**2.3 Ampullary tumours**

Compared to pancreatic carcinoma and cholangiocarcinoma, ampullary neoplasm is the least common and aggressive tumour. In general, ampullary tumour has better clinical outcomes even when the tumour is not resectable (Heinrich and Clavien 2010). Whilst ampullary tumours can occur sporadically, they are often seen in the context of genetic syndromes such as familial adenomatous polyposis and hereditary non-polyposis colorectal cancer, in whom the risk is 100 times more than the general population (Offerhaus et al., 1992). As endoscopic screening and surveillance program is adopted for these at-risk individuals, most tumours are adenomas at detection, though the potential of malignant transformation to carcinomas is high (Jean and Dua, 2003; Fischer and Zhou, 2004). Currently, there is no consensus on the management of ampullary tumors. Factors that impact treatment strategy include the patient's general health, tumor characteristics, and available expertise. Ampullary adenomas, especially those with high-grade dysplasia, warrant therapy because they are "time bombs" for malignancy and may already harbor malignancy missed on biopsy (Heinrich and Clavien 2010). Although endoscopic resection is widely embraced as first-line therapy in patients with benign ampullary tumors (Binmoeller et al., 1993; Beger et al., 1999; Cheng et al., 2004), the final treatment decision is based on the histological findings of the ampullectomized specimen. The presence of invasive carcinoma in the specimen indicates the need for definitive surgical resection. In patients who are poor candidates for surgery or who refuse surgery, endoscopic resection with ablative therapy can be considered despite unfavorable tumor characteristics (Nguyen et al. 2010). Endoscopic ampullectomy has also been reported to successfully eradicate large ampullary adenomas (Zadorova et al., 2001), early T1 ampullary adenocarcinoma

2008). Less than 15% of pancreatic cancers are intraductal mucinous papillary neoplasm (IPMN), solid pseudopapillary neoplasm, pancreatoblastoma, mucinous cystadenocarcinoma, adenosquamous carcinoma and acinar cell carcinoma (Ghaneh et al., 2008). Given the preponderance pancreatic head location of the tumours, painless cholestatic symptoms are the most common presentation (Ghaneh et al., 2008). Anorexia, abdominal

This is rare malignant disease of the epithelial cells in the intra- and extrahepatic bile ducts and the incidence is increasing, especially the intra-hepatic subtype (Patel, 2001). In addition to liver flukes infestation, hepatitis B and C infections have recently been associated with rise of cholangiocarcinoma in the developing countries, and are thought to be responsible for the increasing incidence of intra-hepatic cholangiocarcinomas. In the western countries, primary sclerosing cholangitis and congenital anomalies such as Caroli's syndrome and choledochal

As with pancreatic cancer, most of the cholangiocarcinomas are unresectable at presentation and the prognosis for these patients is dismal. Clinical presentations of cholangiocarcinoma are dependent on tumour location (Patel, 2006). Extrahepatic tumours, including those involving the bifurcation usually show signs of biliary obstruction with jaundice and pale stools. In contrast, intra-hepatic cholangiocarcinomas more often present with late symptoms of malignancy such as weight loss, loss of appetite, and abdominal pain or mass.

Compared to pancreatic carcinoma and cholangiocarcinoma, ampullary neoplasm is the least common and aggressive tumour. In general, ampullary tumour has better clinical outcomes even when the tumour is not resectable (Heinrich and Clavien 2010). Whilst ampullary tumours can occur sporadically, they are often seen in the context of genetic syndromes such as familial adenomatous polyposis and hereditary non-polyposis colorectal cancer, in whom the risk is 100 times more than the general population (Offerhaus et al., 1992). As endoscopic screening and surveillance program is adopted for these at-risk individuals, most tumours are adenomas at detection, though the potential of malignant transformation to carcinomas is high (Jean and Dua, 2003; Fischer and Zhou, 2004). Currently, there is no consensus on the management of ampullary tumors. Factors that impact treatment strategy include the patient's general health, tumor characteristics, and available expertise. Ampullary adenomas, especially those with high-grade dysplasia, warrant therapy because they are "time bombs" for malignancy and may already harbor malignancy missed on biopsy (Heinrich and Clavien 2010). Although endoscopic resection is widely embraced as first-line therapy in patients with benign ampullary tumors (Binmoeller et al., 1993; Beger et al., 1999; Cheng et al., 2004), the final treatment decision is based on the histological findings of the ampullectomized specimen. The presence of invasive carcinoma in the specimen indicates the need for definitive surgical resection. In patients who are poor candidates for surgery or who refuse surgery, endoscopic resection with ablative therapy can be considered despite unfavorable tumor characteristics (Nguyen et al. 2010). Endoscopic ampullectomy has also been reported to successfully eradicate large ampullary adenomas (Zadorova et al., 2001), early T1 ampullary adenocarcinoma

pain or mass and weight loss often indicate the presence of advanced disease.

cysts are the main predisposing risk factors for cholangiocarcinoma (Patel, 2006).

**2.2 Cholangiocarcinoma** 

**2.3 Ampullary tumours** 

(Katsinelos et al., 2007), and even lesions with intraductal growth (Bohnacker et al., 2005). Given its tumour behaviour, clinical presentation and treatment modality are very different to that of pancreatic cancer and cholangiocarcinoma, ampullary tumours will not be discussed further in this chapter.
