Neoplastic Conditions

Ampullary adenocarcinoma: arising from adenomatous dysplasia of either the periampullary duodenal or intra-ampullary duct mucosae, it is one of the commonest causes of death in FAPC. Adenoma may be amenable to local excision but radical surgical resection is often required for large lesions and because a surface biopsy showing epithelial dysplasia may harbour underlying invasive adenocarcinoma. Most cases have a well-defined intestinal pattern but in a minority it can be difficult to separate adenocarcinoma of the duodenal papilla, ampulla, distal pancreatic duct and distal common bile duct as they can share similar well-to-moderately differentiated tubular and ductular patterns. Detailed examination of the exact anatomical location in the resection specimen is required and sometimes the only conclusion possible is adenocarcinoma of the ampullary-pancreatico-biliary region. Secondary involvement of the ampulla by pancreatic cancer can occasionally be specified based on the histological features and pattern of mucosal spread.

Benign pancreatic exocrine tumours: congenital cysts (Von Hippel Lindau Syndrome), acquired retention cyst, serous cystadenoma (elderly, macro-/microcystic, fluid filled, central scar, clear cuboidal epithelium).

Pancreatic exocrine tumours of malignant potential: intraductal papillary/mucinous and mucinous cystic tumours with a benign, borderline and malignant spectrum of behaviour related to the degree of epithelial dysplasia and extent of invasion into pancreatic parenchyma and peripancreatic fat. Often, in middle-aged women and uni- or multicystic, they show indolent growth with local spread to the abdomen but prior to this are potentially resectable. Solid pseudopapillary tumour - young females, pseudopapillae of uniform cells, cystic with necrosis, of low malignant potential, often benign.

Pancreatic exocrine carcinoma: arising from dysplastic pancreatic duct epithelium and forming the vast majority of pancreatic tumours, 80-90% are adenocarcinomas which are graded according to the degree of gland formation. Most (70-80%) arise in the pancreatic head with a minority in the body or tail and occasionally multifocal. Perineural invasion is characteristic and diagnostically helpful in biopsies. There is limited suitability for resection (5-10% of cases only). Node negative tumours of the pancreatic head < 3 cm in size may be resected by a Whipple's procedure with an average increase in survival of 1 year but a majority present with locally advanced disease into regional nodes and retroperitoneal tissues. Treatment is mainly palliative - pain control, nutritional support and relief of jaundice by open or laparoscopic bypass, or endo-scopic stent insertion to combat biliary obstruction.

Other cancers: unusual but include pleomorphic carcinoma, acinar cell carcinoma, small cell carcinoma, malignant lymphoma (usually from an adjacent nodal lymphoma) and sarcoma, which often represents spread from a primary sarcoma of gut or retroperitoneum.

Pancreatic endocrine tumours: single or multiple and forming a minority of pancreatic tumours they can be small (< 1-2 cm), well circumscribed and pale or yellow in colour. They are positive for general neuroendocrine markers (chromogranin, synaptophysin) and specific peptides, e.g., insulin, glucagon, gastrin. Many (60-85%) are associated with a functional hormonal syndrome, e.g., Zollinger-Ellison syndrome due to pancreaticoduodenal gastrinomas. The pancreas is also involved in 80-100% of type I multiple endocrine neoplasia (MEN) syndrome comprising hyperplasia or tumours of parathyroid, pituitary, adrenal glands and pancreas (usually gastrinoma). Histology does not reliably predict behaviour and better indicators of potential malignancy are functionality and established metastases - insulinoma (85% benign), gastrinoma (60-85% malignant), size > 3 cm, site (e.g., duodenal), invasion of vessels, nodes, adjacent organs and liver.

Extrahepatic bile duct carcinoma: there is an increased incidence in various disorders including ulcerative colitis, sclerosing cholangitis, gall stones and congenital bile duct anomalies. The majority (50-75%) arise in the upper third (including the hilum) with lesser numbers in the middle and distal thirds (10-25% each) or even diffuse and multifocal. Sometimes polypoid but often nodular, ulcerated, sclerotic or strictured, prognosis relates to the stage of disease, location and histological grade. There is characteristic perineural invasion often with involvement of regional lymph nodes, peritoneum or the liver (upper third tumours) at presentation. Other rare cancers are carcinoid tumour, malignant melanoma, lymphoma/leukaemia and in childhood, embryonal rhabdomyosarcoma.

Prognosis: prognosis of pancreatic ductal adenocarcinoma is poor with the majority of patients dead within a number of months. Chemotherapy may have a limited palliative role in some patients. Cystadenocarcinomas are relatively rare but potentially resectable. Pancreatic endocrine tumours have an indolent time course with a 50% 10-year survival and potential chemorespon-siveness even in the presence of metastases. Ampullary carcinoma has a five-year survival of 25-50%, improving to 80-85% if early stage (pT1) disease confined to the sphincter of Oddi. Distal bile duct cancers may be potentially resected with 25% five-year survival. Sclerosing bile duct carcinoma at the hilum (Klatskin tumour) can have an indolent course but the majority of bile duct cancers present late with very limited survival and only palliative biliary drainage (open bypass or laparoscopic/endoscopic stent insertion) is justified.

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