cadherin expression, and ki-67 index < 5%. Ltx can be considered in selected patients, whereas it should be withheld in patients with dismal prognostic factors.

Colorectal Liver Metastases
Colorectal liver metastases were once considered an absolute contraindication for ltx. However, in the 1980s and early 1990s, more than 50 patients with colorectal liver metastases have reported to undergone ltx. The eltr reported 1- and 5-year survival rates of 62% and 18%, respectively. Today, oncological treatment as well as diagnostic imaging techniques have significantly improved, and therefore some to believe that this might also lead to a more specific selection of patients and that adjuvant treatment might help to improve the results after transplantation. Recently, the oslo group reported on 21 patients with various stages of colorectal metastasis who underwent ltx. Their 2- and 5-year survival rates were 89% and 60%, respectively. They reported that patients with large tumors (>5.5 Cm), those in whom primary colorectal resection was less than 2 years before ltx, and with cea >50 ug/l, were at greater risk for recurrence and death. Nevertheless many questions remain about the strategy in these cases, including the need for neo-adjuvant chemotherapy after ltx.