Uncommon Hepatobiliary Tumours

Abstract

A large amount of data is available on hepatocellular carcinoma, cholangiocarcinoma, and liver metastases from colorectal or neuroendocrine origin. During the last decade, a multidisciplinary diagnostic and therapeutic approach has led to a considerable improvement in outcome, especially in high volume centres. Based on a large body of evidence, comprehensive treatment algorithms have recently been defined for these frequent entities [1, 5, 27]. The therapeutic backbone remains surgical resection or liver transplant. This has been complemented by interventional radiology, local ablation and innovative chemotherapy [1, 5, 20, 27]. However, only very little is known about the treatment of rare liver tumours, and this chapter will focus entirely on these neglected rare malignancies. Because of their rarity, no large prospective series are available. Prognostic factors and the optimal treatment still need to be defined. We will review the available data on these “orphans”, including rare epithelial and nonepithelial tumours, primary hepatic lymphoma as well as the veritable curiosities such as solitary fibrous tumour or carcinosarcoma. While treatment strategies are nowadays available for metastases from breast cancer, melanoma, and gastric cancer, rarities such as liver tumours of unknown origin constitute diagnostic and therapeutic challenges that have to be treated on a case-by-case basis [4, 5, 17, 19, 28, 32].