Upper GI - 2WW

 Map of Medicine is not responsible for the correctness or accuracy of any content uploaded, referred to or linked to from the system.
Efforts are made to ensure the accuracy and agreement of these guidelines. However, we cannot guarantee this. This guidance does not override the individual responsibility of healthcare professionals to make decisions appropriate to the circumstances of the individual patient, in consultation with the patient and/or guardian or carer, in accordance with the mental capacity act, and informed by the summary of product characteristics of any drugs they are considering. Practitioners are required to perform their duties in accordance with the law and their regulators and nothing in this guidance should be interpreted in a way that would be inconsistent with compliance with those duties

Scope:

• presentation, diagnosis, and management of upper gastrointestinal (oesophageal and gastric) tumours, including:
  • squamous cell cancer of the thoracic oesophagus
  • adenocarcinoma of the oesophagus and stomach
• non-surgical and surgical therapy
• assessment and management of adults with pancreatic ductal adenocarcinoma
• covers primary and specialist care

Out of scope:

• squamous cell cancer of the cervical oesophagus
• diagnosis and management of pre-malignant Barrett’s oesophagus
• other rare tumours (eg lymphoma, small cell cancer, or gastrointestinal stromal tumours)

• management of other pancreatic malignancies, eg:

  • neuroendocrine tumours, eg insulinomas, glucagonomas
  • mucinous cystic neoplasms of the pancreas
  • serous cystic neoplasms of the pancreas
  • other periampullary cancers such as:
    • cancer of the lower end of the bile duct (cholangiocarcinoma)
    • cancer of the Ampulla of Vater
    • duodenal cancer 
  • lymphoma 

Incidence, prevalence, and prognosis:

• upper gastrointestinal (UGI) cancer affects approximately:

  • 1 per 100,000 people age 40 years or younger [2]
  • 155 per 100,000 people age 55 years or older [2]
• the most common oesophago-gastric tumours are lower-third oesophageal and oesophago-gastric junctional (OGJ) adenocarcinomas [1]
• adenocarcinoma of the gastric cardia has also increased, however despite this, the incidence of gastric cancer is declining because of a decreased incidence in distal gastric tumours [1]
• 1-year survival rates are approximately 40% for both oesophageal and gastric cancer [3]
• 5-year survival rates are approximately [3]:
  • 17% for gastric cancer
  • 13% for oesophageal cancer
• the incidence of pancreatic cancer is higher in Western or industrialised countries
• pancreatic cancer is the ninth most common cancer in the UK [19]
• in the UK, pancreatic cancer is the fifth most common cause of cancer death [19]
• the annual incidence of pancreatic cancer is approximately 9.5 per 100,000 in 2009 [19]
• pancreatic cancer is rare before age 40 years however expert opinion says cases regularly present between age 30-40 years [21]
• 80% of cases of pancreatic cancer occur in those age 60 years and over [19]
• pancreatic cancer has a poor prognosis overall [19]
• the annual mortality rate in 2010 from pancreatic cancer is 8.6 per 100,000 [19]
• the one-year and five-year survival rates in the UK (for patients diagnosed 2005-2009) is 18.2% and 3.7%, respectively [19]
• potentially curative surgery is a treatment option for approximately 12-20% of patients [3,4]
• the 5 year survival rate post-resection and chemotherapy is estimated to be between 21% and 29% [4]

Risk factors include:

• age – peak age group affected is between age 50 and 60 years [1]
• male gender – men have approximately twice the rate of developing oesophageal cancer [4]
• Barrett’s oesophagus and reflux [4]:

  • Barrett’s oesophagus is strongly associated with gastroesophageal reflux disease and an increased risk of oesophageal adenocarcinoma

  • 20-fold increased risk of developing oesophageal adenocarcinoma in patients with Barrett's oesophagus
• obesity – strongly associated with oesophageal adenocarcinoma [4]
• Helicobacter pylori (H. pylori) infection [1]:
  • is associated with gastric cancer
  • however, it may also have a protective effect against developing OGJ cancer
• smoking [1]
• excessive alcohol consumption [1]

• many studies have linked periampullary cancer to many potential risk factors, but evidence has provided strong association with the following:

  • smoking − accounts for approximately 25-30% of cases [2]
  • type 2 diabetes mellitus (DM) diagnosed within the last 2 years [2]
  • genetic predisposition (present in 5-10% of patients) including [2,5]:
    • familial pancreatic cancer risk
    • familial cancer syndrome, eg breast (BRCA2 mutation), familial atypical multiple mole melanoma (FAMMM), Puetz-Jeghers syndrome, hereditary non-polyposis colon cancer syndrome (HNPCC), ovarian
    • hereditary pancreatitis
  • age:
    • age 75 years or older increases the risk of pancreatic cancer by 40% [2]
    • pancreatic cancer is uncommon in people under age 40 years [4]
  • other possible risk factors include [5]:
    • increased body mass index (BMI), including abdominal fatness [15] – approximately 1000 cases in the UK each year are linked to excess bodyweight [19]
    • diet:
      • high fat and protein intake
      • low fruit and vegetable intake
    • occupational exposure to chemicals, such as:
      • beta-naphthylamine
      • benzidine
    • high alcohol intake
  • chronic pancreatitis [19]

Prevention:

• there is a major public health need to prevent obesity, which may lead to a decrease in the incidence of UGI cancers [1]
• the following may reduce cancer incidence:
  • reducing tobacco and alcohol consumption [1] – consumption is strongly associated with squamous cell carcinoma of the oesophagus [2]
  • increasing fresh fruit and vegetable intake [1,2]
• H. pylori eradication is important in preventing gastric cancer, however its effect on OGJ cancer needs to be further evaluated [1]

Top Tips for GPs holding a 2WW Conversation 

Macmillan Rapid Referral Toolkit

UHB Patient Information Leaflet

NBT Patient Information Leaflet

WAHT 2WW Patient Information leaflet

• Oesophageal cancer (URL) from Cancer Research UK at http://www.cancerresearchuk.org/
• 'Stomach (gastric) cancer' (URL) from Cancer Research UK at http://www.cancerresearchuk.org/
• 'Stomach cancer' (URL) from Macmillan Cancer Support at http://www.macmillan.org.uk
• Oesophageal cancer (URL) from Macmillan Cancer Support at http://www.macmillan.org.uk

• 'Pancreatic cancer' (URL) from Macmillan Cancer Care at http://www.macmillan.org.uk 
• Pancreatic cancer (URL) from Cancer Research UK at http://www.cancerresearchuk.org/

NSCCG Sign off 2018

Updated in line with NICE Guideline NG12: Suspected cancer: recognition and referral to coincide with updated  BNSSG 2WW Referral forms

 Oncology sign off form

Date of publication: 31-Jan-2014

This care map has been redrafted to provide a more condensed specialist overview of upper gastrointestinal cancer and to include information from the following guidelines:

• [1] Association of Upper Gastrointestinal Surgeons of Great Britain and Ireland (AUGIS), British Society of Gastroenterology (BSG), British Association of Surgical Oncology (BASO) et al. Guidelines for the management of oesophageal and gastric cancer. Gut Online 2011.
• [3] Public Health England (PHE). Poorer outcomes for oesophageal and gastric cancer linked to 'huge variation' in endoscopy referral rates between GP practices.  2013.
• [4] British Society of Gastroenterology (BSG), Bennett C, Vakil N et al. Consensus statements for management of Barrett's dysplasia and early-stage esophageal adenocarcinoma, based on a Delphi process. Gastroenterology 2012; 143: 336-46.

• [6] Royal College of Surgeons of England (RCS).Clinical Effectiveness Unit, Association of Upper GI Surgeons (AUGIS), Royal College of Radiologists (RCR) et al. National oesophago-gastric cancer audit 2013. London: Royal College of Surgeons of England; 2013.

• [7] The Royal College of Physicians (RCP) and The Royal College of Radiologists (RCR). Evidence-based indications for the use of PET-CT in the United Kingdom 2012. London: RCP and RCR; 2012.

• [12] National Institute for Health and Clinical Excellence (NICE). Minimally invasive oesophagectomy. Interventional procedure 407. London: NICE; 2011.

Expert opinion has been added to this care map in line with:

• [10] Practice-informed recommendation; 2014.

Pancreatic screening is indicated for high risk patients [1].

Refer patients with high risk to a specialist centre offering genetic counselling and appropriate genetic testing [2], including people with a family history of:

• pancreatic cancer [4]
• pancreatitis, if a hereditary cause is suspected [4]
• familial cancer syndromes, eg:
  • breast [1] − BRCA2 mutation [4]
  • familial atypical multiple mole melanoma (FAMMM) [4]
  • Puetz-Jeghers syndrome [4]
  • hereditary non-polyposis colon cancer (HNPCC) [4]
  • ovarian [1]
• multiple mole melanoma [4]

A family history includes patients with [4]:

• two first degree relatives
• three cases in the family
• one first degree relative with an additional familial cancer syndrome (eg EUROPAC study)

Patients can present with dyspepsia and any of the alarm features below :

• dyspepsia can be defined broadly to include patients with recurrent epigastric pain, heartburn, or acid regurgitation, with or without bloating, nausea, or vomiting
• alarm features :

  • dysphagia

  • persistent vomiting 
  • progressive unintentional weight loss
  • chronic gastrointestinal bleeding
  • iron deficiency anaemia
  • palpable epigastric mass
  • recent onset dyspepsia in patients age 55 years or older
  • suspicious barium meal result

Presentation of pancreatic cancer in primary care is rare − a typical GP will encounter a case of pancreatic cancer once every 5 years [6].

Presenting symptoms of pancreatic cancer:

•  most common symptoms of pancreatic cancer:
  • abdominal pain:
    • most common presenting symptom
    • occurs in approximately 70% of cases [6]
  • jaundice − occurs in approximately 50-80% of cases with disease in head of pancreas usually obstructive or extrahepatic [6,21]
  • weight loss
• other symptoms may masquerade for underlying pancreatic cancer, though there are a number of other conditions which may give rise to these symptoms and include [21]:
  • nausea
  • anorexia
  • malaise
  • vomiting
  • fatigue
  • loss of appetite
  • pain when eating
  • dysphagia
  • persistent back pain
  • change in bowel habit – constipation, diarrhoea
  • floating stools, steatorrhoea, foul smelling, difficult to flush
  • abdominal distension
  • unprovoked venous thromboembolism (VTE)
  • depression
  • dyspepsia or abdominal discomfort
  • unexplained acute pancreatitis
• recent onset (within the past 2 years) of adult type 2 diabetes mellitus (DM) in patients age 50 years or older [14]

Atypical presentation (usually indicative of inoperability):

• migratory thrombophlebitis
• palpable and fixed epigastric mass
• ascites
• supraclavicular lymphadenopathy

NB: Pancreatic cancer should be considered in patients with type 2 DM who have no predisposing features or family history of DM.

Discuss and document presence of the following symptoms and signs (including details regarding time of onset, rate of progression, and current severity).

History:

• dysphagia [2]
• nausea/vomiting [2]
• weight loss [2]
• heartburn [2]
• indigestion [2]
• acid reflux [2]
• gastrointestinal bleeding [8]
• epigastric pain [8]
• bloating [8]
• early satiety [2]
• sensation of fullness in the stomach [2]
• location and nature of abdominal pain
• constitutional symptoms:
  • malaise
  • anorexia
  • nausea
  • diarrhoea
• pale or floating stools
• change in bowel habit
• yellowing of skin or sclera

Examination:

• signs of anaemia/ jaundice [2,10]
• abdominal masses [2,10]
• enlarged liver [10]
• weight loss [10]
• lymph glands [10]
• NB: patients presenting early may not have any signs on examination and therefore the history is critical [10]
• jaundice
• abdominal mass, including any organomegaly, presence/absence of ascites, palpable gall bladder
• peripheral lymphadenopathy, including supraclavicular
• signs of cachexia, ascites, or unusual naevi or moles (indicative of any familial cancer syndrome)

NB: A normal examination does not exclude pancreatic cancer.

Awareness of 'at risk' individuals is essential to facilitate early referral for assessment – discuss and document presence of the following risk factors (including appropriate detail):

• Barrett's oesophagus [1,8]
• gastro-oesophageal reflux disease (GORD) [1,8]
• obesity [1,8]
• previous peptic ulcer and gastric surgery [1,8]
• Helicobacter pylori (H. pylori) infection [1]
• smoking [2]
• alcohol consumption [2]
• diet [2]
• pernicious anaemia [1,2]
• previous history of acute/chronic pancreatitis
• type 2 diabetes mellitus (DM)
• family history of malignancy

• possible occupational exposure to chemicals, eg:

  • beta-naphthylamine

  • benzidine

Macmillan Cancer Support Rapid Referral Guidelines

If GP suspects symptoms caused by liver of gall bladder arrange an urgent Ultrasound scan.

 If patient is jaundiced or reason to suspect pancreatic cancer do not wait for urgent ultrasound scan.

Perform liver function test in parallel with 2WW referral.

Do not delay referral waiting for the blood resuls or scans if not already performed.

Ensure that the following recent blood results are made in parallel with the referral (less than 8 weeks old):

• FBC
• MCV
• LFT
• U&E
• Iron studies

For suspected liver and pancreatic cancer, include CA 19-9, Clotting.

 
Referral criteria

Jaundiced patient with suspected pancreatic cancer

Non-jaundiced patient

CT indicates pancreatic cancer

Ultrasound scan indicates pancreatic cancer

Upper GI 2WW Services are available at NBT, UHB and WAHT

Ultrasound results indicates gall bladder or liver cancer

Upper GI 2WW Services are available at NBT, UHB and WAHT.

 
 This referral form is available as an EMIS template

Oesophageal and stomach cancer

These patients may be referred directly for an endoscopy.

Referral criteria

• Upper abdominal mass consistent with stomach cancer
• Dysphagia
• Aged over 55 with weight loss and at least one of the following:
  • Upper abdominal pain
  • Reflux
  • Dyspepsia

Upper GI 2WW Services are available at NBT, UHB and WAHT

NBT request 2WW GI referrals via ICE using the GP Diagnostics tab. If the patient doesn't meet the set criteria on ICE but cancer is suspected, or if NBT ICE access is not available please refer via e-RS and note on the referral form why unable to submit on ICE.

Routine referrals for an upper GI appointment or a direct access endoscopy are to be made via ICE or by ERS

 
 
 This referral form is available as an EMIS template

Barrett's oesophagus:

• the replacement of distal oesophageal squamous mucosa with metaplastic columnar epithelium [4]
• found in 4% of patients undergoing an upper gastrointestinal (UGI) endoscopy [4]
• strongly associated with gastroesophageal reflux disease and increased risk of oesophageal adenocarcinoma [4]
• if on endoscopy progression from Barrett's oesophagus to high-grade dysplasia (HGD) or early adenocarcinoma is found, the patient should be referred to an UGI cancer multidisciplinary team (MDT) [4]
• diagnosis of HGD should be made by two histopathologists, one with a special interest in gastrointestinal disease [1]
• patients with low-grade dysplasia (LGD) should have a repeat endoscopy after treating with high dose proton-pump inhibitor (PPI) to exclude artefact caused by inflammatory regeneration [10]
• LGD has an increased risk of progression to HGD or malignancy compared to Barrett's oesophagus without dysplasia and should be followed closely with regular surveillance endoscopies – currently recommended at 6 month intervals [10]
• Barrett's oesophagus without dysplasia has low risk of progression to malignancy – current recommendation for surveillance is at 2 year intervals [10]

References:

[1] Association of Upper Gastrointestinal Surgeons of Great Britain and Ireland (AUGIS), British Society of Gastroenterology (BSG), British Association of Surgical Oncology (BASO) et al. Guidelines for the management of oesophageal and gastric cancer. Gut Online 2011.

[4] British Society of Gastroenterology (BSG), Bennett C, Vakil N et al. Consensus statements for management of Barrett's dysplasia and early-stage esophageal adenocarcinoma, based on a Delphi process. Gastroenterology 2012; 143: 336-46.

[10] Practice-informed recommendation; 2014.

Patients presenting with dyspepsia who are under age 55 years and do not have any alarm features do not require urgent endoscopic investigation [8,10].

Patients can be referred for endoscopic evaluation through a non-urgent pathway if symptoms are unresponsive to empirical therapy, or if there are risk factors for pre-malignant conditions such as Barrett's oesophagus or family history of upper gastrointestinal cancer [10]. 

References:

[8] National Institute for Health and Clinical Excellence (NICE). Referral guidelines for suspected cancer. Clinical guideline 27. London: NICE; 2005.

[10] Practice-informed recommendation; 2014.