Pancreatic cancer. Risk factors. Screening for familial pancreatic cancer

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1 There is so much we don't know in medicine that could make a difference, and often we focus on the big things, and the little things get forgotten. To highlight some smaller but important issues, we've put together a series of pearls that the Red Whale found at the bottom of the ocean of knowledge! Pancreatic cancer This is one of those easily missed cancers because it is difficult to spot! Huge efforts are being made to increase public and professional awareness of symptoms, enabling us to spot it as early as possible. There is also much that primary care can do to support patients through diagnosis and treatment, and optimise their condition either prior to surgery or through symptom-based treatment. Pancreatic Cancer UK and Macmillan Cancer Support provided support for this article with both resources and feedback thank you! Pancreatic cancer statistics (CRUK accessed 2018) There are nearly new cases of pancreatic cancer diagnosed each year in the UK. Only 10% of cases will be resectable at the time of presentation. It has the worst 1y survival rate of any cancer. It is the 5th most common cause of cancer death, despite being the 11th most common cancer. Red flag symptoms often only present after the cancer has invaded other structures. 95% are from the exocrine portion of the pancreas, while 5% are endocrine. Risk factors Increasing age is a clear risk factor, with most cases occurring between age 60 and 80 (though we should remember it can be seen even in patients aged <40 years). The common risk factors for pancreatic cancer include (Lancet 2016;388:73). Smoking. Longstanding diabetes. Chronic pancreatitis. Obesity and low activity levels. Male sex. There is weaker evidence for an association with high red-meat consumption, high alcohol consumption and previous gallstone/gallbladder surgery. Genetics About 10% of cases of pancreatic cancer have a familial basis. Some genetic syndromes have been identified, but the genetic basis for most familial cases is not known. Associated genetic syndromes include: Genetic syndrome Lifetime risk (%) Hereditary pancreatitis (PRSS1, SPINK1) 50 Familial atypical multiple mole syndrome (p16) BRCA1, BRCA2, PALB2 1 2 Peutz Jeghers syndrome (STK11 [LKB1]) Hereditary non-polyposis coli (MLH1, MSH2, MSH6) 4 Screening for familial pancreatic cancer Given that 10% of cases are familial, NICE has issued new guidance on the diagnosis and management of pancreatic cancer, aimed mainly at secondary care (NICE 2018;NG85). However, it recommends: Offer surveillance for pancreatic cancer to people with: Hereditary pancreatitis and a PRSS1 mutation. BRCA1, BRCA2, PALB2 or CDKN2A (p16) mutations, and one or more first-degree relatives with pancreatic cancer. Peutz-Jeghers syndrome. These patients are likely to already be under the genetics clinic.

2 Consider surveillance for pancreatic cancer for people with: Two or more first-degree relatives with pancreatic cancer, across two or more generations. Lynch syndrome (mismatch repair gene [MLH1, MSH2, MSH6 or PMS2] mutations) and any first-degree relatives with pancreatic cancer. In practice, this may mean that we refer patients with two or more first-degree relatives with pancreatic cancer for a genetics assessment. When to refer For the first time, the new NICE 2015 suspected cancer referral guidelines offer us advice on when to suspect pancreatic cancer and how to refer: Refer via suspected cancer pathway (2ww) Patients aged over 40 with jaundice. Consider urgent direct access CT scan referral (within 2w): To assess for pancreatic cancer in patients aged >60 with weight loss and any of the following: Diarrhoea. Back pain. Abdominal pain. Nausea/vomiting. Constipation. New-onset diabetes. If CT is not available, refer for urgent ultrasound, though note this is not a rule-out test and may miss up to 10% of pancreatic cancers. N.B. For some of these symptom combinations, other cancers may be equally or more likely, e.g. colorectal, ovarian and prostate clinical judgement and more than one referral may be necessary. NICE also reminds us that cancers present with non-site-specific symptoms. We should think about pancreatic cancer in our differential for patients presenting with: Unexplained weight loss. Unexplained loss of appetite. Also consider pancreatic cancer in patients with persistent gastrointestinal symptoms and repeated consultations, e.g. constipation, diarrhoea or indigestion. In practice, we may investigate these individuals with colonoscopy or gastroscopy first, but need to remain vigilant if their clinical condition is deteriorating, even if there were initial negative test results. We say this often, but beware making a new diagnosis of IBS in older people it is rare! Macmillan have a handy Rapid Referral Guideline which is based on the NICE 2015 guidance but can be personalised with local contact details. It can be found here Evidence for the new referral criteria NICE bases its referral criteria on high-quality primary care-based cohort and case control studies which look at the positive predictive value of individual and combined presenting symptoms in patients with subsequently diagnosed pancreatic cancer. Any individual or combination of symptoms with a positive predictive value of 3% was included. Interestingly, it doesn t distinguish between all the different types of jaundice, e.g. obstructive vs. non-obstructive, or painful vs. painless, but does consider other causes more likely if the patient is aged <40. So, we still have to use our clinical judgement here because, even in those aged >40, the differential is broad. Why recommend CT or ultrasound? CT may be the gold standard investigation because it can image the whole pancreas and stage disease simultaneously. However, it is not perfect and is not universally available in primary care; this situation is likely to change as a result of the new guidelines. Ultrasound has a sensitivity of 75 89% and specificity of 90 99% for the detection of pancreatic cancer. This depends on the user, how obese the patient is and how much bowel gas is present (Lancet 2016;388;73). NICE included this as an option, recognising that capacity may be an issue during the initial implementation of guidance. However, ultrasound cannot rule out pancreatic cancer, so, if clinical suspicion remains, onward referral will be needed a discussion with a local radiologist is likely to be helpful during the transition!

3 It is possible that endoscopic ultrasound or MRI/MRCP will turn out to be the best imaging method for early disease, and these are considered as options in secondary care in the new NICE Pancreatic Cancer guidelines (NG ). What about tumour markers? NICE reviewed the evidence for tumour markers, including CA19-9, CA7-24, CEA and beta-hcg, and found insufficient primary care-based evidence to recommend their use. The available evidence suggests they are neither sensitive nor specific enough for pancreatic cancer to act as a rule-out test. Do not request tumour markers in patients with suspected pancreatic cancer. However, in patients who have been diagnosed with pancreatic cancer and who are found to have raised tumour markers in secondary care, we may be asked to use these to monitor disease activity. Why is it easily missed? This article from the BMJ Easily Missed series reminds us that pancreatic cancer is easily missed because its initial presenting features are often non-specific and are common to many other conditions which present in a similar age group, e.g. back pain, abdominal pain (BMJ 2014;349:g6385). The classic presentation of painless jaundice is usually a relatively late symptom. The most recent data suggests around 40% of cases present as emergencies; historically, only 11% have been referred through the 2ww pathway, and these cases have had a better prognosis. To diagnose it earlier, we need a higher degree of suspicion. Macmillan Cancer Support and Pancreatic Cancer UK have worked together to produce great top tips for GPs. Key points to remember are: Symptoms can be vague and varied. Consider pancreatic cancer: o In patients with new onset diabetes, especially if aged over 60 or if previously stable diabetes suddenly becomes unstable. o If conditions such as gastro-oesophageal reflux disease, gallstones, IBS, hepatitis or pancreatitis are not improving with treatment. o In new-onset mechanical back pain associated with gastrointestinal symptoms. o In repeated consultations for GI symptoms that are not resolving. You could ask a patient to use a symptom diary for a defined period, e.g. 4 weeks, and safety net to return if symptoms persist ALM.pdf Public awareness appeal A 2017 public health campaign funded by Pancreatic Cancer Action has placed posters, beer mats and toilet adverts to try and raise public awareness of symptoms. They encourage people to report: Unexplained and significant weight loss. Abdominal pain or discomfort that can come and go, which tends to get worse when lying down. Indigestion that s not responding to prescribed medication. Yellowing of the skin and/or eyes, dark urine or very itchy skin (jaundice). Fatty and pale stools that are smelly and hard to flush. Mid-back pain. Macmillan and PCUK also worked together to produce a helpful 10 top tips leaflet for patients which can be useful for safety netting and for giving concerned, higher risk patients to help them know when to report symptoms and signs to their GP. A link to this can be found below. Management of pancreatic cancer A clinical review covers the current treatment options (Lancet 2016;388:73). Treatment with intention of cure Surgery

4 The only curative treatment at present is surgical resection. Only 15 20% of patients are felt to be candidates for surgery at the time of presentation, and many of these will have microscopically positive margins after histology. When performed for curative intent, surgery has a 10 15% 5y survival. It usually involves a pancreaticoduodenectomy (removal of the proximal pancreas, distal stomach, duodenum, distal bile duct and gallbladder) pretty major! Morbidity following surgery is as high as 40%, and 30d mortality is between 2.4 and 9% depending on the volume of procedures performed by that centre. In addition to the tumour being resectable, the patient must be medically fit for surgery. Primary care has a role in this by promoting good nutrition (see below) as soon as possible, and ensuring other long-term conditions are managed as effectively as possible. Chemotherapy For those with resectable tumours, neoadjuvant chemotherapy may be offered prior to surgery to try to increase the chance of disease-free margins. NICE states that this should only be in the context of a clinical trial. Adjuvant chemotherapy may be offered after surgery, and has been shown to improve survival in specific groups. Radiotherapy has not been shown to improve survival. Palliative treatment If surgical resection is not possible, palliative treatment may relieve symptoms and improve quality of life. Surgical bypass and stenting procedures may relieve obstruction. Palliative chemotherapy may be offered to slow disease progress. Some patients report transient improvement in symptoms if there is some tumour shrinkage, and, for some, it offers an important sense of doing something. They may benefit from regular contact with the team. This will be an individual decision. Pancreatic enzymes may be administered to improve absorption of food, and reduce cachexia and symptoms of malabsorption. Pain is a common symptom, and specialist help may be needed if managing this proves difficult. Delayed gastric emptying may be reduced using prokinetics such as metoclopramide, and relieving excess acid with a PPI. Nasogastric decompression may be helpful if very symptomatic, and stenting procedures fail or are not possible. NICE on management: issues for primary care NICE issued guidance in 2018 which is mainly aimed at our secondary care colleagues (NICE NG ). It does not have any impact on the referral criteria for suspected cancer pathways. The main points for us to be aware of are: The importance of psychological support where there are ongoing gastrointestinal symptoms, fatigue and pain. The ongoing need some will have for nutritional support, including pancreatic enzyme supplements. Where pain management is complex, use of coeliac plexus block is recommended. If you have a patient with pancreatic cancer where pain is an ongoing issue, seek specialist advice about this. Pancreatic cancer and pancreatic enzyme replacement therapy Malnutrition and malabsorption can be significant issues in pancreatic cancer. They impact on fitness for treatment, especially surgery, and quality of life and wellbeing. Pancreatic Cancer UK has produced helpful guidelines on nutritional issues for patients with pancreatic cancer; a link can be found below. Pancreatic cancer patients should ideally see a specialist dietician. They should, if appropriate, be prescribed pancreatic enzyme replacement therapy (PERT). This can reduce symptoms and improve quality of life. The usual doses would be: units for a main meal units for a snack or milky drink. Capsules should be taken whole with the first few mouthfuls of food. This dose may need increasing if steatorrhoea or malabsorption symptoms remain. Of note, all prescribed PERT therapy is made from pork products, which may be a barrier for some cultural groups. Jewish and Muslim leaders have stated that use is acceptable in this situation.

5 Take Home Message Further Information Other resources Pancreatic cancer 10% of pancreatic cancer cases have a familial basis. Refer patients aged >40 with jaundice for urgent 2ww assessment. Consider referral of patients aged >60 with weight loss and any other symptom of abdominal pain, back pain, recent onset diabetes, diarrhoea, nausea/vomiting or constipation for urgent CT (or USS if CT is not available). Beware of common conditions, e.g. indigestion or gastrointestinal symptoms that present repeatedly and are not responding as expected to treatment. Remember, a normal USS does not rule out pancreatic cancer it will miss 10% of tumours. Do not request tumour markers. Remember to consider nutritional issues and pancreatic enzyme replacement therapy. Search your patient records for cases of pancreatic cancer in the past 5y. Reflect on whether the new referral guidelines would have helped to make a swifter diagnosis. Does your diabetes diagnosis protocol include consideration of red flag symptoms which may prompt further investigation? Discuss this at a PHCT meeting. Find out if you have access to direct urgent CT imaging liaise with your CCG to see if the service can be commissioned. For professionals: If you are an EMIS or VISION practice, the Cancer Decision Support tool may be helpful and can be integrated into your systems A risk scoring tool which is accessible to everyone can be found at: Macmillan Cancer Support 10 top tips can be found here : Printed copies of Macmillan s NICE endorsed Rapid Referral Guidelines can be ordered free of charge here : For patients: Safety netting and empowering: Symptoms of pancreatic cancer: How is pancreatic cancer diagnosed: Diet and pancreatic cancer: Managing symptoms: Pain Fatigue We make every effort to ensure the information in these articles is accurate and correct at the date of publication, but it is of necessity of a brief and general nature, and this should not replace your own good clinical judgement, or be regarded as a substitute for taking professional advice in appropriate circumstances. In particular check drug doses, side-effects and interactions with the British National Formulary. Save insofar as any such liability cannot be excluded at law, we do not accept any liability for loss of any type caused by reliance on the information in these articles.

6 Did you know that Macmillan provide a wealth of useful information for primary care professionals? Take a look through their quarterly e-newsletter, Primary Care Update and check out the range of useful resources on their Support page! Support for Primary Care: Primary Care Update e-newsletter:

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