ABC of diseases of liver, pancreas, and biliary system: Pancreatic tumours

Abstract
Ductal adenocarcinoma Incidence and prognosis Carcinoma of the pancreas has become more common in most Western countries over the past three decades, and although there is evidence of plateauing in some countries such as the United States, it still ranks as the sixth commonest cause of cancer death in the United Kingdom. Most patients are over the age of 60 years (80%) and many will have concurrent medical illnesses that complicate management decisions, particularly because the median survival from diagnosis is less than six months. Factors predicting poor prognosis Back pain Rapid weight loss Poor performance status—for example, World Health Organization or Karnofsky scoring systems Ascites and liver metastases High C reactive protein and low albumin concentrations Clinical presentation Two thirds of pancreatic cancers develop in the head of the pancreas, and most patients present with progressive, obstructive jaundice with dark urine and pale stools. Pruritus, occurring as a result of biliary obstruction, is often troublesome and rarely responds to antihistamines. Back pain is a poor prognostic sign, often being associated with local invasion of tumours. Severe cachexia, as a result of increased energy expenditure mediated by the tumour, is also a poor prognostic indicator. Cachexia is the usual presenting symptom in patients with tumours of the body or tail of the pancreas. Rarer presentations of pancreatic carcinoma Recurrent or atypical venous thromboses (thrombophlebitis migrans) Acute pancreatitis Late onset diabetes mellitus Upper gastrointestinal bleeding Examination The commonest sign is jaundice, with yellowing of the sclera and, once the bilirubin concentration exceeds 35 μmol/l, the skin. Many patients with high bilirubin concentrations will have skin scratches associated with pruritus. Patients with advanced disease have severe weight loss accompanied by muscle wasting and occasionally an enlarged supraclavicular lymph node. A palpable gall bladder suggests pancreatic malignancy, but it can be difficult to detect when displaced laterally or covered by an enlarged liver. The presence of ascites or a palpable epigastric mass usually indicates end stage disease. Full assessment of the patient's general fitness is essential to develop an individualised management plan. View larger version: In this window In a new window Patient with jaundice, bruising, and weight loss due to pancreatic carcinoma Investigation Because of the poor prognosis, care should be taken not to overinvestigate or embark on treatment strategies based on the unrealistic expectations of patients, their families, or the referring doctor. An increasing number of investigations are available, and the aim is to select patients who will not benefit from major resection by use of the fewest, least invasive, and least expensive means. The choice of investigation will vary according to local availability, particularly of newer investigations such as laparoscopic and endoscopic ultrasonography, and it remains to be seen if these techniques offer major advantages over the latest generation of computed tomography and magnetic resonance imaging scanners. Early cooperation between a gastroenterologist, radiologist, and surgeon should avoid inappropriate investigations and treatment that might interfere with patients' quality of life. Endoscopic retrograde cholangiopancreatography is an important investigation in patients with obstructive jaundice. As well as showing biliary and pancreatic strictures, the pathology can be confirmed by taking brushings for cytology or biopsy specimens of the duct for histology. The technique can also be used to place a stent to relieve biliary obstruction. However, it is important not to use this approach before patients are properly selected for treatment. View larger version: In this window In a new window Investigation and management of pancreatic ductal carcinoma The diagnosis can also be confirmed by fine needle aspiration guided by ultrasonography or computed tomography, but this investigation has a high rate of false negative results and is rarely necessary. Fine needle aspiration should be avoided in patients with potentially resectable tumours as it can cause seeding and spread of the tumour. Treatment Surgical resection does not improve survival in patients with locally advanced or metastatic disease. Tumour stage and the patient's fitness for major surgical resection are the main factors in determining optimal treatment. Resectable tumours Surgical resection, usually a pancreaticoduodenectomy (Whipple's procedure), is the only hope for cure. Less than 15% of tumours are suitable for resection. Very few tumours of the body and tail are resectable (3%) as patients usually present late with poorly defined symptoms. View this table: In this window In a new window Treatment of pancreatic ductal carcinoma The outcome of resection has been shown to be better in specialised pancreatobiliary centres that perform the procedure regularly than in small units. Mortality has fallen to 5-10% in dedicated units. The overall five year survival rate of 10-15% after resection remains disappointing, although survival is as high as 20-30% in some subgroups such as patients with small (100 μmol/l) probably benefit from endoscopic stenting and reduction of bilirubin concentrations before surgery. Locally advanced disease Several options are available for the 65% of patients who have locally advanced disease. These depend on factors such as age, disease stage, and the patient's fitness. Endoscopic insertion of a plastic or metal wall stent relieves jaundice in most patients. Plastic stents are cheaper but have a median half life of three to four months compared with six months for metal stents. Blockage of a stent results in rigors and jaundice, and patients should be given antibiotics and have the stent replaced. View larger version: In this window In a new window Metal wall stent in...