v1 GUIDELINES Guidelines for the management of patients with pancreatic Gut: first published as 10.1136/gut.2004.057059 on 11 May 2005. Downloaded from cancer periampullary and ampullary carcinomas Pancreatic Section of the British Society of Gastroenterology, Pancreatic Society of Great Britain and Ireland, Association of Upper Gastrointestinal Surgeons of Great Britain and Ireland, Royal College of Pathologists, Special Interest Group for Gastro-Intestinal Radiology ............................................................................................................................... Gut 2005;54(Suppl V):v1–v16. doi: 10.1136/gut.2004.057059 1.0 GUIDELINES—SUMMARY lymph node metastases. Variants of ductal DOCUMENT carcinomas and other malignant tumours of The following recommendations are introduced the pancreas are rare. by brief statements which summarise the evi- dence and discussion presented in the relevant section of the full text of the guidelines. Recommendations 1.1 Incidence, mortality rates, and N Proper recognition of variants of ductal aetiology carcinomas and other malignant tumours Pancreatic cancer is an important health problem of the pancreas require specialist pathol- for which no simple screening test is available. ogical expertise (grade C). The strongest aetiological association is with N The minimum data set proposed by the cigarette smoking, although at risk groups Royal College of Pathologists (see appen- include patients with chronic pancreatitis, adult dix for details) should be used for report- onset diabetes of less than two years’ duration, hereditary pancreatitis, familial pancreatic can- ing histological examination of pancreatic cers, and certain familial cancer syndromes. resection specimens (grade C). Periampullary cancers are a feature of familial adenomatous polyposis. 1.3 Clinical features In the majority of patients, the clinical diagnosis Recommendations is fairly straightforward, although there are no http://gut.bmj.com/ positive clinical features which clearly identify a N Continued health education to reduce patient group with potentially curable disease. tobacco consumption should lower the risk There are associated conditions, such as late of developing pancreatic carcinoma onset diabetes mellitus or an unexplained attack (grade B). of acute pancreatitis, which may point to an N All patients at increased inherited risk of underlying cancer. A number of clinical features (persistent back pain, marked and rapid weight pancreatic cancer should be referred to a on September 30, 2021 by guest. Protected copyright. specialist centre offering specialist clinical loss, abdominal mass, ascites, and supraclavicu- advice and genetic counselling and appro- lar lymphadenopathy) usually indicate an incur- priate genetic testing (grade B). able situation N Secondary screening for pancreatic cancer in high risk cases should be carried out as part of an investigational programme Recommendations coordinated through specialist centres (grade B). N The diagnosis of pancreatic cancer should N Examination and biopsy of the periampul- be considered in patients with adult on- lary region is important in patients with set diabetes who have no predisposing longstanding familial adenomatous poly- features or family history of diabetes posis. The frequency of endoscopy is (grade B). determined by the severity of the duodenal N Pancreatic cancer should be excluded polyposis (grade B). during the investigation of patients who N Patients with stage 4 duodenal polyposis have had an unexplained episode of acute ....................... who are fit for surgery should be offered pancreatitis (grade B). Correspondence to: resection (grade B). Mr C D Johnson, University Surgical Unit, Mail point 816, Southampton Abbreviations: CT, computed tomography; MR, General Hospital, 1.2 Pathology magnetic resonance; MRCP, magnetic resonance cholangiopancreatography; ERCP, endoscopic Southampton SO16 6YD, Most pancreatic cancers are of ductal origin and UK; c.d.johnson@soton. retrograde cholangiopancreatography; MRA, magnetic ac.uk present at a stage when they are locally resonance angiography; FAP, familial adenomatous ....................... advanced, and exhibit vascular invasion and polyposis; EUS, endosonography; 5-FU, 5-fluorouracil www.gutjnl.com v2 Pancreatic Section of the British Society of Gastroenterology 1.4 Investigations The workup of patients with suspected pancreatic cancer Recommendations should logically focus initially on establishment of the Gut: first published as 10.1136/gut.2004.057059 on 11 May 2005. Downloaded from diagnosis and an assessment of the patient’s fitness to Stent or surgical palliation undergo potentially curative treatment. In selected patients, N Most patients requiring relief of obstructive jaundice further investigation involves tumour staging and the will be adequately treated by placement of a plastic assessment of local respectability. stent; surgical bypass may be preferred in patients likely to survive more than six months (grade A). N Duodenal obstruction should be treated surgically (grade C). Recommendations Stent insertion N Endoscopic stent placement is preferable to trans- N Clinical presentation suggesting cancer of the pancreas hepatic stenting (grade A). should lead without delay to ultrasound of the liver, bile duct, and pancreas (grade B). N After failure of endoscopic stent placement, percuta- neous placement of a self expanding metal stent, or a N When the diagnosis of pancreatic malignancy is combined radiological/endoscopic approach, will suspected from clinical symptoms and/or abdominal increase the number of patients who can be success- ultrasound findings, the selective use of computerised fully stented (grade B). tomography (CT), endoscopic retrograde cholangio- pancreatography (ERCP), and/or magnetic resonance N Both plastic and self expanding metal stents are (MR), including magnetic resonance cholangiopan- effective in achieving biliary drainage but require creatography (MRCP) and occasionally magnetic further development (grade A). Currently, the choice resonance angiography (MRA), will accurately deline- between these stents depends on clinical factors, local ate tumour size, infiltration, and the presence of availability, and local expertise (grade C). metastatic disease in the majority of cases (grade B). N If a stent is placed prior to surgery, this should be of the N Where available, endosonography and/or laparo- plastic type and it should be placed endoscopically. scopy with laparoscopic ultrasonography may be Self expanding metal stents should not be inserted in appropriate in selected cases (grade B). patients who are likely to proceed to resection (grade C). Resectional surgery N This should be confined to specialist centres, to increase resection rates and reduce hospital morbidity and 1.5 Tissue diagnosis mortality (grade B). N Pancreaticoduodenectomy (with or without pylorus preservation) is the most appropriate resectional http://gut.bmj.com/ procedure for tumours of the pancreatic head (grade B). Recommendations N Extended resections involving the portal vein or total pancreatectomy may be required in some cases but do not increase survival when carried out routinely (grade B). N Attempts should be made to obtain a tissue diagnosis during the course of investigative endoscopic proce- N Resection in the presence of preoperative detection of dures (grade C). portal vein encasement is rarely justified (grade C). on September 30, 2021 by guest. Protected copyright. N Failure to obtain histological confirmation of a N Percutaneous biliary drainage prior to resection in suspected diagnosis of malignancy does not exclude jaundiced patients does not improve surgical outcome the presence of a tumour, and should not delay and may increase the risk of infective complications appropriate surgical treatment (grade C). (grade A). N Efforts should be made to obtain a tissue diagnosis in N Left sided resection (with splenectomy) is appropriate patients selected for palliative forms of therapy (grade for localised carcinomas of the body and tail of the C). pancreas. Involvement of the splenic vein or artery is not in itself a contraindication to such resection. (grade B) N Transperitoneal techniques to obtain a tissue diagnosis have limited sensitivity in patients with potentially Palliative surgery resectable tumours and should be avoided in such N Duodenal bypass should be used during palliative patients (grade C). surgery (grade B). N Biliary bypass should be constructed with the bile duct in preference to the gall bladder (grade B). Non-surgical therapies 1.6 Treatment N This largely centres around palliative surgery undertaken to Adjuvant or neoadjuvant therapies in conjunction with relieve symptoms, resectional surgery with intent to cure, and surgery should only be given in the context of a clinical endoscopic or percutaneous biliary stenting to relieve trial (grade A). jaundice. There is an increasing use of chemotherapy and N If chemotherapy is used for palliation, gemcitabine radiotherapy, both as palliative treatments as well as in an single agent treatment is recommended (grade A). adjuvant setting in conjunction with surgery, although much N Therapy with novel treatments should only be offered to of this practice is not evidence based. Appropriately designed patients within clinical trials (grade C). multicentre clinical trials remain essential. www.gutjnl.com Guidelines for the management of pancreatic periampullary and ampullary carcinomas